1.2 clinical_history has_obo_format_version We scored as TRUE: Any instances noted where the patient required assistance with activities of daily living (ADL). This must be described more-or-less literally: ‘Activities of daily living', ADL or ‘needed help with washing and dressing’, or ‘required daily care’; decreased independence. We also scored as TRUE: ‘patient refused help with ADL or daily care’. We assume here that the patient requires help with ADL even if they are refusing it. We did NOT score: admission to nursing home – these were scored separately under ‘Admission to nursing home'. We did NOT score: any other sentences requiring even minimal interpretation. true UMLS: C0740765 Description: A patient can require help while living at home, due to physical or cognitive deterioration. When it is described in the summary that a patient has help with ‘activities of daily living’ (ADL) activities and/or help with personal care and hygiene. Help in Activities of daily living (ADL) Activities_of_daily_living_impaired http://purl.obolibrary.org/obo/Activities_of_daily_living_impaired Activities of daily living impaired Activities:of:daily:living:impaired UMLS: C0740765 Direct match We scored as TRUE: admission to nursing home; temporary admission to nursing home; admission to (psycho-geriatric) nursing home; admission to closed psychogeriatric ward; permanent admission to psychogeriatric ward; admitted to a home for the elderly. We also scored as TRUE: assisted care living; admitted to a care facility or any facility that are required when a person is no longer able to live independently in their own home. We also scored as TRUE: any mention that the patient is living in a nursing home or assisted living facility. We did NOT score: admission to psychogeriatric or psychiatric hospital. We did NOT score: hospice. We did NOT score: hospital admissions; admissions to psychogeriatric hospital/ward (except where it was clearly mentioned as a ward within the nursing home); psychiatric hospital. We did NOT score: admission to day-care facilities. These were scores separately as ‘Day care'. We did NOT score: coerced admissions. These are scored as ‘Psychiatric admissions’. true UMLS: C1658399 Description: Whenever it is written down that a patient is admitted to a nursing home or care facility, because living at home was not possible anymore due to increased need for help in ADL activities, and personal care and hygiene, we score this as TRUE. It is most often described as: patient was admitted to a nursing home, patient was admitted to a care facility. NOTE: The descriptions ‘nursing home’ and ‘care facility’ are used interchangeably in the clinical summaries of the NBB. Where a patient might get more help in a nursing home than in a care facility, the difference is not objectifiable when reading the clinical summaries. Therefore, we decided to score both these descriptions in this category, because in both cases it indicates that a patient was unable to live at their own home anymore (even though they might already have had help). Admission_to_nursing_home http://purl.obolibrary.org/obo/Admission_to_nursing_home Admission to nursing home Admission:to:nursing:home UMLS: C1658399 Direct match We scored as TRUE: coerced admission; forced or involuntary admission; ‘patient was admitted against their will’. We also scored as TRUE: voluntary psychiatric admissions; admitted to psychiatric ward. We did NOT score: permanent admissions to psychogeriatric nursing home. These were scored as ‘Admission to nursing home’. When it is clear that the admission was to a (closed) psychogeriatric ward of a nursing home, then it was scored as ‘Admission to nursing home’. We did NOT score: temporary admissions to psycho(geriatric) ward/hospital. false UMLS: C0237457 Description: Sometimes, patients with a psychiatric disease are dangerous for themselves or their surroundings, making it necessary to force an admission in a psychiatric care facility. This can also be the case in patients with progressive neurodegenerative diseases who lack insight and awareness into their illness and are in danger due to aggressive or changed behavior. Sometimes it is written down as: ‘crisis intervention followed by compulsory hospitalization/ treatment’, ‘forced admission’; ‘admission with legal authority’. Psychiatric admissions Admission_to_psychiatric_hospital http://purl.obolibrary.org/obo/Admission_to_psychiatric_hospital Admission to psychiatric hospital Admission:to:psychiatric:hospital UMLS: C0237457 Direct match We scored as TRUE: aggression; aggressive behavior. We did NOT score: sentences requiring interpretation such as 'patient hit people'. We did NOT score: angry. This was not scored anywhere. false UMLS: C0001807 Description: Patient shows verbally or physically aggressive behavior, which they normally did not do in certain situations. Also agitation or agitated behavior. We decided to make this a separate parameter, and not include it in ‘changed personality’. aggression Aggressive:behavior Aggressive_behavior Aggressive behavior Aggressive:behavior UMLS: C0001807 Direct match We scored as TRUE: agitation; agitated behavior. We did NOT score: aggressive behavior (this was scored as ‘Aggressive behavior’); restlessness (this is scored as ‘Restlessness’). We did NOT score: irritated; irritation; irritable; frustration; tense/tension; tantrums. These were NOT scored anywhere. false UMLS: C0085631 Definition: “Agitation is a behavioral syndrome characterized by increased, often undirected, motor activity, restlessness, aggressiveness, and emotional distress”. We have decided to score this separately from aggressive behavior, restlessness, changed emotions/moods, as these terms are frequently noted and often appear together. Agitation http://purl.obolibrary.org/obo/Agitation Agitation Agitation UMLS: C0085631 Direct match Definition: “Agitation is a behavioral syndrome characterized by increased, often undirected, motor activity, restlessness, aggressiveness, and emotional distress”. We have decided to score this separately from aggressive behavior, restlessness, changed emotions/moods, as these terms are frequently noted and often appear together. https://pubmed.ncbi.nlm.nih.gov/33935943/ We scored as TRUE: lack of recognition; mistakes in recognition (e.g. patient recognized another man as her husband); did not recognize friend/family (prosopagnosia); agnosia. We did NOT score: disorientation. This was scored separately as ‘Disorientation’. [Note: this may be ambiguous, so use your best judgement when choosing to score as ‘impaired recognition’ versus ‘disorientation (in place)’. Generally, if the word recognition is named, you would score it here]. CAUTION: We have thought about dividing this parameter into more than just this one parameter: ‘visual agnosia’ and other symptoms of posterior cortical atrophy. However, in the clinical summaries of the NBB, too often it was only described as agnosia, and not the specific type of agnosia, and certainly not more specific information about visual impairments. Researchers must be aware of the broad interpretation of the term ‘agnosia’. true UMLS: C0001816 Definition: “Agnosia is a rare disorder characterized by an inability to recognize and identify objects or persons. People with agnosia may have difficulty recognizing the geometric features of an object or face or may be able to perceive the geometric features but not know what the object is used for or whether a face is familiar or not. Agnosia can be limited to one sensory modality such as vision or hearing. For example, a person may have difficulty in recognizing an object as a cup or identifying a sound as a cough. Agnosia can result from strokes, dementia, developmental disorders, or other neurological conditions. It typically results from damage to specific brain areas in the occipital or parietal lobes of the brain. People with agnosia may retain their cognitive abilities in other areas”. Impaired recognition Agnosia http://purl.obolibrary.org/obo/Agnosia Agnosia Agnosia UMLS: C0001816 Direct match Definition: “Agnosia is a rare disorder characterized by an inability to recognize and identify objects or persons. People with agnosia may have difficulty recognizing the geometric features of an object or face or may be able to perceive the geometric features but not know what the object is used for or whether a face is familiar or not. Agnosia can be limited to one sensory modality such as vision or hearing. For example, a person may have difficulty in recognizing an object as a cup or identifying a sound as a cough. Agnosia can result from strokes, dementia, developmental disorders, or other neurological conditions. It typically results from damage to specific brain areas in the occipital or parietal lobes of the brain. People with agnosia may retain their cognitive abilities in other areas”. https://www.ninds.nih.gov/health-information/disorders/agnosia#disorders-r1 We scored as TRUE: amnesia, amnestic, amnestically, amnesic. We did NOT score: anything else. true UMLS: C0002622 Definition: “Amnesia refers to the loss of ability to recall facts, events, or concepts encountered prior to the onset of illness (retrograde amnesia) or to the loss of ability to form new memories (anterograde amnesia), or both. Although anterograde and retrograde amnesia can occur in isolation, they most often appear together following a single cause. That cause is most frequently a neurologic insult or illness but can also be psychogenic. In most cases, the memory loss is permanent, but it can be temporary, as for example, in transient global amnesia”. Amnesia http://purl.obolibrary.org/obo/Amnesia Amnesia Amnesia Definition: “Amnesia refers to the loss of ability to recall facts, events, or concepts encountered prior to the onset of illness (retrograde amnesia) or to the loss of ability to form new memories (anterograde amnesia), or both. Although anterograde and retrograde amnesia can occur in isolation, they most often appear together following a single cause. That cause is most frequently a neurologic insult or illness but can also be psychogenic. In most cases, the memory loss is permanent, but it can be temporary, as for example, in transient global amnesia”. https://link.springer.com/referencework/10.1007/978-0-387-79948-3 UMLS: C0002622 Direct match We scored as TRUE: anomia; word finding problems; minor difficulties with word finding; naming problems; unable to name objects. PLEASE NOTE: Patients with items scored under this parameter may fit a classification of ‘Logopenic aphasia’. However, given the subjective descriptions provided in the NBB reports, we are unable to accurately classify the different forms of aphasia. Furthermore, we cannot make a distinction between primary progressive aphasias and other forms of aphasia. Therefore, interpretation should be made with caution. true UMLS: C0003113 Definition: “Anomia generally refers to instances of word finding difficulty that occur during the course of conversational discourse. It is often documented clinically in confrontation picture or tasks”. This means patients have trouble finding the right words to say. In the clinical summaries of the NBB, ‘word finding problems’ is frequently reported like this. We have decided to score it as a separate parameter. Word-finding difficulties are often a sign of the logopenic variant of a primary progressive aphasia which is “…characterized by preserved syntax and comprehension but frequent and severe word-finding pauses, anomia, circumlocutions, and simplifications during spontaneous speech. Repetition is usually impaired”. Wordfinding problems Anomia http://purl.obolibrary.org/obo/Anomia Anomia Anomia UMLS: C0003113 Direct match Definition: “Anomia generally refers to instances of word finding difficulty that occur during the course of conversational discourse. It is often documented clinically in confrontation picture or tasks”. This means patients have trouble finding the right words to say. In the clinical summaries of the NBB, ‘word finding problems’ is frequently reported like this. We have decided to score it as a separate parameter. Word-finding difficulties are often a sign of the logopenic variant of a primary progressive aphasia which is “…characterized by preserved syntax and comprehension but frequent and severe word-finding pauses, anomia, circumlocutions, and simplifications during spontaneous speech. Repetition is usually impaired”. https://books.google.com/books?id=wNKVBgAAQBAJ https://link.springer.com/referencework/10.1007/978-0-387-79948-3 Mesulam MM. Aphasia, Memory Loss, Hemispatial Neglect, Frontal Syndromes, and Other Cerebral Disorders. In: Jameson JL, Fauci AS, Kasper DL, Hauser SL, Longo DL, Loscalzo J, eds. Harrison's Principles of Internal Medicine, 20e. New York, NY: McGraw-Hill Education; 2018. Raymer A. Anomia. In: Kreutzer JS, DeLuca J, Caplan B, eds. Encyclopedia of Clinical Neuropsychology. New York, NY: Springer New York; 2011:171-172. We scored as TRUE: lack/loss of insight; no insight; loss or lack of awareness. We also scored as TRUE: for example, ‘patient had no real knowledge of her condition'; 'patient had no awareness of disease'. We did NOT score: loss of overview – this was scored under ‘Lack of planning/organization/overview'. true UMLS:C0234507 Definition: An unwillingness or failure to recognize and deal with a deficiency or disease and its consequences. In the clinical summaries of the NBB, it is frequently described separately in the clinical history. It could fall under decreased cognition, but for this project we have decided to score it separately. Lack of insight Anosognosia http://purl.obolibrary.org/obo/Anosognosia Anosognosia Anosognosia UMLS:C0234507 Direct match Definition: An unwillingness or failure to recognize and deal with a deficiency or disease and its consequences. In the clinical summaries of the NBB, it is frequently described separately in the clinical history. It could fall under decreased cognition, but for this project we have decided to score it separately. https://accessmedicine.mhmedical.com/book.aspx?bookID=1477 Ropper AH, Samuels MA, Klein JP, Prasad S. Delirium and Other Acute Confusional States. In: Adams and Victor's Principles of Neurology, 11e. New York, NY: McGraw-Hill Education; 2019. We included any instance where a patient was anxious, or reported anxiety or being afraid of something, regardless of the diagnosis. We scored as TRUE: anxiety; anxious; phobia; panic attacks; fear; nervous(ness). We did NOT score: ambiguous sentences; patient is afraid e.g. patient was afraid of getting cancer/other disease (this can be a temporary/transient fear). “Patient's family were afraid...”. false UMLS: C0003467 Description: Disorders of anxiety include features of excessive fear and anxiety where fear is the emotional response to a real or perceived threat and anxiety is anticipation of future threat. Panic attacks are a particular feature of some anxiety disorders. There is significant overlap between anxiety and fear, and we cannot distinguish between them. Anxiety http://purl.obolibrary.org/obo/Anxiety Anxiety Anxiety UMLS: C0003467 Direct match Description: Disorders of anxiety include features of excessive fear and anxiety where fear is the emotional response to a real or perceived threat and anxiety is anticipation of future threat. Panic attacks are a particular feature of some anxiety disorders. There is significant overlap between anxiety and fear, and we cannot distinguish between them. American Psychiatric Association. Diagnostic and statistical manual of mental disorders, Fifth Edition. Vol 21. Arlington, VA.: American Psychiatric Association; 2013. We scored as TRUE: apathy; apathic; apathetic [please be careful not to confuse with the misspelled aphatic which should be scored as ‘dysphasia/aphasia’] We scored as TRUE: loss of interest. true UMLS: C0085632 Definition: Apathy generally refers to indifference or a lack of feeling or concern. It can manifest as a lack of drive or motivation, a lack of responsiveness (behavioral or emotional) to stimuli, or a lack of initiation, or a reduction in self-generated, purposeful behavior. Lack of feeling or emotion, lack of interest. Often noticed by close relatives of a patient. A patient often is not aware of it. Described as apathy or apathic. Here we want to be as objective as possible. Note: Lack of initiative is frequently described as such. We decided to make this a separate parameter. Apathy / inertia Apathy http://purl.obolibrary.org/obo/Apathy Apathy Apathy UMLS: C0085632 Direct match Definition: Apathy generally refers to indifference or a lack of feeling or concern. It can manifest as a lack of drive or motivation, a lack of responsiveness (behavioral or emotional) to stimuli, or a lack of initiation, or a reduction in self-generated, purposeful behavior. Lack of feeling or emotion, lack of interest. Often noticed by close relatives of a patient. A patient often is not aware of it. Described as apathy or apathic. Here we want to be as objective as possible. Note: Lack of initiative is frequently described as such. We decided to make this a separate parameter. https://pubmed.ncbi.nlm.nih.gov/21810890/ In the NBB reports, we were unable to distinguish PPA aphasias from other forms of aphasia. Therefore, we scored any instance in which the word aphasia (dysphasia) is mentioned. Please note: Literally, the word ‘aphasia’ means absence of language whereas dysphasia is a (mild to severe) loss of language. However, the word aphasia is frequently used as a synonym for dysphasia. We scored as TRUE: dysphasia (dysphasia); phatic (fatic) problems; aphasia (afasia). We did NOT score: anything else except other misspellings that were clearly interpreted. PLEASE NOTE: For this parameter we cannot make any distinction between the different forms of aphasia. true UMLS: C0003537 Definition: Aphasias arising from neurodegenerative diseases are referred to as primary progressive aphasia (PPA) and can be classified as Agrammatic PPA, Semantic PPA or Logopenic PPA. Dysphasia Aphasia http://purl.obolibrary.org/obo/Aphasia Aphasia Aphasia UMLS: C0003537 Direct match Definition: Aphasias arising from neurodegenerative diseases are referred to as primary progressive aphasia (PPA) and can be classified as Agrammatic PPA, Semantic PPA or Logopenic PPA. https://accessmedicine.mhmedical.com/content.aspx?bookid=2129&sectionid=191734545 Mesulam MM. Aphasia, Memory Loss, Hemispatial Neglect, Frontal Syndromes, and Other Cerebral Disorders. In: Jameson JL, Fauci AS, Kasper DL, Hauser SL, Longo DL, Loscalzo J, eds. Harrison's Principles of Internal Medicine, 20e. New York, NY: McGraw-Hill Education; 2018. In the clinical summaries of the NBB, apraxia was often mentioned, not distinguished which kind of apraxia. Sometimes it was more described without using the word ‘apraxia’. For the programming of scoring the right cases, we only labeled cases TRUE when it said ‘apraxia’, or ‘praxis impaired’. We scored as TRUE: apraxia; apraxic; changes in praxis; speech apraxia; apraxic gait AND misspellings that can clearly be interpreted as apraxia e.g. apractical; apractic; apraxy. We did NOT score: anything else. true UMLS: C0003635 Definition: “The inability to correctly carry out a learned, skilled motor act despite the preserved capability of the sensorimotor system to produce the intended movement”. This can present in many different ways in neurodegenerative disorders: Praxis refers to the execution of learned motor movements in the absence of primary deficits in motor and spatial abilities. Apraxia typically presents with difficulty dressing, feeding, and/or bathing that are not explained by clear motor deficits. Ideomotor praxis (the ability to perform learned motor movements) can be evaluated by asking the patient to perform increasingly complex motor tasks. The patient may be asked to demonstrate the use of an object (e.g. comb, hammer, fork), with and without the actual object in their hands. Ideational praxis (the ability to carry out a sequential set of actions towards a final goal) can be evaluated by asking the patient to perform a stepwise series of coordinated tasks, such as “take this piece of paper, fold it in half, and place it in the envelope”. Common praxis errors include using the wrong object or body part to perform a task. Many patients who cannot perform motor tasks to command can do so spontaneously or by imitation. Patients with neurodegenerative disease may also have difficulty performing a series of coordinated tasks. More specific or isolated problems with praxis suggest involvement of the dominant parietal lobe. Ideomotor apraxia is a relatively prominent feature of corticobasal degeneration. Apraxias Apraxias http://purl.obolibrary.org/obo/Apraxias Apraxias Apraxias UMLS: C0003635 Direct match Definition: “The inability to correctly carry out a learned, skilled motor act despite the preserved capability of the sensorimotor system to produce the intended movement”. This can present in many different ways in neurodegenerative disorders: Praxis refers to the execution of learned motor movements in the absence of primary deficits in motor and spatial abilities. Apraxia typically presents with difficulty dressing, feeding, and/or bathing that are not explained by clear motor deficits. Ideomotor praxis (the ability to perform learned motor movements) can be evaluated by asking the patient to perform increasingly complex motor tasks. The patient may be asked to demonstrate the use of an object (e.g. comb, hammer, fork), with and without the actual object in their hands. Ideational praxis (the ability to carry out a sequential set of actions towards a final goal) can be evaluated by asking the patient to perform a stepwise series of coordinated tasks, such as “take this piece of paper, fold it in half, and place it in the envelope”. Common praxis errors include using the wrong object or body part to perform a task. Many patients who cannot perform motor tasks to command can do so spontaneously or by imitation. Patients with neurodegenerative disease may also have difficulty performing a series of coordinated tasks. More specific or isolated problems with praxis suggest involvement of the dominant parietal lobe. Ideomotor apraxia is a relatively prominent feature of corticobasal degeneration. https://books.google.nl/books/about/Encyclopedia_of_Clinical_Neuropsychology.html?id=QxHHAQAACAAJ&redir_esc=y https://www.uptodate.com/contents/the-mental-status-examination-in-adults?search=neuropsychological%20examination&source=search_result&selectedTitle=1~150&usage_type=default&display_rank=1#H28388635 Katz DI. Apraxia. In: Kreutzer JS, DeLuca J, Caplan B, eds. Encyclopedia of Clinical Neuropsychology. New York, NY: Springer New York; 2011:235-236. Mendez MF. The mental status examination in adults. UpToDate. https://www.uptodate.com/contents/the-mental-status-examination-in-adults?search=neuropsychological%20examination&source=search_result&selectedTitle=1~150&usage_type=default&display_rank=1#H28388635. Published 2021. Accessed 9 June, 2021. Grouping variable containing aspecific signs and symptoms Aspecific:symptoms Aspecific_symptoms Aspecific symptoms Aspecific:symptoms We scored as TRUE: ataxia; atactic(al); atactic gait; wide-based gait; walked with a wide base. [NOTE: when these terms refer to gait, it was also scored as TRUE. Other changes in gait are scored elsewhere]. true UMLS: C0004134 Definition: Ataxia is a lack of muscle control or coordination of voluntary movements. It can affect various movements and create difficulties with speech, eye movement, swallowing and gait disorder. Mostly it is the result of damage to the part of the brain that controls muscle coordination, the cerebellum. Many conditions can cause ataxia, including alcohol misuse, certain medication, stroke, tumor, cerebral palsy, brain degeneration and MS, inherited defective genes can also cause ataxia. In the clinical summaries of the NBB, ataxia is mostly noted when it concerns a cerebellar ataxia and observed in neurological examination. We included all cases where the word ‘ataxia’ or words that derive from this word. Furthermore, a wide based gait, wide-based walk or broad-based gait, which often refers to a cerebellar origin, can also be described as atactic gait. Ataxia http://purl.obolibrary.org/obo/Ataxia Ataxia Ataxia UMLS: C0004134 Direct match Definition: Ataxia is a lack of muscle control or coordination of voluntary movements. It can affect various movements and create difficulties with speech, eye movement, swallowing and gait disorder. Mostly it is the result of damage to the part of the brain that controls muscle coordination, the cerebellum. Many conditions can cause ataxia, including alcohol misuse, certain medication, stroke, tumor, cerebral palsy, brain degeneration and MS, inherited defective genes can also cause ataxia. In the clinical summaries of the NBB, ataxia is mostly noted when it concerns a cerebellar ataxia and observed in neurological examination. We included all cases where the word ‘ataxia’ or words that derive from this word. Furthermore, a wide based gait, wide-based walk or broad-based gait, which often refers to a cerebellar origin, can also be described as atactic gait. Gelb D. The detailed neurologic examination in adults. UpToDate; Waltham MA. 2012. Gonzalez-Usigli HA. Coordination Disorders. MSD. https://www.msdmanuals.com/home/brain,-spinal-cord,-and-nerve-disorders/movement-disorders/coordination-disorders. Published 2020. Accessed 9 June, 2021. Grouping variable containing signs/symptoms/findings associated with autnomic dysfunction. Autonomic:dysfunction Autonomic_dysfunction Autonomic dysfunction Autonomic:dysfunction We scored as TRUE: bradykinesia, hypokinesia, akinesia, reduced arm swing and hypokinetic rigid syndrome*; starting problems; general slowness or slowing of movement. [Please note: we have decided to capture general slowness here, however we cannot be certain whether it refers to bradykinesia or bradyphrenia]. *Note: hypokinetic rigid syndrome was also scored as ‘Muscle rigidity’. We also scored as TRUE: freezing or freezing of gait and shuffling gaits. We did NOT score: hypomimia; mask-like facial expression; altered mimic. These were scored separately as 'Facial masking'. We did NOT score: bradyphrenia (slowness in thinking, says more about cognitive functioning). We did NOT score: dyskinesia; monotonic voice; bradycardia (heart beat slow). These were not scored anywhere. We did NOT score: dysdiadochokinesis; this was scored separately in ‘Loss of coordination’. CAUTION: “general slowness” can also be written down as a reference for “bradyphrenia”. However, this is often not specified. We decided to score it here but be aware of the possible meanings. true UMLS: C0233565 Definition: “slowing of spontaneous and automatic movement that can make it difficult to perform simple tasks or rapidly perform routine movements”. Reduction of automatic movements (such as blinking or swinging your arms when walking). Difficulty initiating movements (like getting up out of a chair). Bradykinesia is defined as slowness of movement plus a decrement in amplitude/speed or progressive hesitations/halts as movements are continued. Bradykinesia http://purl.obolibrary.org/obo/Bradykinesia Bradykinesia Bradykinesia UMLS: C0233565 Direct match Definition: “slowing of spontaneous and automatic movement that can make it difficult to perform simple tasks or rapidly perform routine movements”. Reduction of automatic movements (such as blinking or swinging your arms when walking). Difficulty initiating movements (like getting up out of a chair). Bradykinesia is defined as slowness of movement plus a decrement in amplitude/speed or progressive hesitations/halts as movements are continued. https://pubmed.ncbi.nlm.nih.gov/26474316/ National Institute of Neurological Disorders and Stroke (NINDS). Parkinson's disease information page. NIH. https://www.ninds.nih.gov/Disorders/All-Disorders/Parkinsons-disease-Information-Page. Published 2020. Accessed 9 June, 2021. We scored as TRUE: bradyphrenia; bradyfrenia; bradyphrenic; bradyfrenic; reduced mental tempo; slowness in thinking. This should be clear from the description. Use your best judgement. If in doubt do NOT score. We did NOT score: anything else. CAUTION: the term ‘general slowness’ has been encompassed under ‘Bradykinesia’ but it is possible that some instances of general slowness (also) refer to Bradyphrenia. true UMLS: C0554976 Definition: “a pathological slowing of mental processes”. Bradyphrenia http://purl.obolibrary.org/obo/Bradyphrenia Bradyphrenia Bradyphrenia UMLS: C0554976 Direct match Definition: “a pathological slowing of mental processes”. https://shop.elsevier.com/books/encyclopedia-of-movement-disorders/kompoliti/978-0-12-374101-1 Howard AK, Zurowski M. Bradyphrenia. In: Kompoliti K, Metman LV, eds. Encyclopedia of Movement Disorders. Oxford: Academic Press; 2010:161-165. We scored as TRUE: cachexia (literally as it is written, including misspellings), cachectic. false UMLS: C0006625 Definition: cachexia, is a complex metabolic syndrome associated with underlying illness and characterized by loss of muscle with or without loss of fat mass. The prominent clinical feature of cachexia is weight loss in adults (corrected for fluid retention). Cachexia http://purl.obolibrary.org/obo/Cachexia Cachexia Cachexia UMLS: C0006625 Direct match Definition: cachexia, is a complex metabolic syndrome associated with underlying illness and characterized by loss of muscle with or without loss of fat mass. The prominent clinical feature of cachexia is weight loss in adults (corrected for fluid retention). https://pubmed.ncbi.nlm.nih.gov/18718696/ Grouping variable containing signs of cerebellar and vestibular dysfunction. Cerebellar_and_vestibular_system_dysfunction http://purl.obolibrary.org/obo/Cerebellar_and_vestibular_system_dysfunction http://purl.obolibrary.org/obo/http://www.semanticweb.org/irholtman/ontologies/2023/Cerebellar_and_vestibular_system_dysfunction Cerebellar and vestibular system dysfunction Cerebellar:and:vestibular:system:dysfunction NB 'Socially inappropriate behavior' is a separate parameter because loss of decorum is frequently written down as such. One might argue that aggressive behavior and apathy are type of behaviors that contribute to a changed personality. These behavioral parameters have a certain overlap, which could make it difficult to distinguish which one to label as TRUE. A changed personality is often described in frontotemporal dementia, or a psychiatric disease. We labeled changed personality only when it was written down as such, as TRUE. We scored as TRUE: changed personality/character; the personality/character of the person was changed; behavioral changes; changed behavior. We scored as TRUE: anti-social behavior; passive behavior; behavioral insecurity; claiming behavior; socially withdrawn; inadequate/inappropriate behavior; any clear change in a person’s behavior. We did NOT score: was not interested in many things; loss of initiative – this was scored as ‘Lack of initiative'. We did NOT score: paranoia or suspiciousness. These were scored as ‘Paranoia’. We did NOT score: loss of decorum; agitation; aggressive behavior; apathy. These were scored separately as ‘Socially inappropriate behavior’, ‘Agitation’, ‘Aggressive behavior’ or ‘Apathy’, respectively. true UMLS: C0240735 UMLS: C0542299 Description: With a changed personality, we mean that the behavior of a patient/donor has changed significantly compared to his/her ‘normal’ behavior. This is often first noticed by family-members and partners. Patient’s often do not notice it themselves. 'Loss of decorum' is also common as a sign of changed personality, also most often first noticed by family members. Behavioral change Personality change Changed_behavior_personality http://purl.obolibrary.org/obo/Changed_behavior_personality Excluded due to poor model performance Changed behavior/personality Changed:behavior:personality UMLS: C0240735 UMLS code refers to a narrower concept UMLS: C0542299 UMLS code refers to a narrower concept Here we wanted to encompass significant changes in mood or emotion that are not encompassed by other more extreme parameters such as depressed mood, manic, psychosis, delusions, apathy and so on. We still wanted to be as objectively as possible, so we look for the key words emotion or mood. We scored as TRUE: emotional; increased emotionality; altered mood; changed mood; fluctuating moods or emotions; labile/lability. We did NOT score: loss/lack of sympathy; loss/lack of empathy; without emotion; no emotions. These were scored separately as ‘Loss of sympathy / empathy’. We did NOT score: apathy. This was frequently reported and so it was scored separately as 'Apathy'. false UMLS:C0085633 Description: “The emotional life of an individual is expressed in a variety of ways. It is widely appreciated that there are marked individual differences in basic temperament. Throughout their lives some persons are cheerful, gregarious, optimistic, and free from worry, whereas others are just the opposite. The state of emotionality, and changes that are uncharacteristic to the individual lend themselves to observation and have clinical significance. Furthermore, some inherent personality traits may precede the development of overt mental disease. For example, the volatile, person is said to be liable to bipolar disease, and the suspicious, withdrawn, introverted person to schizophrenia and paranoia, but there are frequent exceptions to these statements”. In the behavioral variant of Frontotemporal dementia, this may be exhibited as range of different inappropriate behaviors, most of which are scored separately (e.g. loss of empathy, Apathy, Socially inappropriate behavior). Changed moods or emotions Changed_moods_or_emotions http://purl.obolibrary.org/obo/Changed_moods_or_emotions Changed moods or emotions Changed:moods:or:emotions UMLS:C0085633 Closest UMLS match Description: “The emotional life of an individual is expressed in a variety of ways. It is widely appreciated that there are marked individual differences in basic temperament. Throughout their lives some persons are cheerful, gregarious, optimistic, and free from worry, whereas others are just the opposite. The state of emotionality, and changes that are uncharacteristic to the individual lend themselves to observation and have clinical significance. Furthermore, some inherent personality traits may precede the development of overt mental disease. For example, the volatile, person is said to be liable to bipolar disease, and the suspicious, withdrawn, introverted person to schizophrenia and paranoia, but there are frequent exceptions to these statements”. In the behavioral variant of Frontotemporal dementia, this may be exhibited as range of different inappropriate behaviors, most of which are scored separately (e.g. loss of empathy, Apathy, Socially inappropriate behavior). https://pubmed.ncbi.nlm.nih.gov/21810890/ Ropper AH, Samuels MA, Klein JP, Prasad S. Delirium and Other Acute Confusional States. In: Adams and Victor's Principles of Neurology, 11e. New York, NY: McGraw-Hill Education; 2019. Grouping variable containing signs/symptoms/findings associated with changes in consciousness/awaraness/orientation. Changes_in_consciousness_awareness_orientation http://purl.obolibrary.org/obo/Changes_in_consciousness_awareness_orientation Changes in consciousness/awareness/orientation Changes:in:consciousness:awareness:orientation UMLS CUI: C1516691 Domain variable containing signs/symptoms/findings associated with cognitive disturbances. Cognitive http://purl.obolibrary.org/obo/Cognitive Cognitive Cognitive Grouping variable containing signs and symptoms indicative of cognitive and memory problems. Cognitive_and_memory_impairment http://purl.obolibrary.org/obo/Cognitive_and_memory_impairment Cognitive and memory impairment Cognitive:and:memory:impairment We scored as TRUE: cognitive decline; decreased cognition; cognitive deficits; deterioration in cognition; fluctuating cognition; cognitive slowing; mental deterioration. Here we only scored as TRUE when the word cognitive or cognition was present. We did NOT score: reduced/decreased/loss of consciousness. This was not scored anywhere. We did NOT score: decreased understanding, this was scored separately as ‘Impaired comprehension’; decreased ability to learn. This was not scored anywhere. We did NOT score: loss/lack of insight – this was scored separately as ‘Lack of insight’. We did NOT score: reduced speech; language problems; word-finding difficulties. The latter two were scored separately [‘Language impairment’ or ‘Word-finding problems (anomia)’, respectively]. We did NOT score: communication difficulties – these were scored as ‘Communication impairment’. We did NOT score: imprinting, confusion or claiming behavior as these were scored separately - ‘Poor short-term memory', ‘Confusion', or ‘Changed behavior or personality’, respectively. We did NOT score: diagnoses e.g. Alzheimer's dementia was diagnosed/confirmed. These were scored in a separate section of the database. true UMLS:C0338656 This is a rather broad concept. Whenever there is impairment in memory, concentration, attention, orientation, or language, one could say there is cognitive decline, or decreased cognition. The clinical summaries of the NBB, frequently only state that there is ‘cognitive decline’ or ‘cognitive deterioration’. This is the reason why it is also noted as a single parameter. Impaired cognition Cognitive:decline Cognitive_decline Cognitive decline Cognitive:decline UMLS:C0338656 UMLS code refers to a broader concept We scored as TRUE: communication was difficult; communication was hardly possible; lack of communication; impaired communication; conversation was difficult. [In some cases, this will be subjective. Use your best judgement and when in doubt, do not score]. We did NOT score: dysphasia/aphasia; language impairment; comprehension problems; dysarthria. These were all scored elsewhere as ‘Aphasia (dysphasia)’, ‘Language impairment’, ‘Impaired comprehension’, or ‘Dysarthria’. true UMLS: C0009460 Description: In the NBB reports, difficulties with communication are frequently reported. These cannot be scored as ‘Language impairment’ or ‘Dysphasia’ or anywhere else because they represent a broad range of communication difficulties. For example, it can refer to people with ALS who require a communication device, someone with a particular form of aphasia, or someone with a memory impairment. We have decided to report of communication problems separately. Communication problems Communication:impairment Communication_impairment Communication impairment Communication:impairment UMLS: C0009460 Direct match We scored as TRUE: compulsive behavior; obsessive behavior; perseverated; perseverations; repetitive or stereotypical behavior; urge (e.g. urge to walk/wander but NOT urinary urgency). We did NOT score: anything else. true UMLS: C0600104 Definition: Perseverative, stereotyped, or compulsive ritualistic behaviors include stereotyped speech, simple repetitive movements, and complex ritualistic behaviors such as hoarding, checking, or cleaning. Some patients develop new hobbies or interests, particularly those with a religious aspect, which they pursue obsessively. Other behaviors traditionally associated with obsessive compulsive disorder, such as hand washing and germ phobias, are generally absent. Patients with FTD can develop a rigid personality, rigid food preferences, and inflexibility to changes in routine. Obsessive compulsive behavior Perseverative, stereotyped or compulsive/ritualistic behavior Compulsive:behavior Compulsive_behavior Compulsive behavior Compulsive:behavior UMLS: C0600104 UMLS code refers to a narrower concept Definition: Perseverative, stereotyped, or compulsive ritualistic behaviors include stereotyped speech, simple repetitive movements, and complex ritualistic behaviors such as hoarding, checking, or cleaning. Some patients develop new hobbies or interests, particularly those with a religious aspect, which they pursue obsessively. Other behaviors traditionally associated with obsessive compulsive disorder, such as hand washing and germ phobias, are generally absent. Patients with FTD can develop a rigid personality, rigid food preferences, and inflexibility to changes in routine. https://pubmed.ncbi.nlm.nih.gov/21810890/ American Psychiatric Association. Diagnostic and statistical manual of mental disorders, Fifth Edition. Vol 21. Arlington, VA.: American Psychiatric Association; 2013. Lee SE. Frontotemporal dementia: Clinical features and diagnosis. UpToDate. https://www.uptodate.com/contents/frontotemporal-dementia-clinical-features-and-diagnosis. Published 2020. Accessed 9 June, 2021. In the clinical summaries of the NBB, this term can be found in cases with a neurodegenerative disease and psychiatric cases as well. We scored as TRUE: confabulation(s); confabulate(d); confabulating. That is, we only scored when the word confabulate was present. true UMLS: C0233800 “Confabulation is a neuropsychiatric disorder wherein a patient generates a false memory without the intention of deceit. The patient believes the statement to be truthful, hence the descriptive term “honest lying.” The hypothesis is that the patient generates information as a compensatory mechanism to fill holes in one’s memories. It functions for self-coherence, integration of memories, and self-relevance. Confabulations can include small details such as birthdays, or they may be fantastical and more broadly based. They can be believable or bizarre. Presenting patients with contradictory information may further perpetuate confabulation in an attempt to explain their account”. Confabulation http://purl.obolibrary.org/obo/Confabulation Confabulation Confabulation UMLS: C0233800 Direct match “Confabulation is a neuropsychiatric disorder wherein a patient generates a false memory without the intention of deceit. The patient believes the statement to be truthful, hence the descriptive term “honest lying.” The hypothesis is that the patient generates information as a compensatory mechanism to fill holes in one’s memories. It functions for self-coherence, integration of memories, and self-relevance. Confabulations can include small details such as birthdays, or they may be fantastical and more broadly based. They can be believable or bizarre. Presenting patients with contradictory information may further perpetuate confabulation in an attempt to explain their account”. https://www.ncbi.nlm.nih.gov/books/NBK536961/ We scored as TRUE: confusion; confused; confused behavior. false UMLS: C0009676 Definition: “Confusion is a general term denoting the patient’s incapacity to think with customary speed, clarity, and coherence”. Confusion http://purl.obolibrary.org/obo/Confusion Confusion Confusion UMLS: C0009676 Direct match Definition: “Confusion is a general term denoting the patient’s incapacity to think with customary speed, clarity, and coherence”. https://accessmedicine.mhmedical.com/book.aspx?bookID=1477 Ropper AH, Samuels MA, Klein JP, Prasad S. Delirium and Other Acute Confusional States. In: Adams and Victor's Principles of Neurology, 11e. New York, NY: McGraw-Hill Education; 2019. For this project we included all notations of Constipation, no matter what the cause might be. We scored as TRUE: constipation; obstipation; coprostasis; difficult defecation; slowed defecation; painful defecation. We did NOT score: when receiving medicines for constipation such as laxatives. We did NOT score: diarrh(o)ea; f(a)ecal incontinence; incontinent for f(a)eces; smearing f(a)eces. true UMLS: C0009806 Definition: Slowed or difficult defecation. This can be caused by, amongst other things: decreased motility of the bowel system (which can also be cause by different entities, like inactivity, impaired autonomic nerve system) insufficient fiber intake and diet, medication. It is a common symptom in patients with Parkinson’s disease. Constipation http://purl.obolibrary.org/obo/Constipation Constipation Constipation UMLS: C0009806 Direct match We scored as TRUE: admission to day-care facility; day-care center; dependent on day care; went to day care … times/week. We did NOT score: required daily care. This was scored separately as ‘Activities of daily living impaired’. true UMLS: C0011017 Description: Day care is a facility where patients with a form of dementia or neurodegenerative disorder go during the day, for activities and guidance. The patient still lives at home. Day care Day_care_medical http://purl.obolibrary.org/obo/Day_care_medical Day care, medical Day:care:medical UMLS: C0011017 Direct match We want to score all our donors on this aspect, whether they have lost fine motor skills. In the clinical summaries of the NBB, it is frequently written down as ‘decreased (fine) motor skills’. It mostly refers to “decreased fine motor skills” even though it is written down as “decreased motor skills”. Fine (=hand) motor skills are typically what is described as impaired. The words “motor skills” should be in the sentence, combined with a word that indicates its decline. We also score for reduced hand-writing skills as these are frequently mentioned. One might argue that when a patient suffers from rigidity, automatically fine motor skills will be impaired. For this project we aim to score the parameters as objective as possible, without interpretations. Again, we want to stick to the described parameters as much as possible, so when the summary says something about decreased motor skills, only then will we score it as TRUE. PLEASE NOTE: we scored for any instance where the term ‘motor skills’ is specifically stated. This term nearly always refers to fine motor skills. However, we cannot guarantee that we only captured fine motor skills. We scored as TRUE: reduced or impaired (fine/gross) motor skills; reduced hand-writing skills; micrographia. We did NOT score: motor restlessness; motoric restlessness. These were scored only as ‘Restlessness’. We did NOT score: problems with balance or coordination; reduced propulsion – these were scored separately as ‘Balance problems’. We did NOT score: difficulties/problems with walking; walking with an aid; use of a wheelchair; leaned over when walking; falling to the side when walking. These were scored separately as ‘Impaired mobility’. We did NOT score: altered gait; wide-based gait; shuffling gait; wide-based walk; freezing gait. These were scored elsewhere – either as ‘Bradykinesia’, ‘Ataxia’. We did NOT score: spasticity; spastic gait. These were scored separately as ‘Upper motor neuron signs - Spasticity’. We did NOT score: ataxia, ataxic gait. These are scored as ‘Ataxia’. Imbalanced gait or festinating gait – these were scored as ‘Balance problems’. We did NOT score: other gait problems such as unsure/cautious gait. These were scored as ‘Disturbed gaits’. We did NOT score: extrapyramidal symptoms, this was scored as ‘Extrapyramidal symptoms/Parkinsonism’. We did NOT score: reduced arm swing. This was scored separately as ‘Bradykinesia’. We did NOT score: Dysarthria, this is scored as ‘Dysarthria’; dysphagia, this was scored as ‘Swallowing problems’. We did NOT score: loss of strength, this is scored in “Muscle Weakness”; leaned over/bent over, these were scored as ‘Impaired mobility’. We did NOT score: changed reflexes. These were only scored when they fit under the parameter ‘Upper motor neuron signs – hyperreflexia and other pathological reflexes’. true UMLS: C5233646 UMLS:C1867864 Description: Loss of motor function is common in older persons, but also as a symptom or precursor of a neurodegenerative disease. Motor function is not a unitary process, but different motor abilities derive from the coordinated activity of varied motor control systems located throughout the brain and spinal cord, and that extend via the peripheral nervous system to musculoskeletal structures. Motor control systems that regulate the initiation, planning and execution of motor performances are located in multiple interconnected cortical and subcortical motor regions. Decreased (fine) motor skills Decreased_fine_motor_skills http://purl.obolibrary.org/obo/Decreased_fine_motor_skills http://purl.obolibrary.org/obo/http://www.semanticweb.org/irholtman/ontologies/2023/Decreased_fine_motor_skills Decreased (fine) motor skills Decreased:fine:motor:skills UMLS: C5233646 UMLS code refers to a broader concept UMLS:C1867864 UMLS refers to a narrower concept In the clinical summaries of the NBB, a patient often suffered from a delirium or delirious episode, regardless the underlying diagnosis. We include all delirium or delirious episodes when it has already been established in the medical records, so whenever delirium is written down, or delirious episode, we labeled it as TRUE. We scored as TRUE: only when it was literally written as delirium; delirious; deliriant; or any other word that specifically relates to delirium. We did NOT score: reduced level of consciousness or any other terms; reduced level of awareness (this was too ambiguous to be scored). Explanation: occasionally delirium is described in the NBB case reports as a ‘reduced level of consciousness’. However, in most cases, references to consciousness or reduced awareness indicate a complete loss of consciousness or syncope due to reduced blood flow. To avoid interpretation, we do not score reduced level of consciousness, meaning that some instances of delirium may have been missed. false UMLS: C0011206 Definition: Delirium is “…characterized by disturbance in attention that makes it difficult for the individual to direct, sustain and shift their focus” (p. 635). It can be caused by many different things: drugs or toxins, infections, metabolic derangements. Delirium http://purl.obolibrary.org/obo/Delirium Delirium Delirium UMLS: C0011206 Direct match Definition: Delirium is “…characterized by disturbance in attention that makes it difficult for the individual to direct, sustain and shift their focus” (p. 635). It can be caused by many different things: drugs or toxins, infections, metabolic derangements. https://pubmed.ncbi.nlm.nih.gov/25266297/ We scored as TRUE: Delusion(s); delusional; delusional behavior. We also scored as TRUE: Capgras syndrome, a clear description that indicates a delusion. Use your best judgement. If in doubt, do NOT score. We did NOT score: any other terms. false UMLS: C0011253 Definition: “Strongly held false beliefs or opinions not based on evidence. For example, a person may think his or her spouse is having an affair or that relatives long dead are still living. Another delusion that may be seen in people with LBD is Capgras syndrome, in which the person believes a relative or friend has been replaced by an imposter”. Delusions http://purl.obolibrary.org/obo/Delusions Delusions Delusions UMLS: C0011253 Direct match Definition: “Strongly held false beliefs or opinions not based on evidence. For example, a person may think his or her spouse is having an affair or that relatives long dead are still living. Another delusion that may be seen in people with LBD is Capgras syndrome, in which the person believes a relative or friend has been replaced by an imposter”. National Institute of Neurological Disorders and Stroke (NINDS). Lewy body dementia: Hope through research. NIH. https://www.ninds.nih.gov/Disorders/Patient-Caregiver-Education/Hope-Through-Research/Lewy-Body-Dementia-Hope-Through-Research. Published 2020. Accessed 9 June, 2021. We scored as TRUE: any mention of dementia even if it was suspected/suggested then we scored as TRUE: Alzheimer's dementia; vascular dementia; frontotemporal dementia; ‘Lewy body dementia’; Parkinson’s dementia. Even though these were ‘diagnoses' we want to capture all types of dementia. Therefore, we also scored as TRUE: Alzheimer's disease. Other forms of dementia scored as TRUE: corticobasal degeneration; progressive supranuclear palsy; primary progressive aphasia; chronic traumatic encephalopathy; Pick’s disease. Note: this was the only parameter where we also score diagnoses that include the word of the Parameter ‘dementia’, or which indicate the presence of “dementia”. We did NOT score: Parkinson’s disease or other neurodegenerative diseases unless dementia was mentioned. true UMLS: C0497327 Definition: “Dementia is a syndrome – usually of a chronic or progressive nature – in which there is deterioration in cognitive function (i.e. the ability to process thought) beyond what might be expected from normal ageing. It affects memory, thinking, orientation, comprehension, calculation, learning capacity, language, and judgement. Consciousness is not affected. The impairment in cognitive function is commonly accompanied, and occasionally preceded, by deterioration in emotional control, social behavior, or motivation”. Here we want to score the broader meaning of dementia and not individual symptoms of disease. In the clinical summaries of the NBB, the word ‘dementia’ is frequently used. Either as part of a diagnosis, or as a separate word to describe the state of mind, of when a definitive diagnosis was not present (yet). We included this parameter to score all cases that suffer from “dementia” in any form or diagnosis. Dementia http://purl.obolibrary.org/obo/Dementia Dementia Dementia UMLS: C0497327 Direct match Definition: “Dementia is a syndrome – usually of a chronic or progressive nature – in which there is deterioration in cognitive function (i.e. the ability to process thought) beyond what might be expected from normal ageing. It affects memory, thinking, orientation, comprehension, calculation, learning capacity, language, and judgement. Consciousness is not affected. The impairment in cognitive function is commonly accompanied, and occasionally preceded, by deterioration in emotional control, social behavior, or motivation”. Here we want to score the broader meaning of dementia and not individual symptoms of disease. In the clinical summaries of the NBB, the word ‘dementia’ is frequently used. Either as part of a diagnosis, or as a separate word to describe the state of mind, of when a definitive diagnosis was not present (yet). We included this parameter to score all cases that suffer from “dementia” in any form or diagnosis. World Health Organization. Dementia. WHO. https://www.who.int/news-room/fact-sheets/detail/dementia. Published 2020. Accessed 9 June, 2021. Here we scored depressed mood in every case it is reported. We scored as TRUE: Depressed mood; sad; sadness; somber mood; somber; somberness; felt down; anhedonia; rumination; decreased mood; or felt lonely; depression. We scored as TRUE: major depressive disorder as it was not possible for the machine learning to differentiate disease from symptom. We did NOT score: suicidal ideation, ‘has a death wish’. These were scored separately as 'Suicidal ideation’. false UMLS: C0344315 Description: “Depressive disorders include disruptive mood dysregulation disorder, major depressive disorder (including major depressive episode), persistent depressive disorder (dysthymia), premenstrual dysphoric disorder, substance/medication-induced depressive disorder, depressive disorder due to another medical condition, other specified depressive disorder, and unspecified depressive disorder. The common feature of all of these disorders is the presence of sad, empty, or irritable mood, accompanied by somatic and cognitive changes that significantly affect the individual’s capacity to function. What differs among them are issues of duration, timing, or presumed etiology.” Depressed:mood Depressed_mood Depressed mood Depressed:mood UMLS: C0344315 Direct match Description: “Depressive disorders include disruptive mood dysregulation disorder, major depressive disorder (including major depressive episode), persistent depressive disorder (dysthymia), premenstrual dysphoric disorder, substance/medication-induced depressive disorder, depressive disorder due to another medical condition, other specified depressive disorder, and unspecified depressive disorder. The common feature of all of these disorders is the presence of sad, empty, or irritable mood, accompanied by somatic and cognitive changes that significantly affect the individual’s capacity to function. What differs among them are issues of duration, timing, or presumed etiology.” American Psychiatric Association. Diagnostic and statistical manual of mental disorders, Fifth Edition. Vol 21. Arlington, VA.: American Psychiatric Association; 2013. Disorientation in time, place or person were scored here. We scored as TRUE: Cognitively disorientated, got lost (wearing nothing, but underclothes), orientation problems, disorientation, disoriented in time and/or place. We also scored as TRUE: Any sentence that clearly describes that the patient is disoriented in time and place. Use your best judgement. If in doubt, do NOT score. We did NOT score: problems with spatial orientation; any ambiguous sentences. true UMLS: C0233407 Definition: “Disorientation is an alteration of mental status characterized by lack of awareness of personal identity, place, time, and/or situation. Typically, disorientation occurs first in time, then in place, and finally in person. It is assessed by asking the person specific questions in these spheres. Disorientation is a relatively nonspecific symptom occurring in diffuse disorders (i.e., dementia), focal brain lesions (i.e., stroke), and infectious and metabolic processes, secondary to medications and drug interactions, and in psychological disorders (i.e., schizophrenia)”. Disorientation http://purl.obolibrary.org/obo/Disorientation Disorientation Disorientation UMLS: C0233407 Direct match Definition: “Disorientation is an alteration of mental status characterized by lack of awareness of personal identity, place, time, and/or situation. Typically, disorientation occurs first in time, then in place, and finally in person. It is assessed by asking the person specific questions in these spheres. Disorientation is a relatively nonspecific symptom occurring in diffuse disorders (i.e., dementia), focal brain lesions (i.e., stroke), and infectious and metabolic processes, secondary to medications and drug interactions, and in psychological disorders (i.e., schizophrenia)”. Winegardner J. Disorientation. In: Kreutzer JS, DeLuca J, Caplan B, eds. Encyclopedia of Clinical Neuropsychology. Cham: Springer International Publishing; 2018:1193-1193. https://link.springer.com/referencework/10.1007/978-3-319-57111-9 Grouping variable containing signs/symptoms/findings associated with disturbances in mood and behaviour. Disturbances_in_mood_and_behaviour http://purl.obolibrary.org/obo/Disturbances_in_mood_and_behaviour http://purl.obolibrary.org/obo/http://www.semanticweb.org/irholtman/ontologies/2023/Disturbances_in_mood_and_behaviour Disturbances in mood and behaviour Disturbances:in:mood:and:behaviour Sub-grouping variable containing signs and symptoms associated with disturbances of language and speech. These include aphasia, language impairment, word-finding, and communication. Definition: Aphasia (dysphasia) is the inability to comprehend or produce language (spoken or written). It often occurs following damage to particular brain regions following a trauma or injury to the head, vascular insult such as with cerebrovascular accident (CVA; stroke), or as a consequence of neurodegenerative diseases. All components of the language network are interconnected so deficits arising from injury will depend on the number and distribution of brain regions affected. Affected language aspects include word-finding, word choice, comprehension, spelling, or grammar. Aphasias caused by CVA are generally sudden onset and may be severe whereas aphasias caused by neurodegenerative diseases usually have an insidious onset with a steady and relentless progression. Hence, aphasia arising from neurodegenerative diseases are referred to as primary progressive aphasia (PPA) and can be classified as Agrammatic PPA, Semantic PPA or Logopenic PPA. The neurodegenerative disease most commonly associated with PPA include Alzheimer’s disease and frontotemporal dementias. Signs/symptoms of aphasia are generally only reported in the NBB reports in a subjective manner (i.e. only an example of an individual’s speech/language problem is provided) rather than an objective assessment. Therefore, it is not possible to classify disturbances in language, comprehension and communication as agrammatic, semantic or logopenic aphasias. Disturbances_of_language_and_speech http://purl.obolibrary.org/obo/Disturbances_of_language_and_speech Disturbances of language and speech Disturbances:of:language:and:speech Sub-grouping variable containing signs and symptoms associated with disturbances of language and speech. These include aphasia, language impairment, word-finding, and communication. Definition: Aphasia (dysphasia) is the inability to comprehend or produce language (spoken or written). It often occurs following damage to particular brain regions following a trauma or injury to the head, vascular insult such as with cerebrovascular accident (CVA; stroke), or as a consequence of neurodegenerative diseases. All components of the language network are interconnected so deficits arising from injury will depend on the number and distribution of brain regions affected. Affected language aspects include word-finding, word choice, comprehension, spelling, or grammar. Aphasias caused by CVA are generally sudden onset and may be severe whereas aphasias caused by neurodegenerative diseases usually have an insidious onset with a steady and relentless progression. Hence, aphasia arising from neurodegenerative diseases are referred to as primary progressive aphasia (PPA) and can be classified as Agrammatic PPA, Semantic PPA or Logopenic PPA. The neurodegenerative disease most commonly associated with PPA include Alzheimer’s disease and frontotemporal dementias. Signs/symptoms of aphasia are generally only reported in the NBB reports in a subjective manner (i.e. only an example of an individual’s speech/language problem is provided) rather than an objective assessment. Therefore, it is not possible to classify disturbances in language, comprehension and communication as agrammatic, semantic or logopenic aphasias. https://accessmedicine.mhmedical.com/content.aspx?bookid=2129&sectionid=191734545 Mesulam MM. Aphasia, Memory Loss, Hemispatial Neglect, Frontal Syndromes, and Other Cerebral Disorders. In: Jameson JL, Fauci AS, Kasper DL, Hauser SL, Longo DL, Loscalzo J, eds. Harrison's Principles of Internal Medicine, 20e. New York, NY: McGraw-Hill Education; 2018. UMLS CUI: C0233791 Sub-grouping variable containing signs and symptoms associated with disturbances of memory Disturbances_of_memory http://purl.obolibrary.org/obo/Disturbances_of_memory Disturbances of memory Disturbances:of:memory In the NBB case reports, sometimes the type of gait disturbance is not specified, is non-specific, or cannot be objectively scored. Therefore, any gait disturbances that cannot be scored under one of our other parameters, can be scored here. We scored as TRUE: any gait pattern that cannot be scored as listed below. For example, we scored: gait impairment; disturbed gait pattern; gait disturbed; cautious gait; forward-leaning gait; any other disturbed gait pattern that cannot be scored as below. We also scored as TRUE: other infrequently mentioned or non-specific gait patterns: antalgic gait, myopathic gait; cautious gait; unsure gait. We did NOT Score: anything that could be scored as below: - Upper motor neuron signs: Spasticity: Here we score: spastic gait. - Bradykinesia: here we score freezing; freezing gait; shuffling gait. - Ataxia: here we score ataxic gait; atactical gait; atactic gait; wide-based gait; walked with a wide-base; broad-based gait. - Loss of coordination: here we score impaired tandem gait. - Balance problems: imbalanced gait; festinating gait. true UMLS: C3280869 Description: Gait and balance problems are a major contributor to falls and injury in the elderly and are may have a neurological cause19. When neurological examination has been performed, the gait is also observed. Whenever this is disturbed, it is described as some kind of disturbance in gait. Different abnormal gait patterns can arise due to different kind of pathology. A wide based gait, wide-based walk or broad-based gait often refers to a cerebellar origin (can also be described as atactic gait). Where a shuffling gait and freezing are often seen in patients with Parkinsonism or Parkinson’s disease. Spastic gait often presents after someone has suffered a stroke with a paresis of one leg, (origin of spastic gait in central motor system (pyramidal). Unspecified disturbed gait patterns Disturbed:gait Disturbed_gait Excluded due to poor model performance Disturbed gait Disturbed:gait UMLS: C3280869 Direct match We scored as TRUE: dysarthria; dysarthric (and any misspellings of these words); slurred speech; drunken speech; difficulties with articulation (of speech). We did NOT score: changes in voice e.g. hoarse voice; dysphonia; soft speech. These were not scored anywhere. We did NOT score: Speech apraxia – this was scored as ‘Apraxia’. We did NOT score: speech difficulties; slow speech. These were scored as ‘Language impairment (expressive)’. true UMLS: C0013362 Definition: “A general term applied when weakness or incoordination of the speech muscles prevents clear pronunciation of words. The voice sounds slurred or weak. It may be due to damage affecting the centers in the brain which control movements of the speech muscles, or damage to the muscles themselves. Examples of dysarthria may be found in strokes, cerebral palsy and the latter stages of parkinsonism, multiple sclerosis and motor neuron disease”. In the clinical summaries of the NBB, this was often described as part of the neurological examination. We scored it as a separate parameter, and not as part of ‘loss of coordination’ (which could also be a choice). Dysarthria http://purl.obolibrary.org/obo/Dysarthria Dysarthria Dysarthria UMLS: C0013362 Direct match Definition: “A general term applied when weakness or incoordination of the speech muscles prevents clear pronunciation of words. The voice sounds slurred or weak. It may be due to damage affecting the centers in the brain which control movements of the speech muscles, or damage to the muscles themselves. Examples of dysarthria may be found in strokes, cerebral palsy and the latter stages of parkinsonism, multiple sclerosis and motor neuron disease”. In the clinical summaries of the NBB, this was often described as part of the neurological examination. We scored it as a separate parameter, and not as part of ‘loss of coordination’ (which could also be a choice). Harvey Marcovitch M. Black's medical dictionary. Bloomsbury Information, an imprint of Bloomsbury P; 2017. https://libcat.simmons.edu/Record/b2265386/Details We took into consideration the multiple causes for balance problems and the limitations when we labeled NBB-numbers a TRUE for this parameter. We scored as TRUE: Balance problems; imbalance; disbalance; instability; postural instability; altered equilibrium. We scored as TRUE: any decrease, reduction, or impaired propulsion or retropulsion; impaired retropulsion test, positive pull test, impaired pull test; positive Romberg’s test/sign. We also scorde as TRUE: imbalanced gait; festinating gait. We did NOT score: tandem gait. This was scored as ‘Loss of coordination’. We did NOT score: anything else. CAUTION: Researchers have to be aware of the multiple causes for balance problems and the limitations /implications this has on interpretating the cases with ‘balance problems’. Combined with the clinical diagnosis it could have a better understanding. Postural instability is a typical description. Normally this is a separate symptom for Parkinson’s disease, but we were unable to score it separately because it is not reported enough. That is why we score it as ‘Balance Problems’. The tests to examine postural instability are also scored here. true UMLS: C0575090 Description: In the medical summaries of the NBB donors ‘Balance problems’ are often reported. This is a rather broad term, because balance problems can be caused by different systems and could also mean gait disorders which the patient reports as balance problems. Balance problems can be caused by gait disorders, who on their own are mostly multifactorial and have both neurological and non-neurologic components and have many manifestations. Under balance problems, we score only for imbalanced gait and festinating gait. However, most often balance problems are noted because the donors were specifically asked for it, because a neurodegenerative disorder was in the differential diagnosis. Postural instability — Postural instability is an impairment of centrally mediated postural reflexes that cause a feeling of imbalance and a tendency to fall with a significant risk of injury. Because postural instability usually does not appear until later in the course of PD, patients with parkinsonian signs who fall early in the course of the illness most likely have another parkinsonian syndrome, such as progressive supranuclear palsy or multiple system atrophy (MSA). Postural instability is tested clinically with the "pull" test, where the examiner stands behind the patient and firmly pulls the patient by the shoulders. Patients with normal postural reflexes should be able to maintain balance and retropulse (step backward) no more than one step. Patients with PD and postural instability, on the other hand, are likely to fall or take multiple steps backwards. Initially, a positive pull test may be the only sign of balance impairment. However, as postural instability progresses, the gait may show signs of festination. Once postural reflexes are lost, patients are generally wheelchair-bound. Among the primary motor features of PD, postural instability is the least responsive to dopaminergic therapies. In addition, postural instability and gait difficulty are major contributors to disability in patients with PD. Balance problems Equilibration:disorder Equilibration_disorder Equilibration disorder Equilibration:disorder UMLS: C0575090 Direct match Description: In the medical summaries of the NBB donors ‘Balance problems’ are often reported. This is a rather broad term, because balance problems can be caused by different systems and could also mean gait disorders which the patient reports as balance problems. Balance problems can be caused by gait disorders, who on their own are mostly multifactorial and have both neurological and non-neurologic components and have many manifestations. Under balance problems, we score only for imbalanced gait and festinating gait. However, most often balance problems are noted because the donors were specifically asked for it, because a neurodegenerative disorder was in the differential diagnosis. Postural instability — Postural instability is an impairment of centrally mediated postural reflexes that cause a feeling of imbalance and a tendency to fall with a significant risk of injury. Because postural instability usually does not appear until later in the course of PD, patients with parkinsonian signs who fall early in the course of the illness most likely have another parkinsonian syndrome, such as progressive supranuclear palsy or multiple system atrophy (MSA). Postural instability is tested clinically with the "pull" test, where the examiner stands behind the patient and firmly pulls the patient by the shoulders. Patients with normal postural reflexes should be able to maintain balance and retropulse (step backward) no more than one step. Patients with PD and postural instability, on the other hand, are likely to fall or take multiple steps backwards. Initially, a positive pull test may be the only sign of balance impairment. However, as postural instability progresses, the gait may show signs of festination. Once postural reflexes are lost, patients are generally wheelchair-bound. Among the primary motor features of PD, postural instability is the least responsive to dopaminergic therapies. In addition, postural instability and gait difficulty are major contributors to disability in patients with PD. Chou KL. Clinical manifestations of Parkinson disease. UpToDate Retrieved on. 2013;7:22. https://www.uptodate.com/contents/clinical-manifestations-of-parkinson-disease We only scored this parameter when it was explicitly written down as such in the summary. We scored as TRUE: executive problems; reduced executive function; executive dysfunction; dysexecutive syndrome. That is, we only scored when the word executive was present. We did NOT score: disturbances in the following specific functions – clock-drawing; complex drawing tasks, mental inflexibility. Although other areas are tested as part of executive function, they are seldom reported and are more difficult to interpret. These were NOT scored anywhere. true UMLS: C2748208 The clinical interview provides an overview of the patient's executive function, particularly if reported difficulties in the performance of everyday activities appear to greatly exceed deficits on other cognitive assessments. Impaired insight and judgment are early indicators of executive dysfunction; these can also be best assessed in the clinical history (e.g. "What problems do you think you are having?"; "How would you handle a minor household emergency, such as a small plumbing leak?"). In the clinical summaries of the NBB, executive dysfunction or executive function problems are often written down just like that, without specifications. We are dependent on the medical information we receive from specialists and general practitioners, and in a lot of cases, we only have a conclusional statement that the “executive functions are impaired’ without the results of test that were conducted to come to this conclusion. Executive function disorder Executive:dysfunction Executive_dysfunction Executive dysfunction Executive:dysfunction UMLS: C2748208 Direct match The clinical interview provides an overview of the patient's executive function, particularly if reported difficulties in the performance of everyday activities appear to greatly exceed deficits on other cognitive assessments. Impaired insight and judgment are early indicators of executive dysfunction; these can also be best assessed in the clinical history (e.g. "What problems do you think you are having?"; "How would you handle a minor household emergency, such as a small plumbing leak?"). In the clinical summaries of the NBB, executive dysfunction or executive function problems are often written down just like that, without specifications. We are dependent on the medical information we receive from specialists and general practitioners, and in a lot of cases, we only have a conclusional statement that the “executive functions are impaired’ without the results of test that were conducted to come to this conclusion. Mendez MF. The mental status examination in adults. UpToDate. https://www.uptodate.com/contents/the-mental-status-examination-in-adults?search=neuropsychological%20examination&source=search_result&selectedTitle=1~150&usage_type=default&display_rank=1#H28388635. Published 2021. Accessed 9 June, 2021. NOTE: ‘reduced mimic’ means reduced facial expression arising from a too literal translation of Dutch to English. This can be scored as TRUE for ‘Facial masking'. This is often described together with reduced/decreased eye-blink. We scored as TRUE: masked face; mask-like face; altered mimic, hypomimia, masked facies, masking of facies, mask-like facial expression. We scored other remarks as TRUE: for example: ‘patient’s face was like mask’. We did NOT score: reduced eye-blink/decreased spontaneous eye blink rate (this is a different/separate craniofacial motor feature). true UMLS: C0813217 Description: Next to the cardinal features of Parkinson’s disease (tremor, bradykinesia and rigidity), other motor features are seen in different areas, like craniofacial, visual, musculoskeletal and in gait. Hypomimia, or facial masking, is one of the craniofacial features, is often present in patients with Parkinson's disease and is frequently named, so we decided to score this separately. Occasionally it can be described in other neurodegenerative diseases, like MSA-type P for example. Facial masking Expressionless:face Expressionless_face Expressionless face Expressionless:face UMLS: C0813217 Direct match Description: Next to the cardinal features of Parkinson’s disease (tremor, bradykinesia and rigidity), other motor features are seen in different areas, like craniofacial, visual, musculoskeletal and in gait. Hypomimia, or facial masking, is one of the craniofacial features, is often present in patients with Parkinson's disease and is frequently named, so we decided to score this separately. Occasionally it can be described in other neurodegenerative diseases, like MSA-type P for example. Chou KL. Clinical manifestations of Parkinson disease. UpToDate Retrieved on. 2013;7:22. https://www.uptodate.com/contents/clinical-manifestations-of-parkinson-disease Grouping variable containing extrapyramidal signs. Extrapyramidal:symptoms Extrapyramidal_symptoms Extrapyramidal symptoms Extrapyramidal:symptoms We scored as TRUE: Façade/facade behavior. We scored as TRUE: for example, ‘patients' memory was poor, but she camouflaged it well’; ‘patient didn't admit that she was ill’. We did NOT score: any other memory problems here. false UMLS: C0234507 Description: This is where a patient pretends to know who people or situations are, but they really do not remember. This sometimes occurs in patients with dementia. This can require some interpretation of the sentences (see examples provided) and is subtly different from ‘lack of insight’ so use your best judgement. If unsure, do NOT score. Facade:behavior Facade_behavior Façade behavior Facade:behavior UMLS: C0234507 UMLS code refers to a broader concept We scored as TRUE: fatigue; tired; tiredness; fatigued; general malaise; weariness; drowsy; exhausted. We did NOT score: sleepiness; slept a lot. These were scored separately as ‘Sleep disturbances’. false UMLS: C0015672 Description: Any notes about fatigue, tired, tiredness, fatigued, general malaise (not including nausea), weariness, drowsy or other synonyms for tired, except sleepiness. Fatigue http://purl.obolibrary.org/obo/Fatigue Fatigue Fatigue UMLS: C0015672 Direct match We score as TRUE: suicidal ideation; suicide attempts; suicidal; suicide; patient jumped in front of a train; found with cord around his/her neck. We also score as TRUE: patient had a death wish; also when it states suicidal ideation decreased as this suggests it is still present but to a lesser extent. We do NOT score: ‘suicidal ideation was no longer an issue’, for example. false UMLS: C0424000 Definition: “Suicidal ideations (SI), often called suicidal thoughts or ideas, is a broad term used to describe a range of contemplations, wishes, and preoccupations with death and suicide”. We include suicidal ideation and suicide attempts. Suicidal ideation Feeling:suicidal Feeling_suicidal Feeling suicidal Feeling:suicidal UMLS: C0424000 Direct match Definition: “Suicidal ideations (SI), often called suicidal thoughts or ideas, is a broad term used to describe a range of contemplations, wishes, and preoccupations with death and suicide”. We include suicidal ideation and suicide attempts. American Psychiatric Association. Diagnostic and statistical manual of mental disorders, Fifth Edition. Vol 21. Arlington, VA.: American Psychiatric Association; 2013. Harmer B, Lee S, Duong TVH, Saadabadi A. Suicidal Ideation. In: StatPearls. Treasure Island (FL): StatPearls Publishing. Copyright © 2021, StatPearls Publishing LLC.; 2021. https://www.ncbi.nlm.nih.gov/books/NBK565877/ Sub-grouping variable containing signs/symptoms/findings indicative of sleep disturbances. Findings_related_to_sleep http://purl.obolibrary.org/obo/Findings_related_to_sleep Findings related to sleep Findings:related:to:sleep We scored as TRUE: Forgetfulness; was forgetful; forgot things; did not remember things. We did NOT score: memory impairment; amnesia. These were scored separately as ‘Memory impairment’ or ‘Amnesia’. true UMLS: C0542476 Description: Forgetfulness can occur as a process of normal aging or can be due to a known neurological damage or disease. Ordinarily, we could score forgetfulness as amnesia or memory impairment. Due to the subjective nature of the clinical case studies, we have decided to score this parameter separately. Forgetfulness Forgetful http://purl.obolibrary.org/obo/Forgetful Forgetful Forgetful UMLS: C0542476 Direct match In the clinical summaries of the NBB, it was most frequently written down as ‘frequent falls’ or ‘frequent falling’, ‘fell frequently’ or ‘fell multiple times’. We scored as TRUE: frequent falls; frequent falling; sometimes fell; increased falling. We did NOT score: phrases needing interpretation e.g. falling that occurs more than 2X in a short space of time for; ‘Fell three times last year’, tendency to fall. In this case, you should use your best judgement. Explanation: Researchers must be aware that “frequent falls” described in the clinical summaries of the NBB, could be caused by different problems. Some are more balance induced, some more rigidity induced. true UMLS:C0850703 Description: Frequent falls is asked for when neurologists suspect multiple system atrophy. Usually within three years of motor onset. but can also be caused by postural instability, for example in Parkinson’s disease. Many patients with a neurodegenerative disease that have progressed to a more severe stage, start to get difficulty maintaining balance/coordination/strength, and start to report frequent falling. Frequent:falls Frequent_falls Frequent falls Frequent:falls UMLS:C0850703 Direct match We scored as TRUE: (exaggerated) snout reflex; glabellar reflex; glabellar tap sign; palmomental reflex; grasp reflex; gripping reflex; sucking reflex. We did NOT score: hyperreflexia; Babinski sign; Hoffman’s reflex; exaggerated deep tendon reflexes (DTRs); clonus; clonic jaw jerk; extensor plantar response. These were scored as ‘Upper motor neuron signs – hyperreflexia and other pathological reflexes’. true UMLS: C1833297 Primitive reflexes are typically present in childhood and suppressed during normal development. However, they may reappear in the presence of neurological disease, particularly those affecting the frontal lobes. Frontal release signs are often reported in in the NBB cases and are reported here. Primitive reflexes Frontal_release_signs http://purl.obolibrary.org/obo/Frontal_release_signs Excluded due to poor model performance Frontal release signs Frontal:release:signs UMLS: C1833297 Direct match Primitive reflexes are typically present in childhood and suppressed during normal development. However, they may reappear in the presence of neurological disease, particularly those affecting the frontal lobes. Frontal release signs are often reported in in the NBB cases and are reported here. https://www.ncbi.nlm.nih.gov/books/NBK554606/ https://www.ncbi.nlm.nih.gov/nlmcatalog/101736009 Mesulam MM. Aphasia, Memory Loss, Hemispatial Neglect, Frontal Syndromes, and Other Cerebral Disorders. In: Jameson JL, Fauci AS, Kasper DL, Hauser SL, Longo DL, Loscalzo J, eds. Harrison's Principles of Internal Medicine, 20e. New York, NY: McGraw-Hill Education; 2018. Domain variable containing signs/symptoms/findings associated with general disturbances. General http://purl.obolibrary.org/obo/General General General Grouping variable containing signs/symptoms/findings indicative of general decline in health. General:decline General_decline General decline General:decline We scored as TRUE: deteriorated or declined health or condition; (general) condition declined/deteriorated; progression of symptoms; progression of disease; disease progressed. NOTE: if the text refers specifically to “declined cognition” it was scored as ‘Cognitive decline’. false UMLS: C0563273 Description: In the clinical summaries of the NBB it is often described that a patient’s condition declined, deteriorated, or worsened. Most frequently this considers the condition regarding the neurodegenerative disease. Whenever a patient’s situation, health, physical conditions generally change, we want to score it. Declined or deteriorated health General_health_deterioration http://purl.obolibrary.org/obo/General_health_deterioration General health deterioration General:health:deterioration UMLS: C0563273 Direct match In the clinical summaries of the NBB, the type of hallucination is not always specified. It often describes that a donor suffered from ‘hallucinations’ in general. When specified, most frequently it is written down as ‘acoustic/auditory’ or ‘visual’. We label all hallucinations as TRUE. We also include patients with hallucinations which they were aware of. The machine learning will not be able to learn the difference. We scored as TRUE: visual, acoustic, auditory, and sensory hallucinations, or whatever is written down regarding hallucinations. We also scored as TRUE: descriptions of hallucinations e.g. ' She also seemed to see things which did not exist'; ‘he had trouble sleeping because he saw things that were not there'; hearing voices. NOTE: use your best judgement with these sentences. If in doubt, do NOT score. We did NOT score: for example, items that are ambiguous. false UMLS: C0018524 Definition: “Hallucinations involve sensing things such as visions, sounds, or smells that seem real but are not. These things are created by the mind”.46 Hallucinations http://purl.obolibrary.org/obo/Hallucinations Hallucinations Hallucinations UMLS: C0018524 Direct match We scored any instance of headache or migraine. Anywhere the word ‘headache’ or ‘migraine’ was written down, it was marked as TRUE. We scored as TRUE: (chronic) headache; cluster headache; migraine. false UMLS: C0018681 Definition: headache is pain arising from the head or upper neck and is one of the most commonly reported medical complaints. There are multiple classifications for headaches, most of which fall under episodic tension-type headaches, migraine and cluster headaches. In most cases these are not specified in the case notes so we cannot distinguish between them. Headache / migraine Headache http://purl.obolibrary.org/obo/Headache Headache Headache UMLS: C0018681 Direct match Definition: headache is pain arising from the head or upper neck and is one of the most commonly reported medical complaints. There are multiple classifications for headaches, most of which fall under episodic tension-type headaches, migraine and cluster headaches. In most cases these are not specified in the case notes so we cannot distinguish between them. https://www.medilib.ir/uptodate/show/3349 We scored as TRUE: head-turning sign. We did NOT score: anything else. true UMLS: NA Definition: Head-turning sign or a positive head-turning sign, is an observation during neurological examination, where the patient frequently “turns his/her head” towards the partner/family member joining the patient to the consult, as a sign that they are constantly asking for reassurance of their answer, or they do not know the answer to a question. For a simple question patient’s turn their head to their caregiver/partner, indicating cognitive problems. Headturning:sign Headturning_sign Excluded due to poor model performance Headturning sign Headturning:sign We scored as TRUE: hearing loss; decreased hearing; presb(a)ycusis; changes in the audiogram; required a hearing aid; deaf(ness). Note: presbycusis is a diagnosis. This is one of the only clinical parameters where we scored a Diagnosis as a clinical parameter. We did NOT score: tinnitus; ringing in the ears; ear infections/inflammation. false UMLS: C0260662 Definition: Presbycusis, or age-related hearing loss, is a common cause of hearing loss in adults worldwide. Here we want to encompass: hearing loss, decreased hearing, presbycusis. Hearing loss is frequently present in the elderly patient, which is the main group of donors in the NBB. We think it is valuable to know, because it can have an influence on the disease course of patients with a neurodegenerative disorder. Hearing problems Hearing:problem Hearing_problem Hearing problem Hearing:problem UMLS: C0260662 Direct match We scored as TRUE: hyperorality; put strange things in their mouth; binge eating. We scored as TRUE: excessive alcohol consumption; excessive cigarette smoking. We did NOT score: alcohol abuse. Unless the alcohol abuse is clearly present as a result of developed hyperorality. true UMLS: C1838320 Definition: hyperorality is the tendency or compulsion to examine objects by mouth and can be manifested as binge eating, cravings for sweet foods, attempting to consume inedible objects, or putting excessive amounts of food in the mouth. It can also manifest as increased consumption of alcohol and tobacco. Hyperorality is a common symptom of the behavioral variant of frontotemporal dementia. Hyperorality http://purl.obolibrary.org/obo/Hyperorality Hyperorality Hyperorality UMLS: C1838320 Direct match Definition: hyperorality is the tendency or compulsion to examine objects by mouth and can be manifested as binge eating, cravings for sweet foods, attempting to consume inedible objects, or putting excessive amounts of food in the mouth. It can also manifest as increased consumption of alcohol and tobacco. Hyperorality is a common symptom of the behavioral variant of frontotemporal dementia. https://pubmed.ncbi.nlm.nih.gov/21810890/ Lee SE. Frontotemporal dementia: Clinical features and diagnosis. UpToDate. https://www.uptodate.com/contents/frontotemporal-dementia-clinical-features-and-diagnosis. Published 2020. Accessed 9 June, 2021. We scored as TRUE: hyperreflexia; Babinski sign; Strumpell’s sign; Hoffman’s reflex; exaggerated deep tendon reflexes (DTRs); clonus; clonic jaw jerk; extensor plantar response; positive plantar reflex; reflexes noted as 2+ or above. We did NOT score: when reflexes are absent; increased muscle tone. These were not scored anywhere. We did NOT score: (exaggerated) snout reflex here. This was scored as ‘Frontal release signs’. true UMLS: C0034935 UMLS: C0151889 UMLS: C0576598 Other important upper motor neuron signs can be measured during neurological examination and can be scored here. Symptoms include exaggerated deep tendon reflexes and clonus (a series of rapid muscle contractions). Hyperreflexia Hyperreflexia_and_other_pathological_reflexes http://purl.obolibrary.org/obo/Hyperreflexia_and_other_pathological_reflexes Hyperreflexia and other pathological reflexes Hyperreflexia:and:other:pathological:reflexes UMLS: C0034935 UMLS code refers to a narrower concept UMLS: C0151889 UMLS code refers to a narrower concept UMLS: C0576598 UMLS code refers to a narrower concept Other important upper motor neuron signs can be measured during neurological examination and can be scored here. Symptoms include exaggerated deep tendon reflexes and clonus (a series of rapid muscle contractions). https://pubmed.ncbi.nlm.nih.gov/11464847/ McGee S. Chapter 61 - Examination of the Motor System: Approach to Weakness. In. Fourth Edition ed: Elsevier Inc; 2018:551-568.e552. We scored as TRUE: orthostatic hypotension; orthostasis. We did NOT score: blurred vision. This was scored as ‘Visual problems’. We did NOT score: syncope; dizziness; dizzy; collapse; vasovagal problems. These were NOT scored anywhere. Explanation: As syncope and dizziness can be symptoms from orthostatic hypotension, but also caused by multiple other problems/pathology, we have decided to leave these words out of the scoring. In the clinical summaries of the NBB, ‘dizziness’ and ‘syncope’ are mentioned frequently. However, where orthostatic hypotension can present symptoms of dizziness and syncope, ‘dizziness’ and ‘syncope’ on their own do not necessarily mean that the patient suffered from orthostatic hypotension. These symptoms can also be caused by other pathology/problems. false UMLS: C0020651 Definition: “A sudden fall in blood pressure that occurs when a person assumes a standing position”. Orthostatic hypotension is a finding (sign). Symptoms generally include dizziness, blurred vision, and syncope. Orthostatic hypotension Hypotension:orthostatic Hypotension_orthostatic Hypotension, orthostatic Hypotension:orthostatic UMLS: C0020651 Direct match Definition: “A sudden fall in blood pressure that occurs when a person assumes a standing position”. Orthostatic hypotension is a finding (sign). Symptoms generally include dizziness, blurred vision, and syncope. National Institute of Neurological Disorders and Stroke (NINDS). Orthostatic hypotension information page. NIH. https://www.ninds.nih.gov/Disorders/All-Disorders/Orthostatic-Hypotension-Information-Page. Published 2019. Accessed 9 June, 2021. We scored as TRUE: all walking difficulties that cannot be more objectively scored. For example, walking problems, ‘walking leaning to one side’; mobility impairment; problems with mobility; mobility problems. We scored as TRUE: walking with a walking aid; any mention of wheelchair; leaning walk (leaning in a particular direction when walking); unstable walk; required help/assistance with walking. We did NOT score: reduced arm swing or starting problems– these are scored separately as ‘Bradykinesia'. Freezing or freezing gait pattern are also scored separately as ‘Bradykinesia’. We did not score anything else. No interpretations of sentences. We did NOT score: reduced propulsion/impaired propulsion and impaired retropulsion – these were scored as ‘Balance problems’. We did NOT score: any items that can be scored under ‘Decreased fine motor skills’. Explanation: We scored this parameter to show when a donor started to get difficulties with mobilization due to an underlying disease to indicate the impact on life/general functioning. Researchers must be aware that the cause of the mobility problems can be a variety of things, depending on the diagnoses and other clinical parameters present. true UMLS: C0518456 Description: Here we want to capture disturbances in mobility and walking difficulties as well as motor skill disturbances that cannot be objectively scored as motor skills or anywhere else. Mobility problems Impaired:mobility Impaired_mobility Impaired mobility Impaired:mobility UMLS: C0518456 Direct match In some cases, it will require your best judgement. If in doubt, do NOT score. We scored as TRUE: loss of initiative; lack of initiative; lost initiative. We did NOT score: any ambiguous sentence, for example, ‘patient hardly spoke or did anything’. These were not scored anywhere. We did NOT score: Self-negligence; and loss of personal hygiene. These are scored as ‘Socially inappropriate behavior’. We did NOT score: loss or lack of insight. These were scored separately as ‘Lack of insight’. We did NOT score: loss or lack of interest. These were scored separately as ‘Apathy’. true UMLS: C2364087 Lack of initiative (or inertia) is often assessed as a sub-component of apathy assessments. Since it is very frequently mentioned separately in our case studies, we have decided to score it separately. Actual Low Initiative inertia Lack_of_initiative http://purl.obolibrary.org/obo/Lack_of_initiative Lack of initiative Lack:of:initiative UMLS: C2364087 Direct match Lack of initiative (or inertia) is often assessed as a sub-component of apathy assessments. Since it is very frequently mentioned separately in our case studies, we have decided to score it separately. https://pubmed.ncbi.nlm.nih.gov/12461757/ We scored as TRUE: any instance describing: problems with planning; organization; disturbed judgement; and loss of overview; problem-solving difficulties. true UMLS: C0423906 UMLS: C0517891 Description: The ability to plan is something that is captured in testing the Executive functioning. In the clinical summaries of the NBB, this was frequently mentioned on its own as a conclusion after neuropsychological examination, after an interview with the patient or a family-member or partner (next of kin). Here we want to capture impairments in planning, organization or overview that could not be more objectively scored as ‘Executive dysfunction’. Difficulty in planning Difficulty solving problems Lack_of_planning_organization_overview http://purl.obolibrary.org/obo/Lack_of_planning_organization_overview Lack of planning / organization / overview Lack:of:planning:organization:overview UMLS: C0423906 UMLS code refers to a narrower concept UMLS: C0517891 UMLS code refers to a narrower concept We scored as TRUE: any words relating to the word comprehend; reasoning. We also scored as TRUE: difficulties with reading and writing; neologisms. We also scored as TRUE: lack of understanding. We did NOT score: learning; ‘was not able to learn’ new things; perception. These were not scored anywhere. We did NOT score: any parameter that could be scored under a more specific parameter. PLEASE NOTE: Patients with items scored under this parameter may fit a classification of ‘Comprehensive aphasia’. However, given the subjective descriptions provided in the NBB reports, we are unable to accurately classify the different forms of aphasia. Furthermore, we cannot make any distinction between primary progressive aphasias and other forms of aphasias. Therefore, interpretation should be made with caution. true UMLS: C5436214 Description: Ordinarily, difficulties in comprehension would be encompassed under ‘language impairment’ or ‘dysphasia’ or possibly even ‘decreased cognition’. However, due to the subjective nature of our clinical case studies, we have decided to score impaired comprehension separately to enable more objective scoring of the changes seen in different functions. People with neurocognitive orders often have impairments in language expression described as ‘Comprehensive aphasia (semantic)’. People with this pattern of aphasia may speak easily and fluently in long, complex sentences that don't make sense or include unrecognizable, incorrect or unnecessary words. They usually don't understand spoken language well and often don't realize that others can't understand them. A similar type of asphasia (fluent or Wernicke’s) aphasia can be observed in some people following a CVA. We cannot distinguish between primary progressive aphasias from other forms of aphasia. Language impairment (comprehension) Limited_language_comprehension http://purl.obolibrary.org/obo/Limited_language_comprehension Excluded due to poor model performance. Limited language comprehension Limited:language:comprehension UMLS: C5436214 Direct match Description: Ordinarily, difficulties in comprehension would be encompassed under ‘language impairment’ or ‘dysphasia’ or possibly even ‘decreased cognition’. However, due to the subjective nature of our clinical case studies, we have decided to score impaired comprehension separately to enable more objective scoring of the changes seen in different functions. People with neurocognitive orders often have impairments in language expression described as ‘Comprehensive aphasia (semantic)’. People with this pattern of aphasia may speak easily and fluently in long, complex sentences that don't make sense or include unrecognizable, incorrect or unnecessary words. They usually don't understand spoken language well and often don't realize that others can't understand them. A similar type of asphasia (fluent or Wernicke’s) aphasia can be observed in some people following a CVA. We cannot distinguish between primary progressive aphasias from other forms of aphasia. https://accessmedicine.mhmedical.com/content.aspx?bookid=2129&sectionid=191734545 Mayo Clinic. Aphasia. Mayo Clinic. https://www.mayoclinic.org/diseases-conditions/aphasia/symptoms-causes/syc-20369518. Published 2021. Accessed 11 August 2021. Mesulam MM. Aphasia, Memory Loss, Hemispatial Neglect, Frontal Syndromes, and Other Cerebral Disorders. In: Jameson JL, Fauci AS, Kasper DL, Hauser SL, Longo DL, Loscalzo J, eds. Harrison's Principles of Internal Medicine, 20e. New York, NY: McGraw-Hill Education; 2018. We scored as TRUE: anywhere stating there has been difficult or changed or loss of coordination and which do not fit under other parameters. For example, '...severe coordination problems'. We also scored as TRUE: difficulties with coordination; could not perform tightrope walking/walking straight line; impaired tandem gait test; positive Romberg test; dysdiadochokinesis; diadochokinesis impaired; finger-tapping, rapid alternating movements; finger-to-nose testing; heel-to-shin testing. We did NOT score: ataxic gait; atactic gait. These were scored as ‘Ataxia'. We did NOT score: imbalanced gait. This was scored as ‘Balance problems'. Any other gait problems which are scored elsewhere. Explanation: Researchers must be aware that coordination problems scored in this parameter, can be caused by cerebellar, pyramidal, or extrapyramidal disorders. Depending on the clinical diagnosis, more clarity on the origin of the loss of coordination can be suggested. true UMLS: C2751891 Definition: “Coordination disorders often result from malfunction of the cerebellum, the part of the brain that coordinates voluntary movements and controls balance”.18 Anything that damages the cerebellum can lead to loss of coordination (ataxia). However, many other disorders can also cause loss of coordination. Here we want to capture any reduction or loss of coordination that cannot be objectively scored under one of the other parameters. Dysdiadochokinesis means the inability to execute fast alternating movements. In the neurological examination it is tested by letting the patient rapidly alternate pronation and supination movements of his hands or tapping his foot on the floor as fast as possible. When this is poorly executed or impaired, this is called dysdiadochokinesis, and is a sign of loss of coordination. Dysdiadochokinesis can be present in cerebellar, pyramidal, and extrapyramidal disorders. In the neurological examination, coordination testing is often referred to as cerebellar testing, but this is a misnomer. Although the cerebellum is very important in the production of coordinated movements and particular abnormal findings on coordination testing may suggest cerebellar disease, other systems also play critical roles. As an example, severe arm weakness will prevent a patient from performing finger-to-nose testing even though the cerebellum and its pathways may be intact.10 Finger-tapping, Rapid alternating movements, Finger-to-nose testing and heel-to-shin testing are all parts of the neurological examination to test the coordination. In the clinical summaries of the NBB, these findings are only occasionally reported. Therefore, we decided to leave them out of the scoring. Loss_of_coordination http://purl.obolibrary.org/obo/Loss_of_coordination Loss of coordination Loss:of:coordination UMLS: C2751891 Direct match We scored as TRUE: loss/lack of sympathy; loss/lack of empathy; no emotion true UMLS: C0564548 Definition: Empathy is an emotional response which involves the ability to understand what others feel (cognitive and affective empathy, respectively). The ability to empathize with others can be impaired in certain neurodegenerative disorders, particularly the behavioral variant of frontotemporal dementia. Callous character Loss_of_sympathy_empathy http://purl.obolibrary.org/obo/Loss_of_sympathy_empathy Excluded due to poor model performance Loss of sympathy / empathy Loss:of:sympathy:empathy UMLS: C0564548 UMLS code refers to a broader concept Definition: Empathy is an emotional response which involves the ability to understand what others feel (cognitive and affective empathy, respectively). The ability to empathize with others can be impaired in certain neurodegenerative disorders, particularly the behavioral variant of frontotemporal dementia. https://pubmed.ncbi.nlm.nih.gov/21810890/ Lee SE. Frontotemporal dementia: Clinical features and diagnosis. UpToDate. https://www.uptodate.com/contents/frontotemporal-dementia-clinical-features-and-diagnosis. Published 2020. Accessed 9 June, 2021. Whenever hypomania or mania is written down, we labeled it as TRUE. We scored as TRUE: mania, manic, hypomania, bipolar. When the root word coming from mania was written down. We did NOT score: any other terms unless very clearly specific for mania. false UMLS: C0338831 Definition: Manic episodes involve clinically significant changes in mood, energy, activity, behavior, sleep, and cognition. Period of extreme high energy or mood associated with bipolar disorder. Mania Manic http://purl.obolibrary.org/obo/Manic Manic Manic UMLS: C0338831 Direct match Definition: Manic episodes involve clinically significant changes in mood, energy, activity, behavior, sleep, and cognition. Period of extreme high energy or mood associated with bipolar disorder. American Psychiatric Association. Diagnostic and statistical manual of mental disorders, Fifth Edition. Vol 21. Arlington, VA.: American Psychiatric Association; 2013. Goodwin FK, Jamison KR. Manic-depressive illness: bipolar disorders and recurrent depression. Vol 2: Oxford University Press; 2007. We scored as TRUE: reduced oral intake; reduced fluid intake; patient had reduced food intake; ceased oral intake; dehydration. false UMLS: C0948353 Definition: “With increasing age appetite declines and food consumption declines. Healthy older people are less hungry and are fuller before meals, consume smaller meals, eat more slowly, have fewer snacks between meals and become satiated after meals more rapidly after eating a standard meal than younger people. The average daily intake of food decreases by up to 30% between 20 and 80 years. Most of the age-related decrease in energy is a response to the decline in energy expenditure with age. However in many older people the decrease in energy intake is greater than the decrease in energy expenditure, and therefore body weight is lost”. Reduced oral intake Markedly_reduced_dietary_intake http://purl.obolibrary.org/obo/Markedly_reduced_dietary_intake Markedly reduced dietary intake Markedly:reduced:dietary:intake UMLS: C0948353 Direct match Definition: “With increasing age appetite declines and food consumption declines. Healthy older people are less hungry and are fuller before meals, consume smaller meals, eat more slowly, have fewer snacks between meals and become satiated after meals more rapidly after eating a standard meal than younger people. The average daily intake of food decreases by up to 30% between 20 and 80 years. Most of the age-related decrease in energy is a response to the decline in energy expenditure with age. However in many older people the decrease in energy intake is greater than the decrease in energy expenditure, and therefore body weight is lost”. https://www.ncbi.nlm.nih.gov/pmc/articles/PMC2920201/ Whenever the clinical summary mentions short term memory problems, long-term memory problems, or just ’memory problems’, we score this parameter. We scored as TRUE: memory problems; memory impairment; any difficulties with recall (when specific word ‘recall’ was used). We scored as TRUE: memory for … was impaired; Impaired (short-term) memory; memory declined; memory deteriorated. We did NOT score: forgetfulness. This was scored separately as ‘Forgetful’. We did NOT score: amnesia; amnestic; amnestically; amnesic. These were scored separately as ‘Amnesia’. We did NOT score: confabulations; confabulated. These were scored separately as ‘Confabulations'. true UMLS: C0233794 Definition: “Memory is the capacity to retain information and utilize it for adaptive purposes. Memory loss is an unexpected or unexplained decrease in ability to recall previously learned information”. Ordinarily, we could also score ‘amnesia’ as memory impairment. However, due to the subjective nature of the clinical histories, we have decided to score these separately. Memory:impairment Memory_impairment Memory impairment Memory:impairment UMLS: C0233794 Direct match Definition: “Memory is the capacity to retain information and utilize it for adaptive purposes. Memory loss is an unexpected or unexplained decrease in ability to recall previously learned information”. Ordinarily, we could also score ‘amnesia’ as memory impairment. However, due to the subjective nature of the clinical histories, we have decided to score these separately. https://link.springer.com/referencework/10.1007/978-0-387-79948-3 Flaada JT. Memory Impairment. In: Kreutzer JS, DeLuca J, Caplan B, eds. Encyclopedia of Clinical Neuropsychology. New York, NY: Springer New York; 2011:1563-1565. The following terms occured infrequently or were too vague or uninformative so we have decided that they should NOT be scored. We did NOT score the following terms: - Increased muscle tone ( too infrequent ) - Decreased reflexes ( too infrequent ) - Learning; ‘was not able to learn new things’ ( too ambiguous ) - Dizziness; dizzy ( too uninformative ) - Syncope ( too infrequent ) - Vasovagal response ( too infrequent ) - Bradycardia ( too infrequent ) - Change in consciousness ( too ambiguous ) - Coma ( too infrequent ) - Collapse ( too ambiguous ) - Clock drawing test; difficulties with complex drawing tasks ( too infrequent ) - Bedridden ( too uninformative ) - Motor restlessness ( too ambiguous ) - Restless legs ( scored as clinical diagnosis ) - Changed appetite ( too infrequent ) - Voice disorders ( too ambiguous ) - Irritability ( too ambiguous ) - Anger ( too infrequent ) - Frustration ( too ambiguous ) - Tension ( too ambiguous ) - Clapping behavior; clapping test ( too infrequent ) - Retinopathy ( scored as clinical diagnosis ) - Cataract; ( scored as clinical diagnosis ) - Eye surgeries ( too uninformative ) - Diplopia; double vision ( too infrequent ) - Strabismus divergens ( scored as clinical diagnosis ) - Visual perceptual or visual spatial problems ( too infrequent ) - Decreased urine production due to renal insufficiency or renal failure ( too infrequent ) - Urinary tract infection ( scored as clinical diagnosis ) - Autonomic failure ( too infrequent ) - Erectile dysfunction ( scored as clinical diagnosis ) Miscellaneous:items Miscellaneous_items Miscellaneous items Miscellaneous:items Domain variable containing signs/symptoms/findings associated with motoric disturbances. Motor http://purl.obolibrary.org/obo/Motor Motor Motor Grouping variable containing signs and symptoms related to motor deficits. Motor:deficits Motor_deficits Motor deficits Motor:deficits We scored as TRUE: Rigidity or cog wheel phenomenon; hypokinetic rigid syndrome*; general stiffness**; plastic tone; plastic hypertonia; plastic hypertonus. Note: hypokinetic rigid syndrome was also scored as ‘Bradykinesia’. Note: When scoring stiffness as TRUE, we only scored if clearly the whole body is affected, otherwise it was NOT scored. We did NOT score: hypertonia or stiffness in a single muscle or body part. We did NOT score: freezing or freezing of gait. These were scored as ‘Bradykinesia’. true UMLS: C0026837 Definition: Rigidity is a severe state of hypertonia where muscle resistance occurs throughout the entire range of motion of the affected joint independent of velocity. It is frequently associated with lesions of the basal ganglia. In Neurological examination the cog wheel phenomenon indicates rigidity. In the clinical summaries of the NBB, rigidity is frequently reported in cases with Parkinson’s disease or Parkinson plus syndromes. Rigidity Muscle:rigidity Muscle_rigidity Muscle rigidity Muscle:rigidity UMLS: C0026837 Direct match Definition: Rigidity is a severe state of hypertonia where muscle resistance occurs throughout the entire range of motion of the affected joint independent of velocity. It is frequently associated with lesions of the basal ganglia. In Neurological examination the cog wheel phenomenon indicates rigidity. In the clinical summaries of the NBB, rigidity is frequently reported in cases with Parkinson’s disease or Parkinson plus syndromes. Chou KL. Clinical manifestations of Parkinson disease. UpToDate Retrieved on. 2013;7:22. https://www.uptodate.com/contents/clinical-manifestations-of-parkinson-disease We scored as TRUE: spasticity; spastic; spastic gait; spastic tone; spastic hypertonia; spastic hypertonus; spastic ataxia. Where the root word spastic is written down, and words that derive from that and mean that the patient suffered from spasticity. We also scored as TRUE: contractures; contracted posture of a limb; muscle spasms. We did NOT score: hypertonia; hypertonus; described exaggerated reflexes - these were scored in ‘Upper motor neuron signs – hyperreflexia and other pathological reflexes’; any descriptions that only imply spasticity. We did not make any interpretations. false UMLS: C0026838 Definition: Spasticity is a condition in which there is an abnormal increase in muscle tone or stiffness of muscle, which might interfere with movement, speech, or be associated with discomfort or pain. Spasticity is usually caused by damage to nerve pathways within the brain or spinal cord that control muscle movement. It may occur in association with spinal cord injury, multiple sclerosis, cerebral palsy, stroke, brain or head trauma, amyotrophic lateral sclerosis, hereditary spastic paraplegias, and metabolic diseases such as adrenoleukodystrophy, phenylketonuria, and Krabbe disease. The degree of spasticity varies from mild muscle stiffness to severe, painful, and uncontrollable muscle spasms. Spasticity can interfere with rehabilitation in patients with certain disorders, and often interferes with daily activities. Symptoms may include hypertonicity (increased muscle tone), muscle spasms, scissoring (involuntary crossing of the legs), and fixed joints (contractures). Spastic gait often presents after someone has suffered a stroke with a paresis of one leg, (origin of spastic gait in central motor system (pyramidal)). This is also scored here. Spasticity Upper motor neuron signs Muscle:spasticity Muscle_spasticity Muscle spasticity Muscle:spasticity UMLS: C0026838 Direct match Definition: Spasticity is a condition in which there is an abnormal increase in muscle tone or stiffness of muscle, which might interfere with movement, speech, or be associated with discomfort or pain. Spasticity is usually caused by damage to nerve pathways within the brain or spinal cord that control muscle movement. It may occur in association with spinal cord injury, multiple sclerosis, cerebral palsy, stroke, brain or head trauma, amyotrophic lateral sclerosis, hereditary spastic paraplegias, and metabolic diseases such as adrenoleukodystrophy, phenylketonuria, and Krabbe disease. The degree of spasticity varies from mild muscle stiffness to severe, painful, and uncontrollable muscle spasms. Spasticity can interfere with rehabilitation in patients with certain disorders, and often interferes with daily activities. Symptoms may include hypertonicity (increased muscle tone), muscle spasms, scissoring (involuntary crossing of the legs), and fixed joints (contractures). Spastic gait often presents after someone has suffered a stroke with a paresis of one leg, (origin of spastic gait in central motor system (pyramidal)). This is also scored here. Brooks BR, Miller RG, Swash M, Munsat TL. El Escorial revisited: revised criteria for the diagnosis of amyotrophic lateral sclerosis. Amyotroph Lateral Scler Other Motor Neuron Disord. 2000;1(5):293-299. National institute of Neurological Disorders and Stroke (NINDS). Spasticity information page. NIH. https://www.ninds.nih.gov/Disorders/All-Disorders/Spasticity-Information-page#disorders-r3. Published 2019. Accessed 9 June, 2021. We scored as TRUE: palsy; paresis; monoparesis; hemiparesis; paraparesis; tetraparesis; quadriparesis; plegia; monoplegia; hemiplegia; paraplegia; tetraplegia; quadriplegia; drop foot; foot drop; weakness, muscular weakness. We scored as TRUE: when symptoms indicate a certain paralytic condition but have not been confirmed e.g. ‘patient had symptoms of locked-in syndrome’. We scored as TRUE: decreased strength, reduced strength; loss of strength; reduced/lost function of a limb. We did NOT score: diagnoses e.g. Confirmed cases of locked-in syndrome, Bell’s palsy. These were scored in a separate section of the database. We did NOT score: neuropathy; polyneuropathy. These were NOT scored anywhere. We did NOT score: paresthesia. This was scored separately as ‘Positive sensory symptoms’. Explanation: Researchers must be aware that both central and peripheral pareses are scored here. We do not distinguish between them; it will be impossible for the machine learning to score it in that manner. Depending on the clinical diagnosis the researcher can interpret the most likely origin. true UMLS: C0151786 Definition: Muscular weakness refers to a condition in which muscle movement has become weakened or impaired. You may see it referred to as paresis or paralysis (which indicates partial or complete paralysis), or plegia (when loss of muscular strength is complete)1,2. Although paresis affects your muscles, it usually occurs due to neuronal damage (it can be both nerve damage in the PNS or damage of the first motor neuron in the CNS). In the clinical summaries of the NBB, it can be written down as paresis: monoparesis, hemiparesis, paraparesis or tetraparesis/quadriparesis or plegia: monoplegia, hemiplegia, paraplegia, tetraplegia, quadriplegia). It can also be noted as (mono)paresis of just one muscle or limb. In this parameter, we score both central paresis, caused by a problem in the central nervous system (like MS, stroke), and peripheral paresis, caused by a problem in the peripheral nervous system (peripheral nerve lesion/neuropathy). Muscular weakness Muscle:weakness Muscle_weakness Muscle weakness Muscle:weakness UMLS: C0151786 Direct match Definition: Muscular weakness refers to a condition in which muscle movement has become weakened or impaired. You may see it referred to as paresis or paralysis (which indicates partial or complete paralysis), or plegia (when loss of muscular strength is complete)1,2. Although paresis affects your muscles, it usually occurs due to neuronal damage (it can be both nerve damage in the PNS or damage of the first motor neuron in the CNS). In the clinical summaries of the NBB, it can be written down as paresis: monoparesis, hemiparesis, paraparesis or tetraparesis/quadriparesis or plegia: monoplegia, hemiplegia, paraplegia, tetraplegia, quadriplegia). It can also be noted as (mono)paresis of just one muscle or limb. In this parameter, we score both central paresis, caused by a problem in the central nervous system (like MS, stroke), and peripheral paresis, caused by a problem in the peripheral nervous system (peripheral nerve lesion/neuropathy). https://www.ncbi.nlm.nih.gov/nlmcatalog/101745816 Aminoff MJ. Neurologic Causes of Weakness and Paralysis. In: Jameson JL, Fauci AS, Kasper DL, Hauser SL, Longo DL, Loscalzo J, eds. Harrison's Principles of Internal Medicine, 20e. New York, NY: McGraw-Hill Education; 2018. Asimos AW. Evaluation of the adult with acute weakness in the emergency department. UpToDate, Hockberger, RS (Ed), UpToDate, Grayzel, J. 2013. https://medilib.ir/uptodate/show/294 We scored as TRUE: fasciculations; fibrillations (not atrial fibrillations). true UMLS: C0015644 “Fasciculations are involuntary rapid muscle twitches that are too weak to move a limb but are easily felt by patients and seen or palpated by clinicians. (Most healthy people experience fasciculations at some time, especially in the eyelid muscles.)” Fasciculations Muscular:fasciculation Muscular_fasciculation Muscular fasciculation Muscular:fasciculation UMLS: C0015644 Direct match “Fasciculations are involuntary rapid muscle twitches that are too weak to move a limb but are easily felt by patients and seen or palpated by clinicians. (Most healthy people experience fasciculations at some time, especially in the eyelid muscles.)” McGee S. Chapter 61 - Examination of the Motor System: Approach to Weakness. In. Fourth Edition ed: Elsevier Inc; 2018:551-568.e552. We scored as TRUE: sensory symptoms that are reported as part of a neurological examination or are reported using specific medical terminology (based on your best judgement). When in doubt scored as ‘Positive sensory symptoms’. We scored as TRUE: hypoalgesia/hypalgesia; hypoesthesia/hypesthesia; impaired sensibility, sensory symptoms, sensibility decreased, numbness, gnosis, hypoesthesia, diminished/reduced sense of vibration/pallesthesia. We did NOT score: patient reported sensory symptoms such as “patient reported... tingling sensation, strange feeling, changes in taste, deaf feeling in...”. These were scored as ‘Positive sensory symptoms’. We did NOT score: changes in smell or taste. This was scored as ‘Olfactory or gustatory dysfunction’. We did NOT score: diagnoses e.g. Raynaud's syndrome. These were scored in a separate section of the database. false UMLS: C0020580 Description: Sensory examination as part of the neurological examination involves testing the primary sensory modalities (light touch, pain and temperature, vibration, joint position sense), and the discriminative sensory functions (stereognosis, graphesthesia, point localization, two-point discrimination and extinction). These tests are almost never all performed, but are more often globally tested by neurologists. “Abnormal sensory symptoms can be divided into two categories: positive and negative. Negative phenomena represent loss of sensory function and are characterized by diminished or absent feeling that often is experienced as numbness and by abnormal findings on sensory examination. In disorders affecting peripheral sensation, at least one-half the afferent axons innervating a particular site are probably lost or functionless before a sensory deficit can be demonstrated by clinical examination. If the rate of loss is slow, however, lack of cutaneous feeling may be unnoticed by the patient and difficult to demonstrate on examination, even though few sensory fibers are functioning; if it is rapid, both positive and negative phenomena are usually conspicuous. Subclinical degrees of sensory dysfunction may be revealed by sensory nerve conduction studies or somatosensory evoked potentials”. “Whereas sensory symptoms may be either positive or negative, sensory signs on examination are always a measure of negative phenomena”. In the clinical summaries of the NBB, neurological examination can describe sensory symptoms. With this parameter we mean any impairment in the sensory function examination which can be objectively measured by neurological examination. We strived to be as objective as possible. Hypesthesia Negative_sensory_symptoms http://purl.obolibrary.org/obo/Negative_sensory_symptoms Negative sensory symptoms Negative:sensory:symptoms UMLS: C0020580 Direct match Description: Sensory examination as part of the neurological examination involves testing the primary sensory modalities (light touch, pain and temperature, vibration, joint position sense), and the discriminative sensory functions (stereognosis, graphesthesia, point localization, two-point discrimination and extinction). These tests are almost never all performed, but are more often globally tested by neurologists. “Abnormal sensory symptoms can be divided into two categories: positive and negative. Negative phenomena represent loss of sensory function and are characterized by diminished or absent feeling that often is experienced as numbness and by abnormal findings on sensory examination. In disorders affecting peripheral sensation, at least one-half the afferent axons innervating a particular site are probably lost or functionless before a sensory deficit can be demonstrated by clinical examination. If the rate of loss is slow, however, lack of cutaneous feeling may be unnoticed by the patient and difficult to demonstrate on examination, even though few sensory fibers are functioning; if it is rapid, both positive and negative phenomena are usually conspicuous. Subclinical degrees of sensory dysfunction may be revealed by sensory nerve conduction studies or somatosensory evoked potentials”. “Whereas sensory symptoms may be either positive or negative, sensory signs on examination are always a measure of negative phenomena”. In the clinical summaries of the NBB, neurological examination can describe sensory symptoms. With this parameter we mean any impairment in the sensory function examination which can be objectively measured by neurological examination. We strived to be as objective as possible. Aminoff MJ. Numbness, Tingling, and Sensory Loss. In: Kasper D, Fauci A, Hauser S, Longo D, Jameson JL, Loscalzo J, eds. Harrison's Principles of Internal Medicine, 19e. New York, NY: McGraw-Hill Education; 2014. https://accessmedicine.mhmedical.com/book.aspx?bookId=3095 Gelb D. The detailed neurologic examination in adults. UpToDate; Waltham MA. 2012. We scored as TRUE: nystagmus. We did NOT score: saccadic movements of the eye. These were not scored in any parameter. We did NOT score: any other visual problems. These were scored separately as ‘Visual problems’. true UMLS: C0028738 Definition: Nystagmus is a rhythmic regular oscillation of the eyes. It may consist of alternating phases of a slow drift in one direction with a corrective quick "jerk" in the opposite direction, or of slow, sinusoidal, "pendular" oscillations to and fro. This can occur due to problems in the vestibular system, or it can have a neurological cause. And it an occur when a patient is intoxicated with certain medication, drugs or alcohol. In the clinical summaries of the NBB, the type of nystagmus was most often NOT described. It mostly will be reported in cases with cerebellar ataxia, but sometimes it can be reported without being clear whether it is of a central or peripheral origin. Therefore, it was not possible to determine/deduce the origin of the nystagmus. We have decided to score all types of nystagmus in this parameter. Nystagmus http://purl.obolibrary.org/obo/Nystagmus Nystagmus Nystagmus UMLS: C0028738 Direct match Definition: Nystagmus is a rhythmic regular oscillation of the eyes. It may consist of alternating phases of a slow drift in one direction with a corrective quick "jerk" in the opposite direction, or of slow, sinusoidal, "pendular" oscillations to and fro. This can occur due to problems in the vestibular system, or it can have a neurological cause. And it an occur when a patient is intoxicated with certain medication, drugs or alcohol. In the clinical summaries of the NBB, the type of nystagmus was most often NOT described. It mostly will be reported in cases with cerebellar ataxia, but sometimes it can be reported without being clear whether it is of a central or peripheral origin. Therefore, it was not possible to determine/deduce the origin of the nystagmus. We have decided to score all types of nystagmus in this parameter. Barton J. Overview of nystagmus. In: Wolters Kluwer Health; 2013. We scored as TRUE: decreased or lost sense of smell and/or taste; olfactory dysfunction. We did NOT score: any ambiguous descriptions such as ‘the patient’s smell had gotten worse’. true UMLS: C0013378 UMLS: C5577689 Description: Disorders of smell (olfaction; also called anosmia) and taste (gustation; also called dysgeusia) are often seen in the early stages of Parkinson’s disease and Alzheimer’s disease but are also observed in other neurodegenerative diseases. Dysgeusia Olfactory dysfunction Olfactory_and_gustatory_dysfunction http://purl.obolibrary.org/obo/Olfactory_and_gustatory_dysfunction Olfactory and gustatory dysfunction Olfactory:and:gustatory:dysfunction UMLS: C0013378 UMLS code refers to a narrower concept UMLS: C5577689 UMLS code refers to a narrower concept Description: Disorders of smell (olfaction; also called anosmia) and taste (gustation; also called dysgeusia) are often seen in the early stages of Parkinson’s disease and Alzheimer’s disease but are also observed in other neurodegenerative diseases. https://pubmed.ncbi.nlm.nih.gov/29904888/ https://pubmed.ncbi.nlm.nih.gov/30632380/ https://pubmed.ncbi.nlm.nih.gov/31604557/ https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5249198/ Grouping variable containing other psychiatric signs/symptoms/findings. Other_psychiatric_signs_and_symptoms http://purl.obolibrary.org/obo/Other_psychiatric_signs_and_symptoms http://purl.obolibrary.org/obo/http://www.semanticweb.org/irholtman/ontologies/2023/Other_psychiatric_signs_and_symptoms Other psychiatric signs and symptoms Other:psychiatric:signs:and:symptoms Grouping variable containing other signs and symptoms of cortical dysfunction. Other_signs_and_symptoms_of_cortical_dysfunction http://purl.obolibrary.org/obo/Other_signs_and_symptoms_of_cortical_dysfunction http://purl.obolibrary.org/obo/http://www.semanticweb.org/irholtman/ontologies/2023/Other_signs_and_symptoms_of_cortical_dysfunction Other signs and symptoms of cortical dysfunction Other:signs:and:symptoms:of:cortical:dysfunction We scored as TRUE: nycturia; dysuria; polyuria; anuria; minimal urine production; and other similar terms. We scored as TRUE: problems with urinating/miction/micturition; enuresis; and other urinary problems. We did NOT score: Decreased urine production due to renal insufficiency or renal failure; Urinary tract infection; hydronephrosis, hydronephritis. We did NOT score: inappropriate voiding such ‘patient urinated in random places’ - this was scored as ‘Socially inappropriate behavior'. Explanation: Please be aware that the urinary problems that are scored in this parameter, also include problems caused by local bladder or urethral problems. There is no distinction between urinary problems as a consequence of a neurodegenerative disease, and urinary problems that have a different origin. We decided to feed the machine learning tool all the urinary problems related terminology as listed above, in order not to miss anything. false UMLS: C0260709 Description: Here we want to score other relevant types of urinary problems not captured under ‘Urinary incontinence’: Urinary problems (other) Other_urinary_problems http://purl.obolibrary.org/obo/Other_urinary_problems Other urinary problems Other:urinary:problems UMLS: C0260709 Direct match We scored as TRUE: paranoid; paranoia; suspicious; suspiciousness. We did NOT score: phobias – these were scored under ‘Anxiety’. false UMLS: C1456784 Definition: “Paranoia involves intense anxious or fearful feelings and thoughts often related to persecution, threat, or conspiracy. Paranoia occurs in many mental disorders, but is most often present in psychotic disorders”. It can also be present in patient with a neurodegenerative disorder. Paranoia and suspiciousness Paranoia http://purl.obolibrary.org/obo/Paranoia Paranoia Paranoia UMLS: C1456784 Direct match Definition: “Paranoia involves intense anxious or fearful feelings and thoughts often related to persecution, threat, or conspiracy. Paranoia occurs in many mental disorders, but is most often present in psychotic disorders”. It can also be present in patient with a neurodegenerative disorder. American Psychiatric Association. Diagnostic and statistical manual of mental disorders, Fifth Edition. Vol 21. Arlington, VA.: American Psychiatric Association; 2013. Mental Health America. Paranoia and delusional disorders. Mental Health America. https://www.mhanational.org/conditions/paranoia-and-delusional-disorders. Published 2021. Accessed 9 June, 2021. We scored as TRUE: the word ‘parkinsonism’; Parkinson-like; Parkinson of a specific limb; resembles Parkinson's disease; lower-limb parkinsonism; all suspected and confirmed cases of Parkinson’s disease’. We scored as TRUE: sentences with the literal word ‘extrapyramidal’. We did NOT score: freezing or freezing of gait, which are scored as ‘Bradykinesia’. We did NOT score: Individual symptoms such as bradykinesia, hypomimia, mask-like facial expression, altered mimic, slowness in movement, general slowness, tremor, or rigidity, which are scored separately as ‘Facial masking’, or ‘Bradykinesia’, or ‘Tremor’, or ‘Muscle rigidity’. We did NOT score: ’Multiple system atrophy, progressive supranuclear palsy, corticobasal degeneration, or any disease. These are scored in a separate section of the database. We did NOT score: sentences where it says there are “no extrapyramidal symptoms or signs.” CAUTION: researchers must be aware with machine learning it is not possible to distinguish between parkinsonism as a result of a neurodegenerative disorder, or as a possible side effect of certain medications. From experience we see that in most cases it is a result of a neurodegenerative disorder, but one can only be sure when the medication has been checked. true UMLS: C0242422 Definition: Parkinsonism is a clinical syndrome presenting that requires both of the following: Bradykinesia Rest tremor or rigidity In the clinical summaries of the NBB, the word parkinsonism is sometimes written down, but more often those three motor symptoms are written down separately and are present in different combinations. If that is the case, we score them separately. In the clinical summaries of the NBB, the word “extrapyramidal” is frequently used as a term for the symptoms of patients who suffer from Parkinson’s disease, written down as such by the neurologists (synonym for parkinsonism). It can also be mentioned in psychiatry cases. Parkinsonism aggravated Parkinsonism http://purl.obolibrary.org/obo/Parkinsonism Parkinsonism Parkinsonism UMLS: C0242422 UMLS code refers to a narrower concept Definition: Parkinsonism is a clinical syndrome presenting that requires both of the following: Bradykinesia Rest tremor or rigidity In the clinical summaries of the NBB, the word parkinsonism is sometimes written down, but more often those three motor symptoms are written down separately and are present in different combinations. If that is the case, we score them separately. In the clinical summaries of the NBB, the word “extrapyramidal” is frequently used as a term for the symptoms of patients who suffer from Parkinson’s disease, written down as such by the neurologists (synonym for parkinsonism). It can also be mentioned in psychiatry cases. https://pubmed.ncbi.nlm.nih.gov/26474316/ We scored as TRUE: imprinting disorders or problem; impaired imprinting. true UMLS: C0701811 Description: A notable sign of dementia is the inability to encode and store new information received via the senses (encoding). New information is no longer transferred from working memory to long-term memory and recent events are forgotten (consolidation). This means that someone is no longer able to remember something they have just happened or been seen or heard. This is referred to as an ‘inprentingsstoornis’ or ‘imprinting disorder’ in Dutch and is frequently translated as such. After neuropsychological testing, a conclusion is drawn about whether a patient suffered from impaired imprinting. Imprinting disturbances Poor_short-term_memory http://purl.obolibrary.org/obo/Poor_short-term_memory Poor short-term memory Poor:short-term:memory UMLS: C0701811 Direct match Description: A notable sign of dementia is the inability to encode and store new information received via the senses (encoding). New information is no longer transferred from working memory to long-term memory and recent events are forgotten (consolidation). This means that someone is no longer able to remember something they have just happened or been seen or heard. This is referred to as an ‘inprentingsstoornis’ or ‘imprinting disorder’ in Dutch and is frequently translated as such. After neuropsychological testing, a conclusion is drawn about whether a patient suffered from impaired imprinting. https://pubmed.ncbi.nlm.nih.gov/11556645/ We scored as TRUE: any sensory changes that have clearly been reported by the patient. These might be described as: ‘Patient had/reported’... numbness, deaf feeling in limb, tingling, paresthesia, dysesthesia, hyperesthesia; hyperalgesia; allodynia. We scored as TRUE: painful symptoms described in such as manners as: pins and needles; pricking; bandlike; lightning-like shooting feelings (lancinations); aching; knifelike; twisting; drawing; pulling; tightening; burning; searing; electrical; raw feelings. We also scored hypersensitivity. We did NOT score: gnosis (or any word deriving from root word gnosis (gnostic)/indicating an impaired gnosis), hypoalgesia or sensory changes measured in neurological sensory testing. These were scored as ‘Negative sensory symptoms'. We did NOT score: changes in smell or taste. This was scored as ‘Olfactory or gustatory dysfunction’. We did NOT score: diagnoses such as Raynaud's syndrome. These were scored in a separate section of the database. We did NOT score: Feelings of being cold; cold sensations; ‘patient had cold feet’. false UMLS: C0030554 Description: Here we want to capture patient-reported sensory symptoms or other sensory symptoms that are not captured under the more specific parameter 'Negative sensory symptoms’. Here we include any reported sensory function deficit such as numbness, tingling, burning feeling, numb feeling. “Abnormal sensory symptoms can be divided into two categories: positive and negative. The prototypical positive symptom is tingling (pins and needles); other positive sensory phenomena include itch and altered sensations that are described as pricking, bandlike, lightning-like shooting feelings (lancinations), aching, knifelike, twisting, drawing, pulling, tightening, burning, searing, electrical, or raw feelings. Such symptoms are often painful.” “Positive phenomena usually result from trains of impulses generated at sites of lowered threshold or heightened excitability along a peripheral or central sensory pathway. The nature and severity of the abnormal sensation depend on the number, rate, timing, and distribution of ectopic impulses and the type and function of nervous tissue in which they arise. Because positive phenomena represent excessive activity in sensory pathways, they are not necessarily associated with a sensory deficit (loss) on examination.” Paresthesia Patient reported sensory deficits Positive_sensory_symptoms http://purl.obolibrary.org/obo/Positive_sensory_symptoms Positive sensory symptoms Positive:sensory:symptoms UMLS: C0030554 Direct match Description: Here we want to capture patient-reported sensory symptoms or other sensory symptoms that are not captured under the more specific parameter 'Negative sensory symptoms’. Here we include any reported sensory function deficit such as numbness, tingling, burning feeling, numb feeling. “Abnormal sensory symptoms can be divided into two categories: positive and negative. The prototypical positive symptom is tingling (pins and needles); other positive sensory phenomena include itch and altered sensations that are described as pricking, bandlike, lightning-like shooting feelings (lancinations), aching, knifelike, twisting, drawing, pulling, tightening, burning, searing, electrical, or raw feelings. Such symptoms are often painful.” “Positive phenomena usually result from trains of impulses generated at sites of lowered threshold or heightened excitability along a peripheral or central sensory pathway. The nature and severity of the abnormal sensation depend on the number, rate, timing, and distribution of ectopic impulses and the type and function of nervous tissue in which they arise. Because positive phenomena represent excessive activity in sensory pathways, they are not necessarily associated with a sensory deficit (loss) on examination.” https://pubmed.ncbi.nlm.nih.gov/28205574/ Aminoff MJ. Numbness, Tingling, and Sensory Loss. In: Kasper D, Fauci A, Hauser S, Longo D, Jameson JL, Loscalzo J, eds. Harrison's Principles of Internal Medicine, 19e. New York, NY: McGraw-Hill Education; 2014. https://accessmedicine.mhmedical.com/book.aspx?bookId=3095 UMLS: C0205487 Domain variable containing signs/symptoms/findings associated with psychiatric disturbances. Psychiatric http://purl.obolibrary.org/obo/Psychiatric Psychiatric Psychiatric We scored as TRUE: psychosis when it is noted as such. Or psychotic episode; psychotic behavior; psychosis-like; organic psycho syndrome. As noted above it can be caused by different entities. We also scored as TRUE: for example, ‘patient requested help to prevent from getting another psychosis'; ‘she reported hanging towards a psychosis'. Here we want to capture the word psychosis even when it is only suspected. We did NOT score: symptoms of psychosis such as hallucination and delusions, which were scored separately as ‘Hallucinations’ and ‘Delusions’, respectively. We did NOT score: when it was clearly NOT present. E.g. ‘There was no psychosis'; 'psychosis was not evident/present’. Use your best judgement when scoring. If in doubt, do NOT score. Explanation: Researchers must be aware we include all descriptions of psychosis, regardless the entity. false UMLS: C0033975 Definition: a significant impairment in reality testing, as evidenced by symptoms such as hallucinations, delusions, thought disorganization and grossly disorganized behavior – can result from neurological conditions, from use of certain substances, or as a manifestation of a psychiatric disorder. Psychosis is a condition of the mind broadly defined as a loss of contact with reality. Psychotic symptoms can increase patients’ risk for harming themselves or others or being unable to meet their basic needs. Psychosis http://purl.obolibrary.org/obo/Psychosis Psychosis Psychosis UMLS: C0033975 UMLS code refers to a narrower concept Definition: a significant impairment in reality testing, as evidenced by symptoms such as hallucinations, delusions, thought disorganization and grossly disorganized behavior – can result from neurological conditions, from use of certain substances, or as a manifestation of a psychiatric disorder. Psychosis is a condition of the mind broadly defined as a loss of contact with reality. Psychotic symptoms can increase patients’ risk for harming themselves or others or being unable to meet their basic needs. American Psychiatric Association. Diagnostic and statistical manual of mental disorders, Fifth Edition. Vol 21. Arlington, VA.: American Psychiatric Association; 2013. Marder S. Psychosis in adults: Epidemiology, clinical manifestations, and diagnostic evaluation. https://medilib.ir/uptodate/show/17193 In the clinical summaries of the NBB, restlessness was frequently noted as such. We scored as TRUE: restlessness; restless; night-time restlessness; nightly unrest; motoric restlessness. We did NOT score: restless legs. This was not scored anywhere. We did NOT score: anything else. false UMLS: C3887611 Definition: It has been proposed that three core features characterize restlessness: Diffuse motor activity or motion subject to limited control (e.g., pacing, fidgeting, repetitive motor movements) that is judged by a clinician, caregiver, or observer to be excessive and/or inappropriate to the circumstances. The behavior(s) is(are) generally non-productive or disorganized, failing to appropriately address or target potential underlying causes such as stress or boredom. The behavior(s) is(are) associated with a degree of subjective distress (e.g., nervousness, discomfort) that is either communicated by the person with dementia or extrapolated from the behavior itself. Restlessness http://purl.obolibrary.org/obo/Restlessness Restlessness Restlessness UMLS: C3887611 Direct match Definition: It has been proposed that three core features characterize restlessness: Diffuse motor activity or motion subject to limited control (e.g., pacing, fidgeting, repetitive motor movements) that is judged by a clinician, caregiver, or observer to be excessive and/or inappropriate to the circumstances. The behavior(s) is(are) generally non-productive or disorganized, failing to appropriately address or target potential underlying causes such as stress or boredom. The behavior(s) is(are) associated with a degree of subjective distress (e.g., nervousness, discomfort) that is either communicated by the person with dementia or extrapolated from the behavior itself. https://pubmed.ncbi.nlm.nih.gov/26743166/ We scored as TRUE: epileptic seizure; seizure; epileptic insult; convulsions, tonic-clonic seizure, tonic-clonic insult; epilepsy; patient was clonic; myoclonia. We scored as TRUE: insult or absence or episode if clearly of epileptic nature, otherwise we did NOT score. We did NOT score: collapse; decreased consciousness; coma; vasovagal response. These were NOT score anywhere. Explanation: the term collapse is frequently used when the story of the event does not have the typical description/features of an epileptic seizure (like urinary incontinency, bitten tongue, tonic-clonic muscle jerks) and an epileptic seizure is not highly assumed. Decreased consciousness is a too broad a term, which can also be used to call someone absent-minded. Coma can have more causes than epilepsy and is not a frequently used term in the clinical summaries of the NBB, so we decided to leave it out. Vasovagal response is something different than a seizure. false UMLS: C0036572 Definition: “Seizures are divided into two major categories – focal seizures and generalized seizures. However, there are many different types of seizures in each of these categories”. There are many different causes for a seizure including epilepsy, brain damage and high fever (febrile seizures). In the clinical summaries of the NBB, it is mostly described as a seizure, epileptic seizure, or epileptic insult. Seizures http://purl.obolibrary.org/obo/Seizures Seizures Seizures UMLS: C0036572 Direct match Definition: “Seizures are divided into two major categories – focal seizures and generalized seizures. However, there are many different types of seizures in each of these categories”. There are many different causes for a seizure including epilepsy, brain damage and high fever (febrile seizures). In the clinical summaries of the NBB, it is mostly described as a seizure, epileptic seizure, or epileptic insult. National Institute of Neurological Disorders and Stroke (NINDS). The epilepsies and seizures: Hope through research. NIH. https://www.ninds.nih.gov/Disorders/Patient-Caregiver-Education/Hope-Through-Research/Epilepsies-and-Seizures-Hope-Through#3109_2. Published 2020. Accessed 9 June, 2021. Domain variable containing signs/symptoms/findings associated with sensory and autonomic disturbances. Sensory:autonomic Sensory_autonomic Sensory/autonomic Sensory:autonomic Grouping variable containing signs/symptoms/findings associated with sensory deficits. Sensory:deficits Sensory_deficits Sensory deficits Sensory:deficits UMLS: C0424296 Grouping variable containing signs of (dis)inhibition. Signs_of_dis_inhibition http://purl.obolibrary.org/obo/Signs_of_dis_inhibition Signs of dis/inhibition Signs:of:dis:inhibition Grouping variable containing signs of impaired mobility Signs_of_impaired_mobility http://purl.obolibrary.org/obo/Signs_of_impaired_mobility Signs of impaired mobility Signs:of:impaired:mobility In the clinical summaries of the NBB, it was often described as ‘day night rhythm disturbances’, ‘changed circadian rhythm’. When it was noted as such, we labeled it as TRUE. Here we wanted to be as objective as possible. We scored as TRUE: day-night rhythm disturbances; changed circadian rhythm; day-night rhythm turned around. We did NOT score: any other sleep disturbances – these can be score as such ‘Sleep disturbances’. false UMLS: C0877792 Definition: “Circadian rhythm sleep disorders are caused by desynchronization between internal sleep-wake rhythms and the light-darkness cycle. Patients typically have insomnia, excessive daytime sleepiness, or both, which typically resolve as the body clock realigns itself”. When a patient turns around their circadian rhythm, they are often awake at night, and sleepier during the day (daytime sleepiness). It is frequently described in patients suffering neurodegenerative diseases, like Alzheimer’s disease or Parkinson’s disease. Day / night rhythm disturbances Sleep_disorders_circadian_rhythm http://purl.obolibrary.org/obo/Sleep_disorders_circadian_rhythm Sleep disorders, circadian rhythm Sleep:disorders:circadian:rhythm UMLS: C0877792 Direct match Definition: “Circadian rhythm sleep disorders are caused by desynchronization between internal sleep-wake rhythms and the light-darkness cycle. Patients typically have insomnia, excessive daytime sleepiness, or both, which typically resolve as the body clock realigns itself”. When a patient turns around their circadian rhythm, they are often awake at night, and sleepier during the day (daytime sleepiness). It is frequently described in patients suffering neurodegenerative diseases, like Alzheimer’s disease or Parkinson’s disease. https://pubmed.ncbi.nlm.nih.gov/21570695/ Schwab RJ. Circadian rhythm sleep disorders. MSD. https://www.msdmanuals.com/professional/neurologic-disorders/sleep-and-wakefulness-disorders/circadian-rhythm-sleep-disorders. Published 2020. Accessed 9 June, 2021. We score as TRUE: sleeping problems; sleeping disturbances; insomnia; trouble sleeping; difficulty sleeping; difficulty falling asleep; difficulty maintaining sleep; excessive daytime sleepiness; hypersomnia; hypersomnolence. We did NOT score: When it says that the patient looked somnolent, this is more an observation during neurological examination, and is more about the level of consciousness (this is an observation); This was not scored anywhere. We did NOT score: day-night rhythm disturbances; changes in circadian rhythm. These were scored separately as ‘Day night rhythm disturbances'. We did NOT score: REM sleep behavior or REM sleep behavior disorder (RBD). These were scored in a separate section of the database. We did NOT score: night-time restlessness; restlessness. These were scored separately as ‘Restlessness’. We did NOT score: fatigue, tiredness. These were scored separately as ‘Fatigue’. We did NOT score: any ambiguous sentence such as ‘patient had a changed sleeping pattern’ (i.e. It is not clear if this is day-night alteration). This was not scored anywhere. Explanation: Because multiple words are used interchangeably to address sleeping disturbances, we have decided to score multiple words that indicate sleeping disturbances. This might mean that we include a few cases that might report sleeping problems in the context of a central disorder of hypersomnolence for example, like narcolepsy. However, if we do not include these multiple variables, we might miss a lot of data. Together with the Clinical Diagnoses the sleeping disturbances can be placed into context. false UMLS: C0037317 Description: Sleep disturbances can be present in many different forms. Most of the times it means that patients have trouble falling asleep, but it can also mean that patients suffer from excessive sleeping and excessive daytime sleepiness (EDS). Daytime sleepiness is defined as excessive when it causes a subjective complaint or interferes with function. The International Classification of Sleep Disorders, third Edition (ICSD-3) defines EDS as the inability to maintain wakefulness and alertness during major making episodes of the day, with sleep occurring unintentionally, or at inappropriate times almost daily for at least three months. The terms hypersomnia or hypersomnolence are sometimes used interchangeably with excessive daytime sleepiness. Fatigue refers to a subjective lack of physical or mental energy. This is scored separately because this actual word is used a lot in the clinical histories. The causes of excessive daytime sleepiness are numerous. However, in the cases of the NBB, it is often present in the context of a neurodegenerative brain disease. In the clinical summaries of the NBB, it is frequently noted as ‘sleeping problems’, without further specification. We might assume that by sleeping problems, the neurologists had asked for, generally mean that the patient had trouble sleeping. For this project and because we use a machine learning approach, we have decided to score all “sleeping problems”, including all the possible symptoms, like excessive daytime sleepiness’, hypersomnia, and hypersomnolence. Because we can assume that most of the times a sleeping problem is reported, it means “not being able to sleep as well as before”. Sleep:disturbances Sleep_disturbances Sleep disturbances Sleep:disturbances UMLS: C0037317 Direct match Description: Sleep disturbances can be present in many different forms. Most of the times it means that patients have trouble falling asleep, but it can also mean that patients suffer from excessive sleeping and excessive daytime sleepiness (EDS). Daytime sleepiness is defined as excessive when it causes a subjective complaint or interferes with function. The International Classification of Sleep Disorders, third Edition (ICSD-3) defines EDS as the inability to maintain wakefulness and alertness during major making episodes of the day, with sleep occurring unintentionally, or at inappropriate times almost daily for at least three months. The terms hypersomnia or hypersomnolence are sometimes used interchangeably with excessive daytime sleepiness. Fatigue refers to a subjective lack of physical or mental energy. This is scored separately because this actual word is used a lot in the clinical histories. The causes of excessive daytime sleepiness are numerous. However, in the cases of the NBB, it is often present in the context of a neurodegenerative brain disease. In the clinical summaries of the NBB, it is frequently noted as ‘sleeping problems’, without further specification. We might assume that by sleeping problems, the neurologists had asked for, generally mean that the patient had trouble sleeping. For this project and because we use a machine learning approach, we have decided to score all “sleeping problems”, including all the possible symptoms, like excessive daytime sleepiness’, hypersomnia, and hypersomnolence. Because we can assume that most of the times a sleeping problem is reported, it means “not being able to sleep as well as before”. https://pubmed.ncbi.nlm.nih.gov/25367475/ With this parameter we want to score as objectively as possible. If in doubt, consider scoring under the separate parameter ‘Socially inappropriate behavior’. If still in doubt, do NOT score. We scored as TRUE: any instance describing disinhibition; uninhibited. We scored as TRUE: hypersexuality; impulse control/impulsive; tapping beahvior. We did not score: loss of decorum; other behavioral descriptions that are less objective e.g. patient smeared with feces. We scored these separately as ‘Socially inappropriate behavior'. true UMLS: C0424296 Definition: “Social disinhibition can be defined as the inability to withhold a prepotent response or suppress an inappropriate or unwanted behavior. It can refer to the production of socially inappropriate comments and/or actions”. In the clinical summaries of the NBB, disinhibited behavior or ‘Social disinhibition’ is frequently noted in cases of Frontotemporal dementia, Alzheimer’s disease, Parkinson’s disease, but also in psychiatric patients. There are cases in which the patients show sexual disinhibition, disinhibition regarding food intake (mainly sweets), disinhibited behavior. Disinhibition Social:disinihibition Social_disinihibition Social disinihibition Social:disinihibition UMLS: C0424296 Direct match Definition: “Social disinhibition can be defined as the inability to withhold a prepotent response or suppress an inappropriate or unwanted behavior. It can refer to the production of socially inappropriate comments and/or actions”. In the clinical summaries of the NBB, disinhibited behavior or ‘Social disinhibition’ is frequently noted in cases of Frontotemporal dementia, Alzheimer’s disease, Parkinson’s disease, but also in psychiatric patients. There are cases in which the patients show sexual disinhibition, disinhibition regarding food intake (mainly sweets), disinhibited behavior. Radke A, Cahn-Weiner DA, Johnson JK. Disinhibition. In: Kreutzer JS, DeLuca J, Caplan B, eds. Encyclopedia of Clinical Neuropsychology. Cham: Springer International Publishing; 2018:1189-1192. https://link.springer.com/referencework/10.1007/978-3-319-57111-9 We scored as TRUE: loss of decorum; lack of decorum; poor personal hygiene; self-negligence; self-neglect; unusual behaviors involving feces or urination e.g. 'patient smeared feces'; ‘patient urinated in random places'; other altered behavior involving decorum e.g. 'patient kissed all the other patients'. We also scored as TRUE: yelling; screaming; shouting. true UMLS: C2220010 Description: This is a subtype of Social Disinhibition and includes behaviors that violate social graces/norms. Here we want to capture behavior that involves a loss of (public) decorum. This can include such behaviors as smearing feces; urinating in inappropriate places; offensive language and gestures; poor personal hygiene; untidy eating habits; screaming; inappropriate laughing. Loss of decorum Socially_inappropriate_behavior http://purl.obolibrary.org/obo/Socially_inappropriate_behavior Socially inappropriate behavior Socially:inappropriate:behavior UMLS: C2220010 Direct match We scored as TRUE: stress; stressed. When the word stress was noted. We also scored as TRUE: suspected diagnoses such as ‘suspected post-traumatic stress syndrome (PTSS)', stress headaches when pertaining to mental stress; ‘stress enuresis’; stress ulcus. We did NOT score: confirmed diagnoses of PTSS, anxiety disorders, anxiety (this is scored separately as ‘Anxiety'), panic disorders, surmenage/burn-out. These were scored in a separate section of the database. Explanation: Researchers must be aware that stress is scored whenever this is mentioned. This is different for every individual, but when it was mentioned in the clinical summary, we assume it was relevant enough to mention for that individual, and potentially had an influence on daily functioning or a disease course. false UMLS: C0038435 Definition: “A working definition of stress that fits many human situations is a condition in which an individual is aroused and made anxious by an uncontrollable aversive challenge—for example, stuck in heavy traffic on a motorway, a hostile employer, unpaid bills, or a predator. Stress leads to a feeling of fear and anxiety. Depending on the circumstances, the fear response can lead to either fight or flight. The magnitude of the stress and its physiological consequences are influenced by the individual’s perception of their ability to cope with the stressor”. Stress http://purl.obolibrary.org/obo/Stress Stress Stress UMLS: C0038435 Direct match Definition: “A working definition of stress that fits many human situations is a condition in which an individual is aroused and made anxious by an uncontrollable aversive challenge—for example, stuck in heavy traffic on a motorway, a hostile employer, unpaid bills, or a predator. Stress leads to a feeling of fear and anxiety. Depending on the circumstances, the fear response can lead to either fight or flight. The magnitude of the stress and its physiological consequences are influenced by the individual’s perception of their ability to cope with the stressor”. https://www.sciencedirect.com/book/9780128009512/stress-concepts-cognition-emotion-and-behavior Fink G. Stress, definitions, mechanisms, and effects outlined: lessons from anxiety. In: Stress: Concepts, cognition, emotion, and behavior. Elsevier; 2016:3-11. We scored as TRUE: swallowing problems; dysphagia; aphagia; trouble swallowing; choking; choked on food We did NOT score: Chewing; reduced food intake; lost weight; decreased appetite; fed with nasogastric tube/liquid food. true UMLS: C0392678 Definition: Having trouble swallowing (dysphagia) is a symptom that accompanies a number of neurological disorders. The problem can occur at any stage of the normal swallowing process as food and liquid move from the mouth, down the back of the throat, through the esophagus and into the stomach, to coughing or choking because the food or liquid is entering the windpipe (aspiration). Dysphagia Swallowing:problem Swallowing_problem Swallowing problem Swallowing:problem UMLS: C0392678 Direct match Definition: Having trouble swallowing (dysphagia) is a symptom that accompanies a number of neurological disorders. The problem can occur at any stage of the normal swallowing process as food and liquid move from the mouth, down the back of the throat, through the esophagus and into the stomach, to coughing or choking because the food or liquid is entering the windpipe (aspiration). National Institute of Neurological Disorders and Stroke (NINDS). Swallowing disorders information page. NIH (NINDS). https://www.ninds.nih.gov/Disorders/All-Disorders/Swallowing-Disorders-Information-Page. Published 2019. Accessed 9 June, 2021. We scored as TRUE: Rest tremor; action tremor; tremor; trembling hand. We did NOT score: shaking, as this is often described in the case notes in relation to epilepsy / seizures. CAUTION: Researchers must be aware that the tremor can come from different origins, as it can both be a rest tremor or an action tremor. Depending on the clinical diagnosis, one can assume a certain type of tremor. Because it is often not specified (written only as ‘tremor’), the machine learning will select all tremor cases, which can also mean the action tremors. If we decided to only select rest tremor, we might miss a lot of tremor cases. true UMLS: C0040822 Definition: Tremor is defined as an involuntary, rhythmic, and oscillatory movement of a body part. Tremor is an involuntary, rhythmic muscle contraction leading to shaking movements in one or more parts of the body. It is a common movement disorder that most often affects the hands but can also occur in the arms, head, vocal cords, torso, and legs. In the clinical summaries of the NBB, the type of tremor was often not specified, as the neurologists also do not specify it in their documentation every time. It was mostly reported in summaries where it concerns Parkinson’s disease, so then we can assume it being a rest tremor. But most often, it was NOT specified (no remarks on type of tremor, like ‘rest’ or ‘action’). Therefore, we decided to include the word tremor in general. Tremor http://purl.obolibrary.org/obo/Tremor Tremor Tremor UMLS: C0040822 Direct match Definition: Tremor is defined as an involuntary, rhythmic, and oscillatory movement of a body part. Tremor is an involuntary, rhythmic muscle contraction leading to shaking movements in one or more parts of the body. It is a common movement disorder that most often affects the hands but can also occur in the arms, head, vocal cords, torso, and legs. In the clinical summaries of the NBB, the type of tremor was often not specified, as the neurologists also do not specify it in their documentation every time. It was mostly reported in summaries where it concerns Parkinson’s disease, so then we can assume it being a rest tremor. But most often, it was NOT specified (no remarks on type of tremor, like ‘rest’ or ‘action’). Therefore, we decided to include the word tremor in general. https://pubmed.ncbi.nlm.nih.gov/29193359/ We scored as TRUE: basically anything concerning reduced attention or concentration; difficulty paying attention; difficulty concentrating. In most cases this is clearly written in the clinical history. true UMLS: C0235198 Description: Impairment in concentration or attention. This can be caused by any progressive neurodegenerative disease, a psychiatric disease, but also due to medication, intoxication, or a metabolic disease. In the clinical summaries of the NBB it is often described in a neuropsychological examination, when it concerns a neurodegenerative disease, but also in psychiatric cases. Concentration problems Unable_to_concentrate http://purl.obolibrary.org/obo/Unable_to_concentrate Unable to concentrate Unable:to:concentrate UMLS: C0235198 Direct match UMLS: C0749870 Sub-grouping variable containing signs and symptoms associated with upper motor neuron dysfunction. Upper_motor_neuron_signs http://purl.obolibrary.org/obo/Upper_motor_neuron_signs Upper motor neuron signs Upper:motor:neuron:signs Here we want to score as objectively as possible, therefore: We scored as TRUE: urinary incontinence; incontinent for urine; bladder retention; urine retention; urinary retention; bladder incontinence; catheterized; catheterization; urge incontinence; or stress-incontinence [NOTE: Please be careful to only score urinary catheterization and NOT other types of catheterizations such as a Tenckhoff catheter for pleural fluid drainage]. We did NOT score: Decreased urine production due to renal insufficiency or renal failure; Urinary tract infection (UTI) (this is a diagnosis and will be scored in a separate section of the database.). These were not scored anywhere. We also did not score anuria, which was scored as ‘Other urinary problems’. We did NOT score: inappropriate voiding, such ‘patient urinated in random places’ - this was scored as ‘Socially inappropriate behavior'. true UMLS: C0042024 Definition: Urinary incontinency is the accidental passing of urine. Lower urinary tract dysfunction or lower urinary tract symptoms (LUTS) are common in neurodegenerative diseases and in older adults. In the clinical summaries of the NBB, it could be described as such (‘urinary incontinency’) or when it is mentioned that the patient required a ‘permanent’ or ‘indwelling catheter’. Nocturia, incontinence, and urgency as well as poor bladder emptying are the most common symptoms. Urinary:incontinence Urinary_incontinence Urinary incontinence Urinary:incontinence UMLS: C0042024 Direct match Definition: Urinary incontinency is the accidental passing of urine. Lower urinary tract dysfunction or lower urinary tract symptoms (LUTS) are common in neurodegenerative diseases and in older adults. In the clinical summaries of the NBB, it could be described as such (‘urinary incontinency’) or when it is mentioned that the patient required a ‘permanent’ or ‘indwelling catheter’. Nocturia, incontinence, and urgency as well as poor bladder emptying are the most common symptoms. National Health Service. Urinary incontinence. NHS, UK. https://www.nhs.uk/conditions/urinary-incontinence/. Published 2019. Accessed. We scored as TRUE: language impairment; linguistic disturbances; incoherent/unintelligible talking/speech; word fluency difficulties; impaired repetition; echolalia; paraphasia’s. We also scored as TRUE: mutism, poverty of speech; patient didn’t talk anymore. We also scored as TRUE: speech difficulties; slow speech. We did NOT score: motoric speaking difficulties; word-finding problems, which were scored separately as ‘Dysarthria’, and ‘Word-finding difficulties’, respectively. We did NOT score: impaired comprehension; communication problems. These are scored separately as ‘Impaired comprehension’ and ‘Communication impairment’, respectively. We did NOT score: learning; ‘was not able to learn’ new things. This is not scored anywhere. We also did NOT score: sentences that are highly ambiguous. Use your best judgement. If in doubt, do NOT score. PLEASE NOTE: Patients with items scored under this parameter may fit a classification of ‘Expressive aphasia’. However, given the subjective descriptions provided in the NBB reports, we are unable to accurately classify the different forms of aphasia, it could also mean comprehensive aphasia. Furthermore, we cannot make any distinction between primary progressive aphasias and other forms of aphasia. Therefore, interpretation should be made with caution. true UMLS: C0566027 Description: Here we mean that the patient’s use of language is impaired, or the patient speaks incoherently. Here we want to encompass language problems that are not described as dysphasia/aphasia. People with neurocognitive orders often have impairments in language expression described as ‘Expressive aphasia (non-fluent, agrammatic)’. This is defined as: “People with this pattern of aphasia may understand what other people say better than they can speak. People with this pattern of aphasia struggle to get words out, speak in very short sentences and omit words. A person might say, "Want food" or "Walk park today." A listener can usually understand the meaning, but people with this aphasia pattern are often aware of their difficulty communicating and may get frustrated”. We do not distinguish between aphasias caused by cerebrovascular accidents and neurodegenerative diseases or other causes. Language impairment (expressive) Verbal:impairment Verbal_impairment Verbal impairment Verbal:impairment UMLS: C0566027 Direct match Description: Here we mean that the patient’s use of language is impaired, or the patient speaks incoherently. Here we want to encompass language problems that are not described as dysphasia/aphasia. People with neurocognitive orders often have impairments in language expression described as ‘Expressive aphasia (non-fluent, agrammatic)’. This is defined as: “People with this pattern of aphasia may understand what other people say better than they can speak. People with this pattern of aphasia struggle to get words out, speak in very short sentences and omit words. A person might say, "Want food" or "Walk park today." A listener can usually understand the meaning, but people with this aphasia pattern are often aware of their difficulty communicating and may get frustrated”. We do not distinguish between aphasias caused by cerebrovascular accidents and neurodegenerative diseases or other causes. Mayo Clinic. Aphasia. Mayo Clinic. https://www.mayoclinic.org/diseases-conditions/aphasia/symptoms-causes/syc-20369518. Published 2021. Accessed 11 August 2021. We scored as TRUE: only when it literally stated ‘vertigo'. We did NOT score: diagnoses such as Benign paroxysmal positional vertigo. These are scored in a separate section of the database. We did NOT score: any other words. These were scored as ‘Positive sensory symptoms’. We did NOT score: dizziness; dizzy. These were not scored anywhere (too subjective). We did NOT score: nystagmus. This was scored separately as 'Nystagmus'. Explanation: be aware of the subjective origin of this word in the summaries of the NBB. true UMLS: C0042571 Definition: “Vertigo is the sensation that the environment is spinning around relative to oneself (objective vertigo) or vice versa (subjective vertigo). The term is sometimes used erroneously to mean any form of dizziness. True vertigo is described as a rotary sensation of the patient or surroundings, and is often of vestibular origin.” Vertigo is the predominant symptom of vestibular dysfunction. Patients often experience vertigo as an illusion of motion; some interpret this as self-motion, others as motion of the environment. The most common motion illusion is a spinning sensation. A spinning sensation is not required; vertigo can also be a sense of swaying or tilting. Also, not all patients are able to describe their vertigo in such concrete terms. Vague dizziness, imbalance, or disorientation may eventually prove to be due to a vestibular problem. Vertigo is often present in combination with nystagmus. In the clinical summaries of the NBB, the word vertigo is usually used to indicate problems in the vestibular system, but also frequently as a symptom the patient reported. In the latter it could remain unknown what the origin of this symptom ‘vertigo’ is, as is could possibly be of an cerebellar origin. Vertigo http://purl.obolibrary.org/obo/Vertigo Vertigo Vertigo UMLS: C0042571 Direct match Definition: “Vertigo is the sensation that the environment is spinning around relative to oneself (objective vertigo) or vice versa (subjective vertigo). The term is sometimes used erroneously to mean any form of dizziness. True vertigo is described as a rotary sensation of the patient or surroundings, and is often of vestibular origin.” Vertigo is the predominant symptom of vestibular dysfunction. Patients often experience vertigo as an illusion of motion; some interpret this as self-motion, others as motion of the environment. The most common motion illusion is a spinning sensation. A spinning sensation is not required; vertigo can also be a sense of swaying or tilting. Also, not all patients are able to describe their vertigo in such concrete terms. Vague dizziness, imbalance, or disorientation may eventually prove to be due to a vestibular problem. Vertigo is often present in combination with nystagmus. In the clinical summaries of the NBB, the word vertigo is usually used to indicate problems in the vestibular system, but also frequently as a symptom the patient reported. In the latter it could remain unknown what the origin of this symptom ‘vertigo’ is, as is could possibly be of an cerebellar origin. https://pubmed.ncbi.nlm.nih.gov/16946161/ BMJ. Overview of vertigo. BMJ Best Practice. https://bestpractice.bmj.com/topics/en-gb/965. Published 2018. Accessed 9 June, 2021. We scored as TRUE: visual problems/impairment; reduced vision; blurred vision; visus; reduced eye function. We did NOT score: diagnoses such as retinopathy; cataract; glaucoma; macular degeneration These were scored in a separate section of the database. We did NOT score: eye surgeries; diplopia; double vision; strabismus divergens; visual perception; visual-spatial; or any ambiguous term. These were not scored anywhere. We did NOT score: nystagmus – this was scored separately as ‘Nystagmus’. false UMLS: C3665347 Description: Any descriptions about visual impairment. This can be cause by eye problems, or visual cortex problems, we do not distinguish between them. Cataract is not one of them, as this is a diagnosis, and we cannot fully determine whether a donor had visual problems or not. And, a lot of patients get cataract surgery, after which we can assume the vision is better again. Visual problems Visual:impairment Visual_impairment Visual impairment Visual:impairment UMLS: C3665347 Direct match Here we only labeled TRUE when vivid dreams, or vivid dreaming is written down. We scored as TRUE: dreams; vivid dreams; vivid dreaming; lively dreaming; nightmares; reports of vigorous movements during sleep; everything that mentions a change in the dream or movement during sleep that are suddenly worth reporting. We did NOT score: REM-sleep Behavioral Disorder (RBD). These were scored in a separate section of the database. Explanation: Because we want to capture all the sentences that include something about dreams or nightmares, we decided to include all sentences with the word dream or nightmare. This might mean we do not only catch ‘vivid dreaming’, but together with the Clinical Diagnosis we can place the parameter into context. false UMLS: C0235966 Description: Vivid dreaming is frequently present in patients with Parkinson’s disease. Neurologists often ask the patient whether he/she has vivid dreams or frequent nightmares. Partners of patients are frequently asked about: whether the patient suffers from vigorous movements during sleep, which could indicate REM-sleep Behavioral Disorder (RBD) which is often present in patients with Parkinson’s disease. Dreaming excessive Vivid:dreaming Vivid_dreaming Vivid dreaming Vivid:dreaming UMLS: C0235966 UMLS code refers to a broader concept We scored as TRUE: wander; wandered; wandering; roamed; roaming. We did NOT score: restless walking; disoriented walking; anything else. true UMLS: C0033975 Definition: The term ‘wandering’ is basically defined as seemingly aimless or disoriented ambulating behavior of demented persons with dimensions of pattern (lapping, random, or pacing), frequency, boundary transgressions, and deficits in wayfinding. Wandering roaming Wandering:behavior Wandering_behavior Wandering behavior Wandering:behavior UMLS: C0033975 Direct match Definition: The term ‘wandering’ is basically defined as seemingly aimless or disoriented ambulating behavior of demented persons with dimensions of pattern (lapping, random, or pacing), frequency, boundary transgressions, and deficits in wayfinding. Kwak YT, Yang Y, Koo M-S. Wandering in dementia. Dementia and Neurocognitive Disorders. 2015;14(3):99-105. We scored as TRUE: any mention of loss body weight. false UMLS: C1262477 Description: “Several neurologic illnesses, including stroke, dementia, Parkinson disease, and amyotrophic lateral sclerosis, can lead to weight loss. Weight loss may be due to one or more deficits, such as altered cognition, motor dysfunction, and dysphagia, associated with these disorders”. Weight:loss Weight_loss Weight loss Weight:loss UMLS: C1262477 Direct match Description: “Several neurologic illnesses, including stroke, dementia, Parkinson disease, and amyotrophic lateral sclerosis, can lead to weight loss. Weight loss may be due to one or more deficits, such as altered cognition, motor dysfunction, and dysphagia, associated with these disorders”. Evans A, Gupta R. Approach to the patient with unintentional weight loss. UpToDate. 2016. https://www.uptodate.com/contents/approach-to-the-patient-with-unintentional-weight-loss. Published 2021. Accessed. 1.2 clinical_history