Objective: The present study was done to assess cognitive impairment in HD patients using a neuropsychological battery and to correlate with clinical severity and CAG trinucleotide repeat length.

Background: Cognitive impairment is observed in patients of Huntington’s disease (HD) at various stages and may precede the onset of chorea by many years. These abnormalities impose a great burden on care givers.

Methods: The study was conducted in the department of neurology, at the National institute of mental health and neurosciences (NIMHANS), Bangalore, India. Thirty HD patients with age ≥18 years and 30 age and gender matched controls were included in the study. Clinical severity was assessed using the unified Huntington disease rating scale (UHDRS – motor). We employed a battery of neuropsychological tests such as mini mental status examination (MMSE), frontal assessment battery (FAB), Montreal cognitive assessment, digit span forwards and backwards, verbal fluency, Corsi block tap test, response inhibition (stroop test), Rey’s auditory verbal and learning test (AVLT) and story recall in both groups.

Results: The mean age of the patients was 42.1±14.1 years and controls was 39.4±12.4 years (p=0.61). The mean age at disease onset was 38.5±10.9 years with mean UHDRS score of 36.4±13.3. Neuropsychological assessment revealed severe cognitive impairment that was observed in all the tests (p<0.001). The cognitive impairment did not correlate with CAG repeat length. However a significant negative correlation was seen between the clinical severity (UHDRS) and cognition.

Conclusions: HD patients have profound impairment of cognition, predominantly involving attention, memory and executive functions.

« Back to 2016 International Congress

MDS Abstracts - https://www.mdsabstracts.org/abstract/cognitive-impairment-in-huntingtons-disease/