Familial Creutzfeldt-Jacob disease
The familial forms of Creutzfeldt-Jacob (CJD) disease:
- have a wide spectrum of clinical features
- may vary in its clinical features between and within families
- have an autosomal dominant mode of inheritence
- account for 10% of cases of CJD
In general:
- familial CJD presents earlier, from 40 years on
- the clinical course is longer
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