## Abstract Benign hereditary chorea is an autosomal dominant disorder presenting with childhood‐onset chorea, no dementia, and little or no progression. We present a family with typical clinical features of benign hereditary chorea. Pathological investigation of the brain of an affected family mem
Benign hereditary chorea Clinical and genetic aspects
✍ Scribed by Peter S. Harper
- Book ID
- 119838774
- Publisher
- John Wiley and Sons
- Year
- 2008
- Tongue
- English
- Weight
- 832 KB
- Volume
- 13
- Category
- Article
- ISSN
- 0009-9163
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## Abstract Because of clinical similarities, benign hereditary chorea and myoclonus‐dystonia (DYT11) might be confused. No systematic comparisons of genetically proven cases with thyroid transcription factor‐1 (__TITF‐1__) and ε‐sarcoglycan (__SGCE__) mutations have been performed to date. Three i
## Abstract Benign hereditary chorea is an autosomal dominant disorder characterized by early onset nonprogressive chorea, caused by mutations of the __thyroid transcription factor‐1 (TITF‐1)__ gene. Clinical heterogeneity has been reported and thyroid and respiratory abnormalities may be present.