Juvenile Huntington’s disease: neuropathology

Jean Paul G. Vonsattel, Etty P. Cortes, and Christian E. Keller

in Juvenile Huntington's Disease

Published on behalf of Oxford University Press

Published in print January 2009 | ISBN: 9780199236121
Published online November 2012 | e-ISBN: 9780191753213 | DOI:
Juvenile Huntington’s disease: neuropathology

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Brains from individuals with AOHD or JHD share many pathological features. However, the following features are more likely to occur in brains from individuals with JHD than in brains from individuals with AOHD: ♦ The brain is diffusely smaller than normal, with predominant frontal and parietal atrophy. ♦ The striatal gradient of the severity of neuronal loss and reactive gliosis is blurred; the relative preservation of the nucleus accumbens is no longer visible or barely identifiable. ♦The internal segment of the globus pallidus is often severely atrophic. ♦The presence of relatively preserved parenchymal islets is frequently found within the rostral part of the neostriatum. ♦Atrophy of the thalamus, notably of the centrum medianum, is discrete. ♦There is little or no discrepancy between the severe atrophy of striatal and non-striatal regions. ♦Associated metabolic encephalopathy is either focal or diffuse. ♦There is an absence of changes associated with normal ageing or with neurodegenerative diseases of the elderly. ♦ The neuropathological grade of severity assigned is grade 4 or, less likely, grade 3, unless premature death was due to factors other than HD (e.g. suicide).

Chapter.  7314 words. 

Subjects: Neurology ; Pathology

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