TY - JOUR
T1 - Neuropsychological deficits in huntington's disease gene carriers and correlates of early "conversion"
AU - Brandt, Jason
AU - Inscore, Anjeli B.
AU - Ward, Julianna
AU - Shpritz, Barnett
AU - Rosenblatt, Adam
AU - Margolis, Russell L.
AU - Ross, Christopher A.
PY - 2008/11
Y1 - 2008/11
N2 - The authors examined whether the baseline cognitive functioning of 21 clinically normal huntingtin mutation carriers who developed manifest Huntington's disease on follow-up differed from hat of 49 mutation carriers who remain asymptomatic over the same period in a longitudinal study. One hundred thirty-four gene-negative off spring of Huntington s disease patients were studied as well. Overall, there were no differences in cognitive test performance among the three groups. However, "converters" who developed signs of Huntington's disease within 8.6 years demonstrated poorer performance on the Wisconsin Card Sorting Test at baseline. People with the Huntington s disease mutation who are carefully examined neurologically and found to be asymptomatic have, at most, very minimal problem-solving impairment, and only if they are within a few years ofclinical onset.
AB - The authors examined whether the baseline cognitive functioning of 21 clinically normal huntingtin mutation carriers who developed manifest Huntington's disease on follow-up differed from hat of 49 mutation carriers who remain asymptomatic over the same period in a longitudinal study. One hundred thirty-four gene-negative off spring of Huntington s disease patients were studied as well. Overall, there were no differences in cognitive test performance among the three groups. However, "converters" who developed signs of Huntington's disease within 8.6 years demonstrated poorer performance on the Wisconsin Card Sorting Test at baseline. People with the Huntington s disease mutation who are carefully examined neurologically and found to be asymptomatic have, at most, very minimal problem-solving impairment, and only if they are within a few years ofclinical onset.
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U2 - 10.1176/jnp.2008.20.4.466
DO - 10.1176/jnp.2008.20.4.466
M3 - Article
C2 - 19196932
AN - SCOPUS:61849142263
VL - 20
SP - 466
EP - 472
JO - Journal of Neuropsychiatry and Clinical Neurosciences
JF - Journal of Neuropsychiatry and Clinical Neurosciences
SN - 0895-0172
IS - 4
ER -