CDE Detailed Report

Disease: content
Sub-Domain: Emotional/Behavioral
CRF: welcome

Displaying 1 - 36 of 36
CDE ID CDE Name Variable Name Definition Short Description Additional Notes (Question Text) Permissible Values Description Data Type Disease Specific Instructions Disease Specific Reference Population Classification (e.g., Core) Version Number Version Date CRF Name (CRF Module / Guideline) Sub Domain Name Domain Name Size Input Restrictions Min Value Max Value Measurement Type External Id Loinc External Id Snomed External Id caDSR External Id CDISC
C17214 Problem Behaviours Assessment - Short Form (PBA-S) - delusions paranoid thinking severity score PBASDelusParanThnkSevScore Scores for severities of Delusions/Paranoid Thinking as part of the Problem Behaviours Assessment - Short Form (PBA-S). Scores for severities of Delusions/Paranoid Thinking as part of the Problem Behaviours Assessment - Short Form (PBA-S). Delusions/Paranoid Thinking Severity 0;1;2;3;4;8;9 Symptom absent;Questionable or trivial;Overvalued ideas (not amounting to true delusions) are present for some part of the day but do not affect subject's behaviour;Overvalued ideas are present for much of the day, and subject behaves as if these beliefs were true, although he/she can be persuaded (with difficulty) that he/she is mistaken;Delusions: false beliefs, held with unshakeable conviction, which are not shared by other members of subject's social and cultural group and have been present continuously for at least 7 days;Unable to assess because condition too advanced (e.g. mute and immobile);Absence of a reliable informant Numeric values

Rate the participant's/subject's average behavior over the past 4 weeks. Suggested Prompts: Any abnormal beliefs, including unfounded jealous suspicions and accusations of infidelity, should be rated here. I am going to ask you about unusual experiences that people sometimes have. In the past four weeks, has it ever seemed like people are out to get you or perhaps controlling you? Has it seemed like you have special powers or importance, or that books, TV and radio statements are referring to you? Are there any other unusual things you experience that I have not asked you about?

COPYRIGHT University of Ulm, Faculty of Medicine, EHDN Behavioural Phenotype Working Group (Version 1.4, February 2007). For enquiries, contact permissions@euro-hd.net A paper describing the reliability of the PBA-s (in English, Dutch and French) based on data from the TRACK-HD study is in preparation and will be submitted shortly. Craufurd D, Thompson J, Snowden JS. Behavioural changes in Huntington's disease: the Problem Behaviours Assessment. Neuropsychiatry, Neuropsychology and Behavioural Neurology 2001; 14 (4): 219-226. Adult Supplemental 3.00 2013-07-14 19:48:50.273 Problem Behaviours Assessment HD - Short Version (PBA-s) Emotional/Behavioral Outcomes and End Points

Single Pre-Defined Value Selected

C17193 Problem Behaviours Assessment - Short Form (PBA-S) - suicidal ideation severity score PBASSucidIdeaSevScore The severity score for suicidal ideation over past four weeks as part of the Problem Behaviours Assessment - Short Form (PBA-S). The severity score for suicidal ideation over past four weeks as part of the Problem Behaviours Assessment - Short Form (PBA-S). Suicidal Ideation Severity 0;1;2;3;4;8 Absent;Questionable. also rate 1 if subject plans suicide at a later date when disease is more severe but obtains comfort from this as means to retain control of destiny;Sometimes very pessimistic with fleeting suicidal ideation;Subject has pervasive and distressing feelings of hopelessness and more prolonged or frequent suicidal ideation, but has not yet acted on this in any way;Subject has attempted suicide or has made preparations such as saving up tablets or planning ways to avoid discovery when doing it;Unable to assess because condition too advanced (e.g. mute and immobile) Numeric values

Rate the participant's/subject's average behavior over the past 4 weeks. If the symptom has NOT been present in the past 4 weeks, or the rating for the past month does not adequately reflect symptom severity in the interval since the patient's last assessment, use the third box to rate the WORST level of severity during the interval since patient was last seen. Suggested prompts: In the past four weeks, have you felt that life was not worth living or that you wouldn't care if you didn't wake in the morning? Have you found yourself thinking that life is not worth living or that you would be better off dead? Have you thought about harming yourself or even making an attempt at suicide? Are you planning to hurt yourself or kill yourself? Have you taken any steps towards carrying out your plan?

COPYRIGHT University of Ulm, Faculty of Medicine, EHDN Behavioural Phenotype Working Group (Version 1.4, February 2007). For enquiries, contact permissions@euro-hd.net A paper describing the reliability of the PBA-s (in English, Dutch and French) based on data from the TRACK-HD study is in preparation and will be submitted shortly. Craufurd D, Thompson J, Snowden JS. Behavioural changes in Huntington's disease: the Problem Behaviours Assessment. Neuropsychiatry, Neuropsychology and Behavioural Neurology 2001; 14 (4): 219-226. Adult Supplemental 3.00 2013-07-14 19:48:50.273 Problem Behaviours Assessment HD - Short Version (PBA-s) Emotional/Behavioral Outcomes and End Points

Single Pre-Defined Value Selected

C17225 Problem Behaviours Assessment - Short Form (PBA-S) - informant household member status PBASInfoHousMembStatus The status whether the informant is a member of the participant's/subject's household as part of the Problem Behaviours Assessment - Short Form (PBA-S). The status whether the informant is a member of the participant's/subject's household as part of the Problem Behaviours Assessment - Short Form (PBA-S). Is informant a household member? 1;2;3;4 Household member (i.e. relative or friend who lives with subject);Not a household member but has frequent contact with subject (most days);Not a household member and sees subject less than three or four times a week;Staff of residential care home or hospital Numeric values

Codes for informant details.Categories 2 and 3 could apply to family, friends or professional care workers. A paid carer who stays with subject at home for three hours every weekday would be rated as 2

COPYRIGHT University of Ulm, Faculty of Medicine, EHDN Behavioural Phenotype Working Group (Version 1.4, February 2007). For enquiries, contact permissions@euro-hd.net A paper describing the reliability of the PBA-s (in English, Dutch and French) based on data from the TRACK-HD study is in preparation and will be submitted shortly. Craufurd D, Thompson J, Snowden JS. Behavioural changes in Huntington's disease: the Problem Behaviours Assessment. Neuropsychiatry, Neuropsychology and Behavioural Neurology 2001; 14 (4): 219-226. Adult Supplemental 3.00 2013-07-14 19:48:50.273 Problem Behaviours Assessment HD - Short Version (PBA-s) Emotional/Behavioral Outcomes and End Points

Single Pre-Defined Value Selected

C17204 Problem Behaviours Assessment - Short Form (PBA-S) - angry aggressive behaviour worst score PBASAngrAggrBehavWrstScore The worst score for angry or aggressive behaviour as part of the Problem Behaviours Assessment - Short Form (PBA-S). The worst score for angry or aggressive behaviour as part of the Problem Behaviours Assessment - Short Form (PBA-S). Angry or Aggressive Behaviour Worst 0;1;2;3;4 Normal;Questionable;Verbal outbursts which are outside socially acceptable limits but do not cause significant problems or distress for other household members;Temper tantrums are severe enough to cause significant distress for other household members and/or practical difficulties caring for the subject;Subject has temper tantrums so severe that relationship with carers is compromised, creating risk that subject will be rejected Numeric values

Score "WORST" box if the rating for the past month does not adequately reflect symptom severity in the interval since the participant's/subject's last assessment, rate the WORST level of severity during the interval since the participant/subject was last seen. Rate 2 for mild anticipatory anxiety prior to social events or unfamiliar activities e.g. hospital appointments. Rate 2 if subject becomes angry with self or inanimate objects when confronted with frustrating situations due to disability, such as failure when attempting to rewire a plug. Rate 3 when verbal hostility or anger is directed towards another person (e.g. shouting, sarcastic name-calling, use of foul or abusive language). Also rate 3 if there are explicit verbal threats of violence to another person, or behavior causing a justifiable fear of personal violence (e.g. subject approaches too close, raises fist, mild pushing). Also rate 3 for violence towards property. Rate 4 if there has been any kind of actual physical assault (includes pushing, shoving, hitting, biting, scratching, kicking) or threatening behavior involving weapons.

COPYRIGHT University of Ulm, Faculty of Medicine, EHDN Behavioural Phenotype Working Group (Version 1.4, February 2007). For enquiries, contact permissions@euro-hd.net A paper describing the reliability of the PBA-s (in English, Dutch and French) based on data from the TRACK-HD study is in preparation and will be submitted shortly. Craufurd D, Thompson J, Snowden JS. Behavioural changes in Huntington's disease: the Problem Behaviours Assessment. Neuropsychiatry, Neuropsychology and Behavioural Neurology 2001; 14 (4): 219-226. Adult Supplemental 3.00 2013-07-14 19:48:50.273 Problem Behaviours Assessment HD - Short Version (PBA-s) Emotional/Behavioral Outcomes and End Points

Single Pre-Defined Value Selected

C17215 Problem Behaviours Assessment - Short Form (PBA-S) - delusions paranoid thinking frequency score PBASDelusParanThnkFrqScore The frequency score for Delusions/Paranoid Thinking as part of the Problem Behaviours Assessment - Short Form (PBA-S). The frequency score for Delusions/Paranoid Thinking as part of the Problem Behaviours Assessment - Short Form (PBA-S). Delusions/Paranoid Thinking Frequency 0;1;2;3;4;9 Never/almost never;Seldom (less than once/week);Sometimes (up to 4 times a week);Frequently (most days/5,6 or 7 times a week);Daily/almost daily for most (or all) of day;Not known or not applicable Numeric values

Rate the participant's/subject's average behavior over the past 4 weeks. suggested prompts: Any abnormal beliefs, including unfounded jealous suspicions and accusations of infidelity, should be rated here. I am going to ask you about unusual experiences that people sometimes have. In the past four weeks, has it ever seemed like people are out to get you or perhaps controlling you? Has it seemed like you have special powers or importance, or that books, TV and radio statements are referring to you? Are there any other unusual things you experience that I have not asked you about?

COPYRIGHT University of Ulm, Faculty of Medicine, EHDN Behavioural Phenotype Working Group (Version 1.4, February 2007). For enquiries, contact permissions@euro-hd.net A paper describing the reliability of the PBA-s (in English, Dutch and French) based on data from the TRACK-HD study is in preparation and will be submitted shortly. Craufurd D, Thompson J, Snowden JS. Behavioural changes in Huntington's disease: the Problem Behaviours Assessment. Neuropsychiatry, Neuropsychology and Behavioural Neurology 2001; 14 (4): 219-226. Adult Supplemental 3.00 2013-07-14 19:48:50.273 Problem Behaviours Assessment HD - Short Version (PBA-s) Emotional/Behavioral Outcomes and End Points

Single Pre-Defined Value Selected

C17194 Problem Behaviours Assessment - Short Form (PBA-S) - suicidal ideation frequency score PBASSucidIdeaFreqScore The frequency score for suicidal ideation over past four weeks as part of the Problem Behaviours Assessment - Short Form (PBA-S). The frequency score for suicidal ideation over past four weeks as part of the Problem Behaviours Assessment - Short Form (PBA-S). Suicidal Ideation Frequency 0;1;2;3;4;9 Never/almost never;Seldom (less than once/week);Sometimes (up to 4 times a week);Frequently (most days/5,6 or 7 times a week);Daily/almost daily for most (or all) of day;Not known or not applicable Numeric values

Rate the participant's/subject's average behavior over the past 4 weeks. If the symptom has NOT been present in the past 4 weeks, or the rating for the past month does not adequately reflect symptom severity in the interval since the patient's last assessment, use the third box to rate the WORST level of severity during the interval since patient was last seen. Suggested prompts: In the past four weeks, have you felt that life was not worth living or that you wouldn't care if you didn't wake in the morning? Have you found yourself thinking that life is not worth living or that you would be better off dead? Have you thought about harming yourself or even making an attempt at suicide? Are you planning to hurt yourself or kill yourself? Have you taken any steps towards carrying out your plan?

COPYRIGHT University of Ulm, Faculty of Medicine, EHDN Behavioural Phenotype Working Group (Version 1.4, February 2007). For enquiries, contact permissions@euro-hd.net A paper describing the reliability of the PBA-s (in English, Dutch and French) based on data from the TRACK-HD study is in preparation and will be submitted shortly. Craufurd D, Thompson J, Snowden JS. Behavioural changes in Huntington's disease: the Problem Behaviours Assessment. Neuropsychiatry, Neuropsychology and Behavioural Neurology 2001; 14 (4): 219-226. Adult Supplemental 3.00 2013-07-14 19:48:50.273 Problem Behaviours Assessment HD - Short Version (PBA-s) Emotional/Behavioral Outcomes and End Points

Single Pre-Defined Value Selected

C17205 Problem Behaviours Assessment - Short Form (PBA-S) - lack initiative apathy severity score PBASLckInitApathSevScore The severity score for lack of initiative (apathy) as part of the Problem Behaviours Assessment - Short Form (PBA-S). The severity score for lack of initiative (apathy) as part of the Problem Behaviours Assessment - Short Form (PBA-S). Lack of Initiative (Apathy) Severity 0;1;2;3;4;8;9 Symptom absent;Questionable;Subject no longer tries new things. May need gentle prompting to initiate hobbies or pastimes which he/she usually enjoys, makes less effort to keep up with friends and relatives, tends to put off household tasks which were previously part of normal daily routine and may need gentle prompting to do these things;Needs quite overt prompting to take part in hobbies or pastimes which he/she used to enjoy, or to carry out routine daily household tasks, makes little or no effort to keep up with friends and leaves it to others to initiate any social contacts, able to take part in (and apparently enjoy) conversation but tends to follow and is less likely to initiate a change of subject.;No longer performs any household tasks, even if prompted repeatedly, never initiates activities, and displays no interest in hobbies or pastimes, markedly impoverished speech, rarely initiates new topics of conversation except in relation to own needs, active choices limited to selecting TV programmes to watch and perhaps switching on or changing channel to do this);Unable to assess because condition too advanced (e.g. mute and immobile);Missing data Numeric values

Rate the participant's/subject's average behavior over the past 4 weeks. Suggested prompts: In the past four weeks, have you found that you have lost interest in things that used to be important to you? Are you just as interested as always in trying new things, starting new projects? Do you have to be pushed to get started on chores that need doing? Do you leave it to friends to take the initiative for organizing social activities? Do you sit around a lot doing nothing?

COPYRIGHT University of Ulm, Faculty of Medicine, EHDN Behavioural Phenotype Working Group (Version 1.4, February 2007). For enquiries, contact permissions@euro-hd.net A paper describing the reliability of the PBA-s (in English, Dutch and French) based on data from the TRACK-HD study is in preparation and will be submitted shortly. Craufurd D, Thompson J, Snowden JS. Behavioural changes in Huntington's disease: the Problem Behaviours Assessment. Neuropsychiatry, Neuropsychology and Behavioural Neurology 2001; 14 (4): 219-226. Adult Supplemental 3.00 2013-07-14 19:48:50.273 Problem Behaviours Assessment HD - Short Version (PBA-s) Emotional/Behavioral Outcomes and End Points

Single Pre-Defined Value Selected

C17216 Problem Behaviours Assessment - Short Form (PBA-S) - delusions paranoid thinking worst score PBASDelusParanThnkWrstScore The worst score for Delusions/Paranoid Thinking as part of the Problem Behaviours Assessment - Short Form (PBA-S). The worst score for Delusions/Paranoid Thinking as part of the Problem Behaviours Assessment - Short Form (PBA-S). Delusions/Paranoid Thinking Worst 0;1;2;3;4;8;9 Symptom absent;Questionable or trivial;Overvalued ideas (not amounting to true delusions) are present for some part of the day but do not affect subject's behaviour;Overvalued ideas are present for much of the day, and subject behaves as if these beliefs were true, although he/she can be persuaded (with difficulty) that he/she is mistaken;Delusions: false beliefs, held with unshakeable conviction, which are not shared by other members of subject's social and cultural group and have been present continuously for at least 7 days;Unable to assess because condition too advanced (e.g. mute and immobile);Absence of a reliable informant Numeric Values

Score "WORST" box if the rating for the past month does not adequately reflect symptom severity in the interval since the participant's/subject's last assessment, rate the WORST level of severity during the interval since the participant/subject was last seen. This item will usually be rated 9 (data missing) in the absence of a reliable informant.

COPYRIGHT University of Ulm, Faculty of Medicine, EHDN Behavioural Phenotype Working Group (Version 1.4, February 2007). For enquiries, contact permissions@euro-hd.net A paper describing the reliability of the PBA-s (in English, Dutch and French) based on data from the TRACK-HD study is in preparation and will be submitted shortly. Craufurd D, Thompson J, Snowden JS. Behavioural changes in Huntington's disease: the Problem Behaviours Assessment. Neuropsychiatry, Neuropsychology and Behavioural Neurology 2001; 14 (4): 219-226. Adult Supplemental 3.00 2013-07-14 19:48:50.273 Problem Behaviours Assessment HD - Short Version (PBA-s) Emotional/Behavioral Outcomes and End Points

Single Pre-Defined Value Selected

C17195 Problem Behaviours Assessment - Short Form (PBA-S) - suicidal ideation worst score PBASSucidIdeaWrstScore The worst score for suicidal ideation since patient was last seen as part of the Problem Behaviours Assessment - Short Form (PBA-S). The worst score for suicidal ideation since patient was last seen as part of the Problem Behaviours Assessment - Short Form (PBA-S). Suicidal Ideation Worst 0;1;2;3;4;8 Absent;Questionable. also rate 1 if subject plans suicide at a later date when disease is more severe but obtains comfort from this as means to retain control of destiny;Sometimes very pessimistic with fleeting suicidal ideation;Subject has pervasive and distressing feelings of hopelessness and more prolonged or frequent suicidal ideation, but has not yet acted on this in any way;Subject has attempted suicide or has made preparations such as saving up tablets or planning ways to avoid discovery when doing it;Unable to assess because condition too advanced (e.g. mute and immobile) Numeric values

Score "WORST" box if the rating for the past month does not adequately reflect symptom severity in the interval since the participant's/subject's last assessment, rate the WORST level of severity during the interval since the participant/subject was last seen.

COPYRIGHT University of Ulm, Faculty of Medicine, EHDN Behavioural Phenotype Working Group (Version 1.4, February 2007). For enquiries, contact permissions@euro-hd.net A paper describing the reliability of the PBA-s (in English, Dutch and French) based on data from the TRACK-HD study is in preparation and will be submitted shortly. Craufurd D, Thompson J, Snowden JS. Behavioural changes in Huntington's disease: the Problem Behaviours Assessment. Neuropsychiatry, Neuropsychology and Behavioural Neurology 2001; 14 (4): 219-226. Adult Supplemental 3.00 2013-07-14 19:48:50.273 Problem Behaviours Assessment HD - Short Version (PBA-s) Emotional/Behavioral Outcomes and End Points

Single Pre-Defined Value Selected

C17206 Problem Behaviours Assessment - Short Form (PBA-S) - lack initiative apathy frequency score PBASLckInitApathFreqScore The frequency score for lack of initiative (apathy) as part of the Problem Behaviours Assessment - Short Form (PBA-S). The frequency score for lack of initiative (apathy) as part of the Problem Behaviours Assessment - Short Form (PBA-S). Lack of Initiative (Apathy) Frequency 0;1;2;3;4;9 Never/almost never;Seldom (less than once/week);Sometimes (up to 4 times a week);Frequently (most days/5,6 or 7 times a week);Daily/almost daily for most (or all) of day;Not known or not applicable Numeric values

Rate the participant's/subject's average behavior over the past 4 weeks. In the past four weeks, have you found that you have lost interest in things that used to be important to you? Are you just as interested as always in trying new things, starting new projects? Do you have to be pushed to get started on chores that need doing? Do you leave it to friends to take the initiative for organizing social activities? Do you sit around a lot doing nothing?

COPYRIGHT University of Ulm, Faculty of Medicine, EHDN Behavioural Phenotype Working Group (Version 1.4, February 2007). For enquiries, contact permissions@euro-hd.net A paper describing the reliability of the PBA-s (in English, Dutch and French) based on data from the TRACK-HD study is in preparation and will be submitted shortly. Craufurd D, Thompson J, Snowden JS. Behavioural changes in Huntington's disease: the Problem Behaviours Assessment. Neuropsychiatry, Neuropsychology and Behavioural Neurology 2001; 14 (4): 219-226. Adult Supplemental 3.00 2013-07-14 19:48:50.273 Problem Behaviours Assessment HD - Short Version (PBA-s) Emotional/Behavioral Outcomes and End Points

Single Pre-Defined Value Selected

C17217 Problem Behaviours Assessment - Short Form (PBA-S) - hallucinations severity score PBASHallucSevScore The severity score for Hallucinations as part of the Problem Behaviours Assessment - Short Form (PBA-S). The severity score for Hallucinations as part of the Problem Behaviours Assessment - Short Form (PBA-S). Hallucinations Severity 0;1;2;3;4;8 Symptom absent;Questionable or trivial;Subject reports experiencing hallucinations (when asked) but these do not appear to cause any distress or affect subject's behaviour;Hallucinations which affect subject's behaviour (e.g. looking for source of hidden voices or putting cotton wool in ears) but do not appear to cause much distress;Subject is clearly distressed by hallucinations and preoccupied with them;Unable to assess because condition too advanced (e.g. mute and immobile) Numeric values

Rate the participant's/subject's average behavior over the past 4 weeks. Suggested prompts: In the past four weeks, have you heard things that other people could not hear such as noises or voices of people whispering or talking? Did you ever have visions (when you were awake) or see things that other people could not see? How about any other strange sensations in or on your body? Have you noticed any strange smell or taste that other people seem unable to detect?

COPYRIGHT University of Ulm, Faculty of Medicine, EHDN Behavioural Phenotype Working Group (Version 1.4, February 2007). For enquiries, contact permissions@euro-hd.net A paper describing the reliability of the PBA-s (in English, Dutch and French) based on data from the TRACK-HD study is in preparation and will be submitted shortly. Craufurd D, Thompson J, Snowden JS. Behavioural changes in Huntington's disease: the Problem Behaviours Assessment. Neuropsychiatry, Neuropsychology and Behavioural Neurology 2001; 14 (4): 219-226. Adult Supplemental 3.00 2013-07-14 19:48:50.273 Problem Behaviours Assessment HD - Short Version (PBA-s) Emotional/Behavioral Outcomes and End Points

Single Pre-Defined Value Selected

C17196 Problem Behaviours Assessment - Short Form (PBA-S) - anxiety severity score PBASAnxSevScore The severity score for anxiety as part of the Problem Behaviours Assessment - Short Form (PBA-S). The severity score for anxiety as part of the Problem Behaviours Assessment - Short Form (PBA-S). Anxiety Severity 0;1;2;3;4;8 Absent;Questionable, vague unease (also rate 1 if subject's only worry or anxiety is about prognosis of HD);Subject experiences intermittent worry or anxiety, but symptom is not severe enough to cause significant distress or interfere with everyday activities. rate 2 for mild anticipatory anxiety prior to social events or unfamiliar activities e.g. hospital appointments;Unpleasant anxiety is present much of the time, and has a significant impact on subject's behaviour (e.g. avoids going to places or events associated with provoking anxiety);Worry, anxiety or panic are present all the time and have a major impact on subject's lifestyle (e.g. agoraphobia such that subject cannot leave home without an escort);Unable to assess because condition too advanced (e.g. mute and immobile) Numeric values

Rate the participant's/subject's avearge behavior over the past 4 weeks.If the symptom has NOT been present in the past 4 weeks, or the rating for the past month does not adequately reflect symptom severity in the interval since the patient's last assessment, use the third box to rate the WORST level of severity during the interval since patient was last seen. Suggested prompts: In the past four weeks, have you found yourself getting worried about things? (Evidence of anxiety includes worrying, panic, feeling frightened or fearful for no apparent reason). Further questions: In the past four weeks, have you found yourself getting worried about things? (Evidence of anxiety includes worrying, panic, feeling frightened or fearful for no apparent reason). Have you been worrying a great deal? What is it like when you worry? Have you often felt on edge, or keyed up, or mentally strained? Have you had difficulty in relaxing? Do your muscles feel tensed up? When people get anxious or panicky they often feel their heart beating fast or they start shaking or sweating or can't get their breath. Have you had feelings like that?

COPYRIGHT University of Ulm, Faculty of Medicine, EHDN Behavioural Phenotype Working Group (Version 1.4, February 2007). For enquiries, contact permissions@euro-hd.net A paper describing the reliability of the PBA-s (in English, Dutch and French) based on data from the TRACK-HD study is in preparation and will be submitted shortly. Craufurd D, Thompson J, Snowden JS. Behavioural changes in Huntington's disease: the Problem Behaviours Assessment. Neuropsychiatry, Neuropsychology and Behavioural Neurology 2001; 14 (4): 219-226. Adult Supplemental 3.00 2013-07-14 19:48:50.273 Problem Behaviours Assessment HD - Short Version (PBA-s) Emotional/Behavioral Outcomes and End Points

Single Pre-Defined Value Selected

C17207 Problem Behaviours Assessment - Short Form (PBA-S) - lack initiative apathy worst score PBASLckInitApathWrstScore The worst score for lack of initiative (apathy) as part of the Problem Behaviours Assessment - Short Form (PBA-S). The worst score for lack of initiative (apathy) as part of the Problem Behaviours Assessment - Short Form (PBA-S). Lack of Initiative (Apathy) Worst 0;1;2;3;4;8;9 Symptom absent;Questionable;Subject no longer tries new things|may need gentle prompting to initiate hobbies or pastimes which he/she usually enjoys|makes less effort to keep up with friends and relatives|tends to put off household tasks which were previously part of normal daily routine and may need gentle prompting to do these things;Needs quite overt prompting to take part in hobbies or pastimes which he/she used to enjoy, or to carry out routine daily household tasks|makes little or no effort to keep up with friends and leaves it to others to initiate any social contacts|able to take part in (and apparently enjoy) conversation, but tends to follow and is less likely to initiate a change of subject;No longer performs any household tasks, even if prompted repeatedly.never initiates activities, and displays no interest in hobbies or pastimes.markedly impoverished speech, rarely initiates new topics of conversation except in relation to own needs.active choices limited to selecting TV programmes to watch, and perhaps switching on or changing channel to do this);Unable to assess because condition too advanced (e.g. mute and immobile);Absence of reliable information Numeric values

Score "WORST" box if the rating for the past month does not adequately reflect symptom severity in the interval since the participant's/subject's last assessment, rate the WORST level of severity during the interval since the participant/subject was last seen. Rate 2 for mild anticipatory anxiety prior to social events or unfamiliar activities e.g. hospital appointments. This item will usually be rated 9 (data missing) in the absence of a reliable informant.

COPYRIGHT University of Ulm, Faculty of Medicine, EHDN Behavioural Phenotype Working Group (Version 1.4, February 2007). For enquiries, contact permissions@euro-hd.net A paper describing the reliability of the PBA-s (in English, Dutch and French) based on data from the TRACK-HD study is in preparation and will be submitted shortly. Craufurd D, Thompson J, Snowden JS. Behavioural changes in Huntington's disease: the Problem Behaviours Assessment. Neuropsychiatry, Neuropsychology and Behavioural Neurology 2001; 14 (4): 219-226. Adult Supplemental 3.00 2013-07-14 19:48:50.273 Problem Behaviours Assessment HD - Short Version (PBA-s) Emotional/Behavioral Outcomes and End Points

Single Pre-Defined Value Selected

C17218 Problem Behaviours Assessment - Short Form (PBA-S) - hallucinations frequency score PBASHallucFreqScore The frequency score for hallucinations as part of the Problem Behaviours Assessment - Short Form (PBA-S). The frequency score for hallucinations as part of the Problem Behaviours Assessment - Short Form (PBA-S). Hallucinations Fequency 0;1;2;3;4;9 Never/almost never;Seldom (less than once/week);Sometimes (up to 4 times a week);Frequently (most days/5,6 or 7 times a week);Daily/almost daily for most (or all) of day;Not known or not applicable Numeric values

Rate the participant's/subject's average behavior over the past 4 weeks. Suggested prompts: In the past four weeks, have you heard things that other people could not hear such as noises or voices of people whispering or talking? Did you ever have visions (when you were awake) or see things that other people could not see? How about any other strange sensations in or on your body? Have you noticed any strange smell or taste that other people seem unable to detect?

COPYRIGHT University of Ulm, Faculty of Medicine, EHDN Behavioural Phenotype Working Group (Version 1.4, February 2007). For enquiries, contact permissions@euro-hd.net A paper describing the reliability of the PBA-s (in English, Dutch and French) based on data from the TRACK-HD study is in preparation and will be submitted shortly. Craufurd D, Thompson J, Snowden JS. Behavioural changes in Huntington's disease: the Problem Behaviours Assessment. Neuropsychiatry, Neuropsychology and Behavioural Neurology 2001; 14 (4): 219-226. Adult Supplemental 3.00 2013-07-14 19:48:50.273 Problem Behaviours Assessment HD - Short Version (PBA-s) Emotional/Behavioral Outcomes and End Points

Single Pre-Defined Value Selected

C17197 Problem Behaviours Assessment - Short Form (PBA-S) - anxiety frequency score PBASAnxFrqScore The frequency score for anxiety as part of the Problem Behaviours Assessment - Short Form (PBA-S). The frequency score for anxiety as part of the Problem Behaviours Assessment - Short Form (PBA-S). Anxiety Frequency 0;1;2;3;4;9 Never/almost never;Seldom (less than once/week);Sometimes (up to 4 times a week);Frequently (most days/5,6 or 7 times a week);Daily/almost daily for most (or all) of day;Not known or not applicable Numeric values

Rate the participant's/subject's average behavior over the past 4 weeks.If the symptom has NOT been present in the past 4 weeks, or the rating for the past month does not adequately reflect symptom severity in the interval since the patient's last assessment, use the third box to rate the WORST level of severity during the interval since patient was last seen. Suggested prompts: In the past four weeks, have you found yourself getting worried about things? (Evidence of anxiety includes worrying, panic, feeling frightened or fearful for no apparent reason). Further questions: In the past four weeks, have you found yourself getting worried about things? (Evidence of anxiety includes worrying, panic, feeling frightened or fearful for no apparent reason). Have you been worrying a great deal? What is it like when you worry? Have you often felt on edge, or keyed up, or mentally strained? Have you had difficulty in relaxing? Do your muscles feel tensed up? When people get anxious or panicky they often feel their heart beating fast or they start shaking or sweating or can't get their breath. Have you had feelings like that?

COPYRIGHT University of Ulm, Faculty of Medicine, EHDN Behavioural Phenotype Working Group (Version 1.4, February 2007). For enquiries, contact permissions@euro-hd.net A paper describing the reliability of the PBA-s (in English, Dutch and French) based on data from the TRACK-HD study is in preparation and will be submitted shortly. Craufurd D, Thompson J, Snowden JS. Behavioural changes in Huntington's disease: the Problem Behaviours Assessment. Neuropsychiatry, Neuropsychology and Behavioural Neurology 2001; 14 (4): 219-226. Adult Supplemental 3.00 2013-07-14 19:48:50.273 Problem Behaviours Assessment HD - Short Version (PBA-s) Emotional/Behavioral Outcomes and End Points

Single Pre-Defined Value Selected

C17208 Problem Behaviours Assessment - Short Form (PBA-S) - perseverative thinking behavior severity score PBASPersevThnkBehavSevScore The severity score for Perseverative Thinking or Behaviour as part of the Problem Behaviours Assessment - Short Form (PBA-S). The severity score for Preservative Thinking or Behaviour as part of the Problem Behaviours Assessment - Short Form (PBA-S). Perseverative Thinking or Behaviour Severity 0;1;2;3;4;8;9 Symptom absent;Questionable;Mild perseverative behaviours or abnormal preoccupations are present but do not interfere with everyday life or cause significant distress for subject or carers;Abnormal preoccupations or repetitive behaviours occupy a significant proportion of subject's attention and cause distress for subject or practical problems for carers;Abnormal preoccupations occupy most of subject's attention for several days at a time, causing major problems or distress for subject and carers, or subject cannot be diverted from repetitive behaviours (pacing, smoking, repeatedly visiting the toilet) which interfere significantly with everyday care;Unable to assess because condition too advanced (e.g. mute and immobile);Absence of a reliable informant Numeric values

Rate the participant's/subject's average behavior over the past 4 weeks. Suggested prompts: In the past four weeks, have you found yourself getting stuck on certain ideas or actions? Have your family or friends complained that you are getting obsessed about something, or going on about it more than you should, or doing something over and over again? rate 2 if carer reports that subject tends to come out with comments which refer to an earlier topic of conversation, or when rater observes perseverative phenomena during examination (e.g. continues tandem walking after test completed). Rate 3 if carers report that subject will not let matter drop after an argument, and keeps returning to the same contentious issue all day, or has repetitive behaviours (see below) which cause some interference with everyday care

COPYRIGHT University of Ulm, Faculty of Medicine, EHDN Behavioural Phenotype Working Group (Version 1.4, February 2007). For enquiries, contact permissions@euro-hd.net A paper describing the reliability of the PBA-s (in English, Dutch and French) based on data from the TRACK-HD study is in preparation and will be submitted shortly. Craufurd D, Thompson J, Snowden JS. Behavioural changes in Huntington's disease: the Problem Behaviours Assessment. Neuropsychiatry, Neuropsychology and Behavioural Neurology 2001; 14 (4): 219-226. Adult Supplemental 3.00 2013-07-14 19:48:50.273 Problem Behaviours Assessment HD - Short Version (PBA-s) Emotional/Behavioral Outcomes and End Points

Single Pre-Defined Value Selected

C17219 Problem Behaviours Assessment - Short Form (PBA-S) - hallucinations worst score PBASHallucWrstScore The worst score for hallucinations as part of the Problem Behaviours Assessment - Short Form (PBA-S). The worst score for hallucinations as part of the Problem Behaviours Assessment - Short Form (PBA-S). Hallucinations Worst 0;1;2;3;4;8 Symptom absent;Questionable or trivial;Subject reports experiencing hallucinations (when asked) but these do not appear to cause any distress or affect subject's behaviour;Hallucinations which affect subject's behaviour (e.g. looking for source of hidden voices or putting cotton wool in ears) but do not appear to cause much distress;Subject is clearly distressed by hallucinations and preoccupied with them;Unable to assess because condition too advanced (e.g. mute and immobile) Numeric Values

Score "WORST" box if the rating for the past month does not adequately reflect symptom severity in the interval since the participant's/subject's last assessment, rate the WORST level of severity during the interval since the participant/subject was last seen. See instrument for additional instructions and scoring guide.

COPYRIGHT University of Ulm, Faculty of Medicine, EHDN Behavioural Phenotype Working Group (Version 1.4, February 2007). For enquiries, contact permissions@euro-hd.net A paper describing the reliability of the PBA-s (in English, Dutch and French) based on data from the TRACK-HD study is in preparation and will be submitted shortly. Craufurd D, Thompson J, Snowden JS. Behavioural changes in Huntington's disease: the Problem Behaviours Assessment. Neuropsychiatry, Neuropsychology and Behavioural Neurology 2001; 14 (4): 219-226. Adult Supplemental 3.00 2013-07-14 19:48:50.273 Problem Behaviours Assessment HD - Short Version (PBA-s) Emotional/Behavioral Outcomes and End Points

Single Pre-Defined Value Selected

C17198 Problem Behaviours Assessment - Short Form (PBA-S) - anxiety worst score PBASAnxWrstScore The worst score for anxiety since patient was last seen as part of the Problem Behaviours Assessment - Short Form (PBA-S). The worst score for anxiety since patient was last seen as part of the Problem Behaviours Assessment - Short Form (PBA-S). Anxiety Worst 0;1;2;3;4;8 Absent;Questionable, vague unease (also rate 1 if subject's only worry or anxiety is about prognosis of HD);Subject experiences intermittent worry or anxiety, but symptom is not severe enough to cause significant distress or interfere with everyday activities;Unpleasant anxiety is present much of the time, and has a significant impact on subject's behavior (e.g. avoids going to places or events associated with provoking anxiety);Worry, anxiety or panic are present all the time and have a major impact on subject's lifestyle (e.g. agoraphobia such that subject cannot leave home without an escort);Unable to assess because condition too advanced (e.g. mute and immobile) Numeric values

Score "WORST" box if the rating for the past month does not adequately reflect symptom severity in the interval since the participant's/subject's last assessment, rate the WORST level of severity during the interval since the participant/subject was last seen. Rate 2 for mild anticipatory anxiety prior to social events or unfamiliar activities e.g. hospital appointments.

© COPYRIGHT University of Ulm, Faculty of Medicine, EHDN Behavioural Phenotype Working Group (Version 1.4, February 2007). For enquiries, contact permissions@euro-hd.net A paper describing the reliability of the PBA-s (in English, Dutch and French) based on data from the TRACK-HD study is in preparation and will be submitted shortly. Craufurd D, Thompson J, Snowden JS. Behavioural changes in Huntington's disease: the Problem Behaviours Assessment. Neuropsychiatry, Neuropsychology and Behavioural Neurology 2001; 14 (4): 219-226. Adult Supplemental 3.00 2013-07-14 19:48:50.273 Problem Behaviours Assessment HD - Short Version (PBA-s) Emotional/Behavioral Outcomes and End Points

Single Pre-Defined Value Selected

C17209 Problem Behaviours Assessment - Short Form (PBA-S) - perseverative thinking behavior frequency score PBASPersevThnkBehavFrqScore The frequency score for Perseverative Thinking or Behaviour as part of the Problem Behaviours Assessment - Short Form (PBA-S). The frequency score for Preservative Thinking or Behaviour as part of the Problem Behaviours Assessment - Short Form (PBA-S). Perseverative Thinking or Behaviour Frequency 0;1;2;3;4;9 Never/almost never;Seldom (less than once/week);Sometimes (up to 4 times a week);Frequently (most days/5,6 or 7 times a week);Daily/almost daily for most (or all) of day;Not known or not applicable Numeric values

Rate the participant's/subject's average behavior over the past 4 weeks. In the past four weeks, have you found yourself getting stuck on certain ideas or actions? Have your family or friends complained that you are getting obsessed about something, or going on about it more than you should, or doing something over and over again?

COPYRIGHT University of Ulm, Faculty of Medicine, EHDN Behavioural Phenotype Working Group (Version 1.4, February 2007). For enquiries, contact permissions@euro-hd.net A paper describing the reliability of the PBA-s (in English, Dutch and French) based on data from the TRACK-HD study is in preparation and will be submitted shortly. Craufurd D, Thompson J, Snowden JS. Behavioural changes in Huntington's disease: the Problem Behaviours Assessment. Neuropsychiatry, Neuropsychology and Behavioural Neurology 2001; 14 (4): 219-226. Adult Supplemental 3.00 2013-07-14 19:48:50.273 Problem Behaviours Assessment HD - Short Version (PBA-s) Emotional/Behavioral Outcomes and End Points

Single Pre-Defined Value Selected

C17220 Problem Behaviours Assessment - Short Form (PBA-S) - hallucinations modality type PBASHallucModTyp The modality type of hallucinations as part of the Problem Behaviours Assessment - Short Form (PBA-S). The modality type of hallucinations as part of the Problem Behaviours Assessment - Short Form (PBA-S). Modality of hallucinations Auditory;Visual;Tactile;Olfactory;Gustatory Auditory;Visual;Tactile;Olfactory;Gustatory Alphanumeric

No instructions available

COPYRIGHT University of Ulm, Faculty of Medicine, EHDN Behavioural Phenotype Working Group (Version 1.4, February 2007). For enquiries, contact permissions@euro-hd.net A paper describing the reliability of the PBA-s (in English, Dutch and French) based on data from the TRACK-HD study is in preparation and will be submitted shortly. Craufurd D, Thompson J, Snowden JS. Behavioural changes in Huntington's disease: the Problem Behaviours Assessment. Neuropsychiatry, Neuropsychology and Behavioural Neurology 2001; 14 (4): 219-226. Adult Supplemental 3.00 2013-07-14 19:48:50.273 Problem Behaviours Assessment HD - Short Version (PBA-s) Emotional/Behavioral Outcomes and End Points

Single Pre-Defined Value Selected

C17199 Problem Behaviours Assessment - Short Form (PBA-S) - irritability severity score PBASIrritSevScore The severity score for irritability as part of the Problem Behaviours Assessment - Short Form (PBA-S). The severity score for irritability as part of the Problem Behaviours Assessment - Short Form (PBA-S). Irritability Severity 0;1;2;3;4 No more irritable than the average person;Questionable or trivial. within normal limits but worse than he/she used to be;Definitely more irritable than is reasonable but not to an extent which causes significant problems or distress for other household members.rate 2 if subject appeared to be in a bad mood, but rater considered that subject might have become angry if not treated with tact;Subject very irritable and looses temper over trivial matters. household members have to be careful what they say and do to avoid problems.rate 3 if subject's appearance and behaviour suggestive of angry mood, such that outbursts would almost certainly have occurred if care had not been taken to placate subject or to keep out of his/her way;Subject very irritable and looses temper without any obvious reason at all. living with him/her is like walking on eggshells Numeric values

Rate the participant's/subject's average behavior over the past 4 weeks. If the symptom has NOT been present in the past 4 weeks, or the rating for the past month does not adequately reflect symptom severity in the interval since the patient's last assessment, use the third box to rate the WORST level of severity during the interval since patient was last seen. This item is used to rate the ease with which the subject looses his/her temper, rather than the degree to which self-control is lost once the subject is angry (the latter is rated in the next item). It should also be used to record irritable moods which might have developed into an angry outburst if the carer had not acted with increased tact or discretion. In the past four weeks, have you been irritable, bad-tempered, moody or 'cranky'? Do you think you get cross more easily than you used to? (if yes to above) How does this affect the people around you? Do you think they treat you differently when you are like that?

COPYRIGHT University of Ulm, Faculty of Medicine, EHDN Behavioural Phenotype Working Group (Version 1.4, February 2007). For enquiries, contact permissions@euro-hd.net A paper describing the reliability of the PBA-s (in English, Dutch and French) based on data from the TRACK-HD study is in preparation and will be submitted shortly. Craufurd D, Thompson J, Snowden JS. Behavioural changes in Huntington's disease: the Problem Behaviours Assessment. Neuropsychiatry, Neuropsychology and Behavioural Neurology 2001; 14 (4): 219-226. Adult Supplemental 3.00 2013-07-14 19:48:50.273 Problem Behaviours Assessment HD - Short Version (PBA-s) Emotional/Behavioral Outcomes and End Points

Single Pre-Defined Value Selected

C17210 Problem Behaviours Assessment - Short Form (PBA-S) - perseverative thinking behaviour worst score PBASPersevThnkBehavWrstScore The worst score for Perseverative Thinking or Behaviour as part of the Problem Behaviours Assessment - Short Form (PBA-S). The worst score for Preservative Thinking or Behaviour as part of the Problem Behaviours Assessment - Short Form (PBA-S). Perseverative Thinking or Behaviour Worst 0;1;2;3;4;8;9 Symptom absent;Questionable;Mild perseverative behaviours or abnormal preoccupations are present but do not interfere with everyday life or cause significant distress for subject or carers;Abnormal preoccupations or repetitive behaviours occupy a significant proportion of subject's attention and cause distress for subject or practical problems for carers;Abnormal preoccupations occupy most of subject's attention for several days at a time, causing major problems or distress for subject and carers, or subject cannot be diverted from repetitive behaviours (pacing, smoking, repeatedly visiting the toilet) which interfere significantly with everyday care;Unable to assess because condition too advanced (e.g. mute and immobile);Absence of a reliable informant Numeric Values

Score "WORST" box if the rating for the past month does not adequately reflect symptom severity in the interval since the participant's/subject's last assessment, rate the WORST level of severity during the interval since the participant/subject was last seen. Rate 2 if carer reports that subject tends to come out with comments which refer to an earlier topic of conversation, or when rater observes perseverative phenomena during examination (e.g. continues tandem walking after test completed). Rate 3 if carers report that subject will not let matter drop after an argument, and keeps returning to the same contentious issue all day, or has repetitive behaviours (see below) which cause some interference with everyday care. This item will usually be rated 9 (data missing) in the absence of a reliable informant.

COPYRIGHT University of Ulm, Faculty of Medicine, EHDN Behavioural Phenotype Working Group (Version 1.4, February 2007). For enquiries, contact permissions@euro-hd.net A paper describing the reliability of the PBA-s (in English, Dutch and French) based on data from the TRACK-HD study is in preparation and will be submitted shortly. Craufurd D, Thompson J, Snowden JS. Behavioural changes in Huntington's disease: the Problem Behaviours Assessment. Neuropsychiatry, Neuropsychology and Behavioural Neurology 2001; 14 (4): 219-226. Adult Supplemental 3.00 2013-07-14 19:48:50.273 Problem Behaviours Assessment HD - Short Version (PBA-s) Emotional/Behavioral Outcomes and End Points

Single Pre-Defined Value Selected

C17221 Problem Behaviours Assessment - Short Form (PBA-S) - disoriented behaviour severity score PBASDisorientBehavSevScore The severity score for disoriented behaviour as part of the Problem Behaviours Assessment - Short Form (PBA-S). The severity score for disoriented behaviour as part of the Problem Behaviours Assessment - Short Form (PBA-S). Disoriented Behaviour Severity 0;1;2;3;4;8 Symptom absent;Questionable or trivial (e.g. subject gets day wrong ± one day, or fails to recognise people when meeting them out of their normal context);Subject does not seem to be fully aware of surroundings or the passage of time, but this does not cause significant practical problems;Evidence of confusion at night (subject appears disoriented in time, place or person to an extent that causes practical problems for carers) but normal during daylight hours;Subject is confused and disoriented all the time, unaware of time of day / day of week / date and wrongly identifying surroundings or the people around him (e.g. mistakes nursing home for a prison and nursing staff as prison warders) and consequently resists efforts of carers to look after him/her;Unable to assess because condition too advanced (e.g. mute and immobile) Numeric values

Rate the participant's/subject's average behavior over the past 4 weeks. If the symptom has not been present in the past 4 weeks, or the rating for the past month does not adequately reflect symptom severity in the interval since the participant's/subject's last assessment, rate the worst level of severity during the interval since the participant/subject was last seen. Suggeseted prompts: Can you tell me what day of the week it is today? What time is it (morning / afternoon / evening)? Do you know the date? Where are we now? In the past four weeks, have there been any spells when you were muddled or confused and got these things wrong?

COPYRIGHT University of Ulm, Faculty of Medicine, EHDN Behavioural Phenotype Working Group (Version 1.4, February 2007). For enquiries, contact permissions@euro-hd.net A paper describing the reliability of the PBA-s (in English, Dutch and French) based on data from the TRACK-HD study is in preparation and will be submitted shortly. Craufurd D, Thompson J, Snowden JS. Behavioural changes in Huntington's disease: the Problem Behaviours Assessment. Neuropsychiatry, Neuropsychology and Behavioural Neurology 2001; 14 (4): 219-226. Adult Supplemental 3.00 2013-07-14 19:48:50.273 Problem Behaviours Assessment HD - Short Version (PBA-s) Emotional/Behavioral Outcomes and End Points

Single Pre-Defined Value Selected

C17200 Problem Behaviours Assessment - Short Form (PBA-S) - irritability frequency score PBASIrritFreqScore The frequency score for irritability frequencies as part of the Problem Behaviours Assessment - Short Form (PBA-S). The frequency score for irritability frequencies as part of the Problem Behaviours Assessment - Short Form (PBA-S). Irritability Frequency 0;1;2;3;4;9 Never/almost never;Seldom (less than once/week);Sometimes (up to 4 times a week);Frequently (most days/5,6 or 7 times a week);Daily/almost daily for most (or all) of day;Not known or not applicable Numeric values

Rate the participant's/subject's behavior over the past 4 weeks. If the symptom has NOT been present in the past 4 weeks, or the rating for the past month does not adequately reflect symptom severity in the interval since the patient's last assessment, use the third box to rate the WORST level of severity during the interval since patient was last seen. This item is used to rate the ease with which the subject looses his/her temper, rather than the degree to which self-control is lost once the subject is angry (the latter is rated in the next item). It should also be used to record irritable moods which might have developed into an angry outburst if the carer had not acted with increased tact or discretion. In the past four weeks, have you been irritable, bad-tempered, moody or 'cranky'? Do you think you get cross more easily than you used to? (if yes to above) How does this affect the people around you? Do you think they treat you differently when you are like that?

COPYRIGHT University of Ulm, Faculty of Medicine, EHDN Behavioural Phenotype Working Group (Version 1.4, February 2007). For enquiries, contact permissions@euro-hd.net A paper describing the reliability of the PBA-s (in English, Dutch and French) based on data from the TRACK-HD study is in preparation and will be submitted shortly. Craufurd D, Thompson J, Snowden JS. Behavioural changes in Huntington's disease: the Problem Behaviours Assessment. Neuropsychiatry, Neuropsychology and Behavioural Neurology 2001; 14 (4): 219-226. Adult Supplemental 3.00 2013-07-14 19:48:50.273 Problem Behaviours Assessment HD - Short Version (PBA-s) Emotional/Behavioral Outcomes and End Points

Single Pre-Defined Value Selected

C17211 Problem Behaviours Assessment - Short Form (PBA-S) - obsessive compulsive behavior severity score PBASObsCompBehvSevScore The severity score for Obsessive-Compulsive Behaviours as part of the Problem Behaviours Assessment - Short Form (PBA-S). The severity score for Obsessive-Compulsive Behaviours as part of the Problem Behaviours Assessment - Short Form (PBA-S). Obsessive-Compulsive Behaviours Severity 0;1;2;3;4 Symptom absent;Questionable or trivial;Obsessional thoughts or mild compulsive behaviours which do not interfere with everyday life or cause subject significant distress;Obsessive-compulsive behaviours are present to a degree which interferes with everyday life or causes significant distress for subject;Obsessional phenomena cause serious distress, are time consuming (>1 hour/day) or significantly interfere with the person's normal routine, occupational functioning or usual social activities or relationships Numeric values

Rate the participant's/subject's average behavior over the past 4 weeks. Obsessive-compulsive phenomena diagnosed according to DSM-IV: • recurrent or persistent thoughts, impulses or images that are experienced, at some time during the disturbance, as intrusive and inappropriate, and that cause marked anxiety or distress • The thoughts, impulses or images are not simply excessive worries about real-life problems • The person attempts to ignore or suppress such thoughts, impulses or images, or to neutralise them with some other thought or action • The person recognises that the obsessional thoughts, impulses or images are a product of his or her own mind (not imposed from without) • Repetitive behaviours (e.g. hand-washing, ordering, checking) or mental acts (e.g. praying, counting, repeating words silently) that the person feels driven to perform in response to an obsession, or according to rules that must be applied rigidly • The behaviours or mental acts are aimed at preventing or reducing distress or preventing some dreaded event or situation; however, these behaviours or mental acts are not connected in any realistic way with what they are designed to neutralise, or are clearly excessive Rate 2 if subject has mild obsessive-compulsive traits such as double checking (a small number of times) that doors are locked or ashtrays empty at night. Rate 3 if subject displays mild ritualistic behaviours such as hand-washing, turning lights on and off repetitively or 'evening-up' after touching things by touching with the other hand too

COPYRIGHT University of Ulm, Faculty of Medicine, EHDN Behavioural Phenotype Working Group (Version 1.4, February 2007). For enquiries, contact permissions@euro-hd.net A paper describing the reliability of the PBA-s (in English, Dutch and French) based on data from the TRACK-HD study is in preparation and will be submitted shortly. Craufurd D, Thompson J, Snowden JS. Behavioural changes in Huntington's disease: the Problem Behaviours Assessment. Neuropsychiatry, Neuropsychology and Behavioural Neurology 2001; 14 (4): 219-226. Adult Supplemental 3.00 2013-07-14 19:48:50.273 Problem Behaviours Assessment HD - Short Version (PBA-s) Emotional/Behavioral Outcomes and End Points

Single Pre-Defined Value Selected

C17190 Problem Behaviours Assessment - Short Form (PBA-S) - depressed mood severity score PBASDepMoodSevScore The score for severity of average depressed mood change over past four weeks as part of the Problem Behaviours Assessment - Short Form (PBA-S). The score for severity of average depressed mood change over past four weeks as part of the Problem Behaviours Assessment - Short Form (PBA-S). Depressed Mood Severity 0;1;2;3;4;8 Absent;Questionable;Low mood is present intermittently but does not interfere with everyday function;Subject feels sad much of the time and takes no pleasure from things that he/she usually enjoys, but may still be able to cheer up sometimes with a big effort;Subject feels sad and utterly miserable all day, takes no pleasure from things that he/she usually enjoys, does not cheer up anytime;Unable to assess because condition too advanced (e.g. mute and immobile) Numeric values

Rate the severity of participant's/subject's average behavior over the past 4 weeks. If the symptom has NOT been present in the past 4 weeks, or the rating for the past month does not adequately reflect symptom severity in the interval since the patient's last assessment, use the third box to rate the WORST level of severity during the interval since patient was last seen. Suggested Prompts: Start the interview with an open ended question "Have you noticed any change in your mood since the last visit?" and then continue with more specific questioning as follows: In the past four weeks, have you been feeling sad? (or blue, or low in spirits?) Have you found yourself doing something you would ordinarily enjoy and realized you are not having fun? (Evidence of sad mood from behavioral observation includes sad voice or expression, tearfulness) (if yes to either of the above) Has your mood affected your daily activities? Does the depressed mood come and go or does it seem always to be there? Is there any change throughout the day? Can you snap out of it if someone tries to cheer you up?

COPYRIGHT University of Ulm, Faculty of Medicine, EHDN Behavioural Phenotype Working Group (Version 1.4, February 2007). For enquiries, contact permissions@euro-hd.net A paper describing the reliability of the PBA-s (in English, Dutch and French) based on data from the TRACK-HD study is in preparation and will be submitted shortly. Craufurd D, Thompson J, Snowden JS. Behavioural changes in Huntington's disease: the Problem Behaviours Assessment. Neuropsychiatry, Neuropsychology and Behavioural Neurology 2001; 14 (4): 219-226. Adult Supplemental 3.00 2013-07-14 19:48:50.273 Problem Behaviours Assessment HD - Short Version (PBA-s) Emotional/Behavioral Outcomes and End Points

Single Pre-Defined Value Selected

C17222 Problem Behaviours Assessment - Short Form (PBA-S) - disoriented behaviours frequency score PBASDisorientBehavFreqScore The severity score disoriented behaviour as part of the Problem Behaviours Assessment - Short Form (PBA-S). The severity score disoriented behaviour as part of the Problem Behaviours Assessment - Short Form (PBA-S). Disoriented Behaviour Frequency 0;1;2;3;4;9 Never/almost never;Seldom (less than once/week);Sometimes (up to 4 times a week);Frequently (most days/5,6 or 7 times a week);Daily/almost daily for most (or all) of day;Not known or not applicable Numeric values

Rate the participant's/subject's avearge behavior over the past 4 weeks. If the symptom has not been present in the past 4 weeks, or the rating for the past month does not adequately reflect symptom severity in the interval since the participant's/subject's last assessment, rate the worst level of severity during the interval since the participant/subject was last seen. Suggeseted prompts: Can you tell me what day of the week it is today? What time is it (morning / afternoon / evening)? Do you know the date? Where are we now? In the past four weeks, have there been any spells when you were muddled or confused and got these things wrong?

COPYRIGHT University of Ulm, Faculty of Medicine, EHDN Behavioural Phenotype Working Group (Version 1.4, February 2007). For enquiries, contact permissions@euro-hd.net A paper describing the reliability of the PBA-s (in English, Dutch and French) based on data from the TRACK-HD study is in preparation and will be submitted shortly. Craufurd D, Thompson J, Snowden JS. Behavioural changes in Huntington's disease: the Problem Behaviours Assessment. Neuropsychiatry, Neuropsychology and Behavioural Neurology 2001; 14 (4): 219-226. Adult Supplemental 3.00 2013-07-14 19:48:50.273 Problem Behaviours Assessment HD - Short Version (PBA-s) Emotional/Behavioral Outcomes and End Points

Single Pre-Defined Value Selected

C17201 Problem Behaviours Assessment - Short Form (PBA-S) - irritability worst score PBASIrritWrstScore The worst score for irritability over the last four weeks as part of the Problem Behaviours Assessment - Short Form (PBA-S). The worst score for irritability over the last four weeks as part of the Problem Behaviours Assessment - Short Form (PBA-S). Irritability Worst 0;1;2;3;4 No more irritable than the average person;Questionable or trivial|within normal limits but worse than he/she used to be;Definitely more irritable than is reasonable but not to an extent which causes significant problems or distress for other household members;Subject very irritable and looses temper over trivial matters|household members have to be careful what they say and do to avoid problems;Subject very irritable and looses temper without any obvious reason at all|living with him/her is like walking on eggshells Numeric Values

Rate the participant's/subject's average behavior over the past 4 weeks. If the symptom has NOT been present in the past 4 weeks, or the rating for the past month does not adequately reflect symptom severity in the interval since the patient's last assessment, use the third box to rate the WORST level of severity during the interval since patient was last seen.

COPYRIGHT University of Ulm, Faculty of Medicine, EHDN Behavioural Phenotype Working Group (Version 1.4, February 2007). For enquiries, contact permissions@euro-hd.net A paper describing the reliability of the PBA-s (in English, Dutch and French) based on data from the TRACK-HD study is in preparation and will be submitted shortly. Craufurd D, Thompson J, Snowden JS. Behavioural changes in Huntington's disease: the Problem Behaviours Assessment. Neuropsychiatry, Neuropsychology and Behavioural Neurology 2001; 14 (4): 219-226. Adult Supplemental 3.00 2013-07-14 19:48:50.273 Problem Behaviours Assessment HD - Short Version (PBA-s) Emotional/Behavioral Outcomes and End Points

Single Pre-Defined Value Selected

C17212 Problem Behaviours Assessment - Short Form (PBA-S) - obsessive compulsive behavior frequency score PBASObsCompBehvFrqScore The frequency score for Obsessive-Compulsive Behaviours as part of the Problem Behaviours Assessment - Short Form (PBA-S). The frequency score for Obsessive-Compulsive Behaviours as part of the Problem Behaviours Assessment - Short Form (PBA-S). Obsessive-Compulsive Behaviours Frequency 0;1;2;3;4;9 Never/almost never;Seldom (less than once/week);Sometimes (up to 4 times a week);Frequently (most days/5,6 or 7 times a week);Daily/almost daily for most (or all) of day;Not known or not applicable Numeric values

Rate the participant's/subject's average behavior over the past 4 weeks. Obsessive-compulsive phenomena diagnosed according to DSM-IV.

COPYRIGHT University of Ulm, Faculty of Medicine, EHDN Behavioural Phenotype Working Group (Version 1.4, February 2007). For enquiries, contact permissions@euro-hd.net A paper describing the reliability of the PBA-s (in English, Dutch and French) based on data from the TRACK-HD study is in preparation and will be submitted shortly. Craufurd D, Thompson J, Snowden JS. Behavioural changes in Huntington's disease: the Problem Behaviours Assessment. Neuropsychiatry, Neuropsychology and Behavioural Neurology 2001; 14 (4): 219-226. Adult Supplemental 3.00 2013-07-14 19:48:50.273 Problem Behaviours Assessment HD - Short Version (PBA-s) Emotional/Behavioral Outcomes and End Points

Single Pre-Defined Value Selected

C17191 Problem Behaviours Assessment - Short Form (PBA-S) - depressed mood frequency score PBASDepMoodFrqScore The score for frequency of average depressed mood change over past four weeks as part of the Problem Behaviours Assessment - Short Form (PBA-S). The score for frequency of average depressed mood change over past four weeks as part of the Problem Behaviours Assessment - Short Form (PBA-S). Depressed Mood Frequency 0;1;2;3;4;9 Never/almost never;Seldom (less than once/week);Sometimes (up to 4 times a week);Frequently (most days/5,6 or 7 times a week);Daily/almost daily for most (or all) of day;Not known or not applicable Numeric values

Rate the frequency of participant's/subject's average behavior over the past 4 weeks. If the symptom has NOT been present in the past 4 weeks, or the rating for the past month does not adequately reflect symptom severity in the interval since the patient's last assessment, use the third box to rate the WORST level of severity during the interval since patient was last seen. Suggested Prompts: Start the interview with an open ended question "Have you noticed any change in your mood since the last visit?" and then continue with more specific questioning as follows: In the past four weeks, have you been feeling sad? (or blue, or low in spirits?) Have you found yourself doing something you would ordinarily enjoy and realized you are not having fun? (Evidence of sad mood from behavioral observation includes sad voice or expression, tearfulness) (if yes to either of the above) Has your mood affected your daily activities? Does the depressed mood come and go or does it seem always to be there? Is there any change throughout the day? Can you snap out of it if someone tries to cheer you up?

COPYRIGHT University of Ulm, Faculty of Medicine, EHDN Behavioural Phenotype Working Group (Version 1.4, February 2007). For enquiries, contact permissions@euro-hd.net A paper describing the reliability of the PBA-s (in English, Dutch and French) based on data from the TRACK-HD study is in preparation and will be submitted shortly. Craufurd D, Thompson J, Snowden JS. Behavioural changes in Huntington's disease: the Problem Behaviours Assessment. Neuropsychiatry, Neuropsychology and Behavioural Neurology 2001; 14 (4): 219-226. Adult Supplemental 3.00 2013-07-14 19:48:50.273 Problem Behaviours Assessment HD - Short Version (PBA-s) Emotional/Behavioral Outcomes and End Points

Single Pre-Defined Value Selected

C17223 Problem Behaviours Assessment - Short Form (PBA-S) - disoriented behaviours worst score PBASDisorientBehavWrstScore The worst score disoriented behaviour as part of the Problem Behaviours Assessment - Short Form (PBA-S). The worst score disoriented behaviour as part of the Problem Behaviours Assessment - Short Form (PBA-S). Disoriented Behaviour Worst 0;1;2;3;4;8 Symptom absent;Questionable or trivial (e.g. subject gets day wrong ± one day, or fails to recognize people when meeting them out of their normal context);Subject does not seem to be fully aware of surroundings or the passage of time, but this does not cause significant practical problems;Evidence of confusion at night (subject appears disoriented in time, place or person to an extent that causes practical problems for carers) but normal during daylight hours;Subject is confused and disoriented all the time, unaware of time of day/day of week/date and wrongly identifying surroundings or the people around him (e.g. mistakes nursing home for a prison and nursing staff as prison warders) and consequently resists efforts of carers to look after him/her.;Unable to assess because condition too advanced (e.g. mute and immobile) Numeric values

Score "WORST" box if the rating for the past month does not adequately reflect symptom severity in the interval since the participant's/subject's last assessment, rate the WORST level of severity during the interval since the participant/subject was last seen.

COPYRIGHT University of Ulm, Faculty of Medicine, EHDN Behavioural Phenotype Working Group (Version 1.4, February 2007). For enquiries, contact permissions@euro-hd.net A paper describing the reliability of the PBA-s (in English, Dutch and French) based on data from the TRACK-HD study is in preparation and will be submitted shortly. Craufurd D, Thompson J, Snowden JS. Behavioural changes in Huntington's disease: the Problem Behaviours Assessment. Neuropsychiatry, Neuropsychology and Behavioural Neurology 2001; 14 (4): 219-226. Adult Supplemental 3.00 2013-07-14 19:48:50.273 Problem Behaviours Assessment HD - Short Version (PBA-s) Emotional/Behavioral Outcomes and End Points

Single Pre-Defined Value Selected

C17202 Problem Behaviours Assessment - Short Form (PBA-S) - angry aggressive behaviour severity score PBASAngrAggrBehavSevScore The severity score for angry or aggressive behaviour as part of the Problem Behaviours Assessment - Short Form (PBA-S). The severity score for angry or aggressive behaviour as part of the Problem Behaviours Assessment - Short Form (PBA-S). Angry or Aggressive Behaviour Severity 0;1;2;3;4 Normal;Questionable;Verbal outbursts which are outside socially acceptable limits but do not cause significant problems or distress for other household members. for example, rate 2 if subject becomes angry with self or inanimate objects when confronted with frustrating situations due to disability, such as failure when attempting to rewire a plug;Temper tantrums are severe enough to cause significant distress for other household members and/or practical difficulties caring for the subject. rate 3 when verbal hostility or anger is directed towards another person (e.g. shouting, sarcastic name-calling, use of foul or abusive language). also rate 3 if there are explicit verbal threats of violence to another person, or behaviour causing a justifiable fear of personal violence (e.g. subject approaches too close, raises fist, mild pushing). also rate 3 for violence towards property;Subject has temper tantrums so severe that relationship with carers is compromised, creating risk that subject will be rejected. rate 4 if there has been any kind of actual physical assault (includes pushing, shoving, hitting, biting, scratching, kicking) or threatening behaviour involving weapons Numeric values

Rate the participant's/subject's behavior over the past 4 weeks. If the symptom has NOT been present in the past 4 weeks, or the rating for the past month does not adequately reflect symptom severity in the interval since the patient's last assessment, use the third box to rate the WORST level of severity during the interval since patient was last seen. Suggested prompts: In the past four weeks, have you had any emotional or angry outbursts? Have you had times when you lost control of your temper? Have you hit, shoved or thrown things or expressed your temper in a physical way? Have you used threats or hostile words?

COPYRIGHT University of Ulm, Faculty of Medicine, EHDN Behavioural Phenotype Working Group (Version 1.4, February 2007). For enquiries, contact permissions@euro-hd.net A paper describing the reliability of the PBA-s (in English, Dutch and French) based on data from the TRACK-HD study is in preparation and will be submitted shortly. Craufurd D, Thompson J, Snowden JS. Behavioural changes in Huntington's disease: the Problem Behaviours Assessment. Neuropsychiatry, Neuropsychology and Behavioural Neurology 2001; 14 (4): 219-226. Adult Supplemental 3.00 2013-07-14 19:48:50.273 Problem Behaviours Assessment HD - Short Version (PBA-s) Emotional/Behavioral Outcomes and End Points

Single Pre-Defined Value Selected

C17213 Problem Behaviours Assessment - Short Form (PBA-S) - obsessive compulsive behavior worst score PBASObsesCompBehvWrstScore The worst score for Obsessive-Compulsive Behaviours as part of the Problem Behaviours Assessment - Short Form (PBA-S). The worst score for Obsessive-Compulsive Behaviours as part of the Problem Behaviours Assessment - Short Form (PBA-S). Obsessive-Compulsive Behaviours Worst 0;1;2;3;4 Symptom absent;Questionable or trivial;Obsessional thoughts or mild compulsive behaviours which do not interfere with everyday life or cause subject significant distress;Obsessive-compulsive behaviours are present to a degree which interferes with everyday life or causes significant distress for subject;Obsessional phenomena cause serious distress, are time consuming (>1 hour/day) or significantly interfere with the person's normal routine, occupational functioning or usual social activities or relationships Numeric Values

Score "WORST" box if the rating for the past month does not adequately reflect symptom severity in the interval since the participant's/subject's last assessment, rate the WORST level of severity during the interval since the participant/subject was last seen. Rate 2 if subject has mild obsessive-compulsive traits such as double checking (a small number of times) that doors are locked or ashtrays empty at night. Rate 3 if subject displays mild ritualistic behaviors such as hand-washing, turning lights on and off repetitively or 'evening-up' after touching things by touching with the other hand too.;

© COPYRIGHT University of Ulm, Faculty of Medicine, EHDN Behavioural Phenotype Working Group (Version 1.4, February 2007). For enquiries, contact permissions@euro-hd.net A paper describing the reliability of the PBA-s (in English, Dutch and French) based on data from the TRACK-HD study is in preparation and will be submitted shortly. Craufurd D, Thompson J, Snowden JS. Behavioural changes in Huntington's disease: the Problem Behaviours Assessment. Neuropsychiatry, Neuropsychology and Behavioural Neurology 2001; 14 (4): 219-226. Adult Supplemental 3.00 2013-07-14 19:48:50.273 Problem Behaviours Assessment HD - Short Version (PBA-s) Emotional/Behavioral Outcomes and End Points

Single Pre-Defined Value Selected

C17192 Problem Behaviours Assessment - Short Form (PBA-S) - depressed mood worst rating score PBASDepMoodWrstScore The worst score for severity of Depressive Mood as part of the Problem Behaviours Assessment - Short Form (PBA-S). The worst score for severity of Depressive Mood as part of the Problem Behaviours Assessment - Short Form (PBA-S). Depressed Mood Worst 0;1;2;3;4;8 Absent;Questionable;Low mood is present intermittently but does not interfere with everyday function;Subject feels sad much of the time and takes no pleasure from things that he/she usually enjoys, but may still be able to cheer up sometimes with a big effort;Subject feels sad and utterly miserable all day, takes no pleasure from things that he/she usually enjoys, does not cheer up anytime;Unable to assess because condition too advanced (e.g. mute and immobile) Numeric Values

Score "WORST" box if the rating for the past month does not adequately reflect symptom severity in the interval since the participant's/subject's last assessment, rate the worst level of severity during the interval since the participant/subject was last seen. Rate 2 if subject can easily enjoy amusing activities or visits from friends. Rate 3 if low mood has definite effect on subject's lifestyle, e.g. unable to enjoy company of friends or amusing diversions. See instrument for additional instructions and scoring guide.

COPYRIGHT University of Ulm, Faculty of Medicine, EHDN Behavioural Phenotype Working Group (Version 1.4, February 2007). For enquiries, contact permissions@euro-hd.net A paper describing the reliability of the PBA-s (in English, Dutch and French) based on data from the TRACK-HD study is in preparation and will be submitted shortly. Craufurd D, Thompson J, Snowden JS. Behavioural changes in Huntington's disease: the Problem Behaviours Assessment. Neuropsychiatry, Neuropsychology and Behavioural Neurology 2001; 14 (4): 219-226. Adult Supplemental 3.00 2013-07-14 19:48:50.273 Problem Behaviours Assessment HD - Short Version (PBA-s) Emotional/Behavioral Outcomes and End Points

Single Pre-Defined Value Selected

C17224 Problem Behaviours Assessment - Short Form (PBA-S) - informant relationship code PBASInfoRelatCode The relationship of the informant to the participant/subject as part of the Problem Behaviours Assessment - Short Form (PBA-S). The relationship of the informant to the participant/subject as part of the Problem Behaviours Assessment - Short Form (PBA-S). Is informant a relative? 1;2;3;4;5;6;7;8;9 Spouse or partner;Parent;Sibling;Child;Other relative;Friend or neighbor;Professional care worker;Other;No informant-subject came alone Numeric values

Codes for informant details.

COPYRIGHT University of Ulm, Faculty of Medicine, EHDN Behavioural Phenotype Working Group (Version 1.4, February 2007). For enquiries, contact permissions@euro-hd.net A paper describing the reliability of the PBA-s (in English, Dutch and French) based on data from the TRACK-HD study is in preparation and will be submitted shortly. Craufurd D, Thompson J, Snowden JS. Behavioural changes in Huntington's disease: the Problem Behaviours Assessment. Neuropsychiatry, Neuropsychology and Behavioural Neurology 2001; 14 (4): 219-226. Adult Supplemental 3.00 2013-07-14 19:48:50.273 Problem Behaviours Assessment HD - Short Version (PBA-s) Emotional/Behavioral Outcomes and End Points

Single Pre-Defined Value Selected

C17203 Problem Behaviours Assessment - Short Form (PBA-S) - angry aggressive behaviour frequency score PBASAngrAggrBehavFrqScore The frequency score for angry or aggressive behaviour as part of the Problem Behaviours Assessment - Short Form (PBA-S). The frequency score for angry or aggressive behaviour as part of the Problem Behaviours Assessment - Short Form (PBA-S). Angry or Aggressive Behaviour Frequency 0;1;2;3;4;9 Never/almost never;Seldom (less than once/week);Sometimes (up to 4 times a week);Frequently (most days/5,6 or 7 times a week);Daily/almost daily for most (or all) of day;Not known or not applicable Numeric values

Rate the participant's/subject's average behavior over the past 4 weeks. If the symptom has NOT been present in the past 4 weeks, or the rating for the past month does not adequately reflect symptom severity in the interval since the patient's last assessment, use the third box to rate the WORST level of severity during the interval since patient was last seen. Suggested prompts: In the past four weeks, have you had any emotional or angry outbursts? Have you had times when you lost control of your temper? Have you hit, shoved or thrown things or expressed your temper in a physical way? Have you used threats or hostile words?

COPYRIGHT University of Ulm, Faculty of Medicine, EHDN Behavioural Phenotype Working Group (Version 1.4, February 2007). For enquiries, contact permissions@euro-hd.net A paper describing the reliability of the PBA-s (in English, Dutch and French) based on data from the TRACK-HD study is in preparation and will be submitted shortly. Craufurd D, Thompson J, Snowden JS. Behavioural changes in Huntington's disea+V1091se: the Problem Behaviours Assessment. Neuropsychiatry, Neuropsychology and Behavioural Neurology 2001; 14 (4): 219-226. Adult Supplemental 3.00 2013-07-14 19:48:50.273 Problem Behaviours Assessment HD - Short Version (PBA-s) Emotional/Behavioral Outcomes and End Points

Single Pre-Defined Value Selected

CSV