HuntingtonsDiseasechorea - Learning Objective: Huntingtons...

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Learning Objective: Huntington’s Disease, including info about Huntington’s chorea nm Definition - an incurable, adult-onset, autosomal dominant inherited disorder associated with cell loss within a specific subset of neurons int eh basal ganglia and cortex - was first called “hereditary chorea” Causes - selective neuronal dysfunction and subsequent loss of neurons in the striatum, cerebral cortex, and other parts of the brain Risk Factors/ Predisposing Factors - family history of HD Epidemiology - occurrence: 4.1-8.4 per 100,000 people - mean age of onset ranges from 35-44 years old - juvenile HD (onset of HD in pts <20y/o) accounts for about 5-10% of all affected pts - no gender predilection Pathophysiology - gross atrophy of the caudate nucleus and putamen is accompanied by selective neuronal loss and astrogliosis - marked neuronal loss is also seen in deep layers of the cerebral cortex - the extent of gross striatal pathology, neuronal loss, and gliosis provides a basis for grading the severity of HD pathology (grade 0-4): Grade 0 - no detectable histologic neuropathology in the presence
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HuntingtonsDiseasechorea - Learning Objective: Huntingtons...

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