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J Neurol Sci. 2020 Jan 15;408:116523. doi: 10.1016/j.jns.2019.116523. Epub 2019 Oct 15.

Mild cognitive impairment and dementia in motor manifest Huntington's disease: Classification and prevalence.

Author information

1
Division of Behavioral and Cognitive Neurology, Department of Neurology, University of Florida College of Medicine, Gainesville, FL, USA. Electronic address: p.julayanont@ttuhsc.edu.
2
Center for Movement Disorders and Neurorestoration, Department of Neurology, University of Florida College of Medicine, Gainesville, FL, USA. Electronic address: mcfarland@neurology.ufl.edu.
3
Division of Behavioral and Cognitive Neurology, Department of Neurology, University of Florida College of Medicine, Gainesville, FL, USA; Malcom Randall Veterans Affairs Medical Center, Gainesville, FL, USA. Electronic address: heilman@neurology.ufl.edu.

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.

KEYWORDS:

Chorea; Cognitive disorders/dementia; Huntington's disease; Mild cognitive impairment

PMID:
31678902
DOI:
10.1016/j.jns.2019.116523

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