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 |
[i] 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.