Prion Diseases (Chronic Form: Genetic CJD, Fatal Familial Insomnia)

Prion Diseases (Chronic Form: Genetic CJD, Fatal Familial Insomnia) is indexed under Rare / Orphan Chronic, Prion Disease. Also known as genetic CJD, FFI, GSS.

US prevalence

~10-15% of CJD is genetic (~300 cases/year total; ~30-45 genetic) Source: CDC CJD Surveillance; NINDS. This figure comes from a professional society, registry, or study rather than a federal source. Label: REPORTED.

ICD-10-CM codes

A81.0, A81.9

Typical onset

Genetic forms: 30-60 years

Course

progressive

Body systems

neurological

Sex differences

Equal (autosomal dominant)

Racial and ethnic differences

Certain PRNP mutations more common in specific populations (e.g., E200K in Libyan Jews)

What this page does not establish

These figures describe populations, not individuals, and none of them is a diagnosis or a treatment recommendation. Prevalence estimates are approximate and reflect published ranges. Disparity figures are reported patterns, not causes.

Taxonomy last reviewed 2026-05-19. Each figure on the interactive page carries its own source and retrieval date, which can be later than this review.

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