familial Creutzfeldt-Jacob disease

Last reviewed 01/2018

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