An Exploratory Pilot Study of Neuropsychological Performance in Two Huntington Disease Centers of Excellence Clinics

• Published in: Archives of clinical neuropsychology Vol. 39; no. 1; pp. 24 - 34
• Main Authors: Rossetti, M Agustina, Anderson, Kendra M, Hay, Kaitlyn R, Del Bene, Victor A, Celka, Andrea S, Piccolino, Adam, Nelson Sheese, Amelia L, Huynh, Melissa, Zhu, Liang, Claassen, Daniel O, Furr Stimming, Erin, Considine, Ciaran M
• Format: Journal Article
• Language: English
• Published: United States Oxford University Press 19-01-2024
• Subjects: Adult; Humans; Huntington Disease - complications; Huntington Disease - psychology; Neuropsychological Tests; Pilot Projects; Retrospective Studies; Movement disorder; Huntington disease; Assessment
• ISSN: 1873-5843; 1873-5843
• DOI: 10.1093/arclin/acad054

Abstract
Objectives
To describe the characteristics of patients receiving a clinical referral for neuropsychological evaluation in two Huntington’s Disease Society of America Centers of Excellence (HDSA COE). In this exploratory pilot study, we used an empirically supported clinical neuropsychological battery to assess differences in cognitive performance between premanifest and manifest HD patient groups (compared with each other and normative expectations).
Method
Clinical data from 76 adult genetically confirmed patients referred for neuropsychological evaluations was retrospectively collected from two HDSA COEs. ANOVA and Chi-square tests were used to compare variables between pre-manifest (n = 14) and manifest (n = 62) groups for demographic, cognitive, neuropsychiatric, and disease severity variables.
Results
Our clinics serviced a disproportionate number of motor manifest patients. Six measures were excluded from analyses due to infrequent administration. The full WAIS-IV Digit Span was disproportionately administered to the manifest group. The premanifest group showed stronger cognitive performance with effect sizes in the large range on subtests of the WAIS-IV Digit Span, HVLT-R, SDMT, and verbal fluency.
Conclusions
This is the first study to assess an empirically supported neuropsychological research battery in a clinical setting with a relatively large sample size given the rarity of HD. The battery adequately captured areas of impairment across the disease spectrum. Application of the current battery with larger premanifest samples is warranted.