ICD-11 classes
01 Certain infectious or parasitic diseases
Human prion diseases
8E02 — Genetic prion diseases
8E02.2 — Fatal familial insomnia

ICD-11 8E02.2 — Fatal familial insomnia

A disease of the brain, caused by inheritance of mutation(s) of normal prion protein genes. This disease is characterised by severe insomnia and autonomic system dysfunction, and is fatal. Confirmation is by pathological examination of the brain and genetic testing.

The diagnosis includes nothing.

The diagnosis excludes nothing.

It has no clarifying diagnoses.

The diagnosis is coded elsewhere:

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