When does Huntington's disease begin?

Recent studies have detected basal ganglia atrophy in clinically asymptomatic persons with the genetic mutation that causes Huntington's disease (HD). Whether reductions in caudate and putamen volume on MRI scans are associated with changes in cognitive and neurologic functioning was examined i...

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Bibliographic Details
Published in:Journal of the International Neuropsychological Society 1998-09, Vol.4 (5), p.467-473
Main Authors: CAMPODONICO, JEFFREY R., AYLWARD, ELIZABETH, CODORI, ANN-MARIE, YOUNG, CANDACE, KRAFFT, LAURA, MAGDALINSKI, MICHAEL, RANEN, NEAL, SLAVNEY, PHILLIP R., BRANDT, JASON
Format: Article
Language:English
Subjects:
Adult
Atrophy - pathology
Basal Ganglia - pathology
Cognitive tests
Female
Genetics
Humans
Huntington Disease - genetics
Huntington Disease - pathology
Huntington Disease - psychology
Huntington's disease
Learning - physiology
Magnetic Resonance Imaging
Male
Memory - physiology
MRI scans
Neostriatum - pathology
Neuropsychological Tests
Presymptomatic testing
Psychomotor Performance - physiology
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Summary:Recent studies have detected basal ganglia atrophy in clinically asymptomatic persons with the genetic mutation that causes Huntington's disease (HD). Whether reductions in caudate and putamen volume on MRI scans are associated with changes in cognitive and neurologic functioning was examined in 13 healthy adults with the IT-15 mutation. Reduced striatal volume was found to correlate with greater neurologic (largely motor) impairment, slower mental processing speed, and poorer verbal learning, although none of the participants met even liberal criteria for clinical diagnosis of HD. These correlations are strikingly similar to those observed in symptomatic HD patients, possibly reflecting the earliest manifestations of disease. (JINS, 1998, 4, 467–473.)
ISSN:1355-6177
1469-7661
DOI:10.1017/S1355617798455061