遗传性进行性舞蹈病，无痴呆症状。
Hereditary progressive chorea without dementia.
作者信息
Schady W, Meara R J
机构信息
University Department of Neurology, Manchester Royal Infirmary, UK.
出版信息
J Neurol Neurosurg Psychiatry. 1988 Feb;51(2):295-7. doi: 10.1136/jnnp.51.2.295.
Abstract
A family with hereditary non-Huntington's chorea is presented. Transmission was autosomal dominant with variable penetrance. Chorea commenced in childhood and affected predominantly the head, face and upper limbs. Dysarthria appeared later, followed in two family members by elements of an axial dystonia. There was no intellectual impairment. Unlike previously described families, symptoms progressed steadily up to the eighth decade, causing considerable physical disability.
摘要
本文报告了一个患有遗传性非亨廷顿舞蹈症的家族。其遗传方式为常染色体显性遗传，外显率可变。舞蹈症始于童年，主要影响头部、面部和上肢。构音障碍随后出现，两名家族成员继而出现轴性肌张力障碍的症状。没有智力障碍。与之前描述的家族不同，症状一直稳步发展至八十岁，导致严重的身体残疾。
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本文引用的文献
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2
Benign (non-paroxysmal) familial chorea. Paediatric perspectives.良性（非阵发性）家族性舞蹈症。儿科视角
Arch Dis Child. 1981 Aug;56(8):616-21. doi: 10.1136/adc.56.8.616.
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Benign familial chorea with onset in childhood.儿童期起病的良性家族性舞蹈症。
JAMA. 1973 Sep 24;225(13):1603-7.
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Hereditary nonprogressive chorea of early onset.早发性遗传性非进行性舞蹈症。
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