Longitudinal Evaluation of "Presymptomatic" Carriers of Huntington's Disease

The authors evaluated motor, behavioral, and cognitive functioning over a 3-year period in 33 presymptomatic carriers for Huntington's disease and compared them with 73 noncarriers. Investigators blind to the participant's gene status utilized the Unified Huntington's Disease Rating S...

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Bibliographic Details
Published in:The journal of neuropsychiatry and clinical neurosciences 2007-07, Vol.19 (3), p.310-317
Main Authors: Witjes-Ané, Marie-Noëlle W, Mertens, Bart, van Vugt, Jeroen P.P, Bachoud-Lévi, Anne-Catherine, van Ommen, Gert-Jan B, Roos, Raymund A.C
Format: Article
Language:English
Subjects:
Adult
Adult and adolescent clinical studies
Behavior - physiology
Biological and medical sciences
Cross-Sectional Studies
Degenerative and inherited degenerative diseases of the nervous system. Leukodystrophies. Prion diseases
Female
Humans
Huntington Disease - genetics
Huntington Disease - physiopathology
Longitudinal Studies
Male
Medical sciences
Mental Status Schedule
Middle Aged
Motor Activity - genetics
Nervous system (semeiology, syndromes)
Nervous system as a whole
Neurology
Neuropsychological Tests - statistics & numerical data
Organic mental disorders. Neuropsychology
Psychology. Psychoanalysis. Psychiatry
Psychopathology. Psychiatry
Reproducibility of Results
Statistics, Nonparametric
Trinucleotide Repeats - genetics
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Summary:The authors evaluated motor, behavioral, and cognitive functioning over a 3-year period in 33 presymptomatic carriers for Huntington's disease and compared them with 73 noncarriers. Investigators blind to the participant's gene status utilized the Unified Huntington's Disease Rating Scale (UHDRS) and performed an extensive neuropsychological assessment (global cognitive, memory, language, psychomotor). Successive evaluations of motor and behavioral patterns showed inconsistencies. The rate of cognitive changes in carriers was similar to that in noncarriers. Commonly used tools are inadequate for detecting markers in preclinical Huntington's disease, limiting the design of therapeutic trials. Research should focus on tracking suitable endpoints combining clinical markers and biomarkers that change linearly with disease progression.
ISSN:0895-0172
1545-7222
DOI:10.1176/jnp.2007.19.3.310