Congratulations Drs. ParunyouJulayanont, Nikolaus R.McFarland, and Kenneth M.Heilman on the publication of “Mild cognitive impairment and dementia in motor manifest Huntington’s disease: Classification and prevalence,” which was published in the October issue of the Journal of the Neurological Sciences.
Abstract
Objectives
To identify the characteristics and prevalence of mild cognitive impairment in patients with motor-manifest Huntington’s disease (HD) and to propose a new mild cognitive impairment (HD-MCI) classification for HD.
Methods
We included 307 motor-manifest HD participants from the ENROLL-HD study who completed the evaluation in four neurocognitive domains including executive functions, processing speed, language, and memory. Cognitive impairment in each domain was determined by age- and education-adjusted cutoffs (> 1.5 standard deviations below the mean). HD-MCI was defined as an impairment in at least one cognitive domain without a loss of functional independence (Function Independence Scale, FIS ≥85). Dementia (HD-Dem) was defined as at least two domains of cognitive impairment with functional impairment (FIS ≤80).
Results
At the onset of motor symptoms, MCI was present in 84% and dementia in 5% of patients. After 5 years of motor symptoms, 24% of participants met the criteria for MCI and 69% for dementia. Executive dysfunction was the most common impairment, being present in 70% of participants, followed by slowed processing speed in 67%. Language impairment was reported in 55% and memory deficits in 53%. MCI subtypes were classified as “Executive-predominant” (executive impairment and slowed processing speed), “Representational-predominant” (impaired language and memory) and “Mixed Executive-Representational”. Executive-predominant MCI comprised 30%, Representational-predominant 15% and Mixed 55% of this cohort.
Conclusion
MCI is highly prevalent in the early stage of motor-manifest HD. Three MCI subgroups are defined suggesting at the earlier stage of this disease the frontal-striatal-executive and/or the temporoparietal-representational functional network can be impaired.