Onset of Huntington's disease: can it be purely cognitive?

Jane S Paulsen; Jeffrey D Long

doi:10.1002/mds.25997

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Onset of Huntington's disease: can it be purely cognitive?

Journal article

Peer reviewed

Onset of Huntington's disease: can it be purely cognitive?

Jane S Paulsen and Jeffrey D Long

Movement disorders, Vol.29(11), pp.1342-1350

09/15/2014

DOI: 10.1002/mds.25997

PMCID: PMC4193504

PMID: 25142616

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Abstract

Knowledge of the cognitive manifestation of Huntington's disease has burgeoned over the past two decades. Many studies from independent datasets have shown that cognitive impairment is evident before motor diagnosis, and annual cognitive decline is a robust marker of disease progression. Additionally, cognition is a critical concern to patients and families and is associated with meaningful outcomes, including functional capacity, driving, loss of accustomed work, and quality of life. In the past few years, Huntington's disease animal models of cognition have increased, preparing for preclinical experimental therapeutics with cognitive endpoints. A longitudinal analysis of cognitive variables was conducted with 559 gene-positive cases and 233 controls showing no signs of motor abnormalities over approximately a 3-year period. Results show statistically significant differences in rate of annual change for some cognitive variables, such that the cases group had worsening performance over time. These findings show that cognitive deterioration can be seen in persons with the Huntington's disease gene expansion with no overt motor signs or symptoms, suggesting that cognitive onset of Huntington's disease may precede motor.

Severity of Illness Index

Humans

Middle Aged

Male

Nerve Tissue Proteins - genetics

Huntington Disease - complications

Neuropsychological Tests

Cognition Disorders - diagnosis

Huntingtin Protein

Cognition Disorders - etiology

Huntington Disease - genetics

Trinucleotide Repeat Expansion - genetics

Adult

Female

Longitudinal Studies

Details

Title: Subtitle: Onset of Huntington's disease: can it be purely cognitive?
Creators: Jane S Paulsen - Department of Psychiatry, Carver College of Medicine, The University of Iowa, Iowa City, IA, USA; Department of Neurology, Carver College of Medicine, The University of Iowa, Iowa City, IA, USA; Department of Psychology, The University of Iowa, Iowa City, IA, USA
Jeffrey D Long
Resource Type: Journal article
Publication Details: Movement disorders, Vol.29(11), pp.1342-1350
DOI: 10.1002/mds.25997
PMID: 25142616
PMCID: PMC4193504
NLM abbreviation: Mov Disord
ISSN: 0885-3185
eISSN: 1531-8257
Publisher: United States
Grant note: R01 NS040068 / NINDS NIH HHS 5R01NS040068 / NINDS NIH HHS M01 RR000059 / NCRR NIH HHS R01 NS054893 / NINDS NIH HHS 5R01NS054893 / NINDS NIH HHS U01 NS082089 / NINDS NIH HHS UL1 TR000442 / NCATS NIH HHS
Language: English
Date published: 09/15/2014
Academic Unit: Psychiatry; Psychological and Brain Sciences; Biostatistics
Record Identifier: 9984003445502771

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