Kuru

This code represents Kuru, a rare, incurable, and fatal neurodegenerative disorder. It is a transmissible spongiform encephalopathy (TSE) caused by prions, characterized by progressive cerebellar ataxia, tremors, and eventually dementia. The disease was historically prevalent among the Fore people of Papua New Guinea due to ritualistic endocannibalism.

Use this code when documentation clearly indicates a diagnosis of Kuru. This diagnosis is typically made based on clinical presentation in individuals with a history of exposure, particularly in endemic regions or populations. Supporting documentation would include neurological examination findings consistent with cerebellar dysfunction and a relevant epidemiological history.

• Kuru is extremely rare; ensure documentation explicitly confirms this specific prion disease.
• Do not confuse with other prion diseases like Creutzfeldt-Jakob disease (CJD) (A81.0x) or Gerstmann-Sträussler-Scheinker syndrome (A81.82).
• This code is highly specific and should only be assigned when Kuru is definitively diagnosed.