Abstract
The “genetic choreas” consist of a heterogeneous group of disorders, similar in that chorea is typically the most prominent or presenting clinical feature and that degeneration of the striatum is the most characteristic neuropathological feature. Here we discuss the prototypical hereditary choreiform disorder, Huntington’s disease (HD), and a series of other familial disorders in which chorea is more or less part of the clinical syndrome and that may resemble HD, including Huntington’s disease-like 2 (HDL2), the neuroacanthocytoses, benign hereditary chorea, the iron accumulation diseases, and paroxysmal movement disorders. We conclude by providing comprehensive tables of the dominant and nondominant genetic choreas.
| Original language | English (US) |
|---|---|
| Title of host publication | Movement Disorder Genetics |
| Publisher | Springer International Publishing |
| Pages | 147-167 |
| Number of pages | 21 |
| ISBN (Electronic) | 9783319172231 |
| ISBN (Print) | 9783319172224 |
| DOIs | |
| State | Published - Jan 1 2015 |
Keywords
- Basal ganglia
- Chorea
- Dyskinesia
- Hereditary
- Huntington’s disease
- Neuroacanthocytosis
ASJC Scopus subject areas
- General Medicine
- General Biochemistry, Genetics and Molecular Biology
- General Neuroscience
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