Table 1. Studies of Unawareness in HD.

Author	HD Stage/Participants	Domain of Impairment	Tests	Outcome	Specific Tests
Deckel et al 19934 Prospective	19 affected HD pts Several years to>10 years 14 controls non-HD	Cog, motor, and emotional	8 item self-report rating cog and motor abilities cf staff ratings	One-third of HD patients had anosognosia cf controls and cf raters	WCST WAIS-R Verbal/performance split and picture completion
Snowden et al 1998 5 Prospective	40 affected HD pts duration 1–20 years	Motor	Subjective report questionnaire of direct experience/consequences and QNE	Unawareness of choreiform movement but aware of nonchoreic motor consequences	Cog test correlates expected for HD stage
Vitale et al 200119 Prospective	9 affected HD pts and 13 PD	Motor	Pts asked to rate dyskinesias	Unawareness in HD related to disease duration and severity	Score of unawareness of dyskinesias when performing four motor tasks
Chatterjee et al 20059 Prospective	53 affected HD pts 53 caregivers	Behav	Self-report cf Caregiver rating on BDI, apathy, and irritability scales	Moderate – good for depression, low for apathy and fair at best for irritability	Caregiver assessment for moods and apathy as cognition worsens
Ho et al 200611 Prospective	75 affected HD pts All stages 67 caregivers	Motor Functional Cog	DEX, BDI and Telephone interview of cog status. Pts and caregivers rated themselves and each other	Pts underestimate cf caregiver ratings, which correlated with disease severity on UHDRS	DEX
Hoth et al 200712 Prospective	66 patient and collateral pairs	Cog/emotional and functional	Patient Competency Rating Scale UHDRS, BDI, and Cog measures including WCST	Pts rated themselves better and did not correlate with findings on any subscales; greatest variance was for emotional	WCST greatest correlation with patient/collateral disagreement
Kaptein et al 200722 Prospective	51 couples (HD pts early to mod and partner)	QoL	UHDRS MMSE IPQ Medical Outcome Study of SF-36	Spouses report of more symptoms than the HD pts correlated with QoL	IPQ and MOS SF-36 to HD pts and spouses/partners
Duff et al 201010 Retrospective Data set enquiry	Premanifest 745 HD mutation and 163 without	Cog/behav	FrSBe difference participant and companion	Participants rating of frontal behaviors were less at low and high probability of diagnosis than mid. In contrast, companion rating of frontal behaviors was proportionate to probability of diagnosis	Total FrSBe
Sitek et al 201118 Prospective Specific	HD (23) cf PD with dyskinesia (25), PD without dyskinesia (21) and cervical dystonia 20	Motor and ADL	MIS Self-Assessment Disability Scale Patient and proxy and cog scores	Disparity in motor reports but not IADL	MIS AVLT
Hocaoglu et al 201221	105 HD pts (36 early/18 mod/50 adv) with proxies	QoL	Self and proxy report of HrQoL Proxy perception of QoL	Good correlation between raters. Proxys perception of pt QoLbetter than pts'	HrQoL
Justo et al 201316 Prospective	28 Early HD 28 premanifest and 12 controls	Motor	Self attribution of motor scores vs observer when looking at video of themselves and assessing movements including non pathological involuntary movements	Underestimated movments cf with observer but controls underestimate their non pathological movements	Self attribution of motor scores vs observer.
McCusker et al 201317 Retrospective Data set enquiry	Premanifest HD 550 cf 163 nonmutation	Motor	UHDRS motor Participant HD History of symptoms, self-report of progression, cog, behav, and imaging measures	∼50% with new motor diagnosis had no symptoms or report of progression	UHDRS motor and symptom history, self-report of progression
Cleret de Langavant et al 201320 Prospective	46 Early to mild HD 33 proxies	Cog memory	Subjective memory questionnaires given independently and before cog testing	Early stage HD 1 aware of memory impairment, HD 2 less aware but more aware than proxies	FCSRT Subjective memory score

Abbreviations: ADL, Activities of Daily Living; AVLT, Auditory Verbal Learning Test; BDI, Beck Depression Inventory; Behav, Behavioral; cf, Compared to; Cog, Cognitive; DEX, Dysexecutive Questionnaire; FCSRT, Free and Cued Selective Reminding Test; FrSBe, Frontal Systems Behavior Scale; HD, Huntington Disease; HrQoL, Health-related Quality of Life; IADL, Instrumental Activities of Daily Living Scale; IPQ, Illness Perception Questionnaire; MIS, Motor impairment scale; MMSE, Mini-Mental State Examination; MOS, Medical Outcome Study of the 36-Item Short Form Health Survey; PD, Parkinson Disease; Pts, Patients; QNE, Quantified Neurologic Examination; QoL, Quality of Life; SF-36, Short Form (36) Health Survey; UHDRS, Unified Huntington Disease Rating Scale; WAIS, Wechsler Adult Intelligence Scale; WCST, Wisconsin Card Sort Test.