Hereditary benign chorea
β Scribed by Thomas D. Bird
- Publisher
- John Wiley and Sons
- Year
- 2002
- Tongue
- English
- Weight
- 78 KB
- Volume
- 17
- Category
- Article
- ISSN
- 0885-3185
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## Abstract Benign hereditary chorea is an autosomal dominant disorder presenting with childhoodβonset 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
## Abstract We report a nonconsanguineous family in whom two (of three) sons developed isolated chorea in early childhood, suggesting a diagnosis of benign hereditary chorea (BHC). However, cerebellar ataxia and oculomotor apraxia, without telangiectasia, subsequently developed. Chromosome analysis
## Abstract Benign hereditary chorea (BHC) recently has been associated with mutations in TITFβ1 gene, although a pathological study of an individual with BHC and a TITFβ1 mutation revealed no significant gross or microscopic abnormalities using standard methods. Immunohistochemical staining of str
## Abstract Benign hereditary chorea (BHC) has been characterized as an autosomal dominant disorder manifesting nonprogressive chorea without dementia. However, there has been controversy regarding its existence. Diagnosis has been based solely on clinical criteria with many patients and families d
Snmmerp: Benign hereditary chorea (BHC) is an autosomal dominant disorder characterized by chorea of early onset with little or no progression. There is marked clinical variability in this disease with some subjects having onset in infancy and others with onset in early adulthood. In contrast to Hun