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Efforts to understand Huntington disease (HD) have advanced neuroscience and clinical neurology as much as any human disorder and have led to improved research methods at every level of investigation, from molecular modeling to neuroimaging to population genetics. Among the vexing questions remaining in HD is why motor manifestations differ so starkly between childhood and adult HD. When HD manifests before the age of 20 (juvenile-onset, JOHD), symptoms commonly present with a hypokinetic-rigid syndrome that differs markedly from the hyperkinetic phenotype seen in adult-onset HD.1 Neuroanatomical investigations suggest more widespread deterioration of striatal projections in hypokinetic-rigid HD,2 although the pathophysiologic underpinnings of this difference in motor manifestations are unknown. Do higher CAG repeats lead to distinct molecular pathologies? Does neurodegeneration affect different regions in JOHD? Is HD and JOHD pathology identical, but manifests differently because of the shifts in receptor profile, neurotransmitter synthesis, and regional connectivity that are the hallmarks of development?
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- © 2019 American Academy of Neurology
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