8E02.2

ICD-11 MMS 2026-01

Fatal familial insomnia

Description

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.

Index terms

  • Fatal familial insomnia
  • FFI - [fatal familial insomnia]

Exclusions from above levels

  • 05 – Endocrine, nutritional or metabolic diseases
  • 18 – Complications of pregnancy, childbirth and the puerperium
  • 19 – Certain conditions originating in the perinatal period
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  • 22 – Injury, poisoning or certain other consequences of external causes

Sibling codes

  • 8E02.0 – Genetic Creutzfeldt-Jakob disease
  • 8E02.1 – Gerstmann-Straussler-Scheinker syndrome
  • 8E02.Y – Other specified genetic prion diseases
  • 8E02.Z – Genetic prion diseases, unspecified

Foundation entity

Fatal familial insomnia – http://id.who.int/icd/entity/669154658

Allowed postcoordination axes

Part of: 8E02: Genetic prion diseases › 08: Diseases of the nervous system