Abstract

Introduction: Cruetzfeldt Jakob disease (CJD) is a form of neurologic and mental illness associated with progressive central nervous system degeneration, first described by Creutzfeldt and Jakob in the early 1920s. CJD principally affects the grey matter of cerebral cortex, brainstem and molecular layer of the cerebellum. It is believed to be caused by histologically unconventional infectious agents called PRIONS. It is uniformly fatal and the annual incidence rate is 1–2 per million worldwide. Most cases are sporadic, but 5–15% of cases have a genetic basis and have been clustered within families. It is inherited as an autosomal dominant trait with variable penetrance.

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