Related Subjects:
|Dementias
|Gerstmann-Straussler-Scheinker Syndrome (GSS)
|Fatal Familial Insomnia (FFI)
|Creutzfeldt Jakob disease (CJD)
|Variant Creutzfeldt Jakob disease (vCJD)
|Kuru
🧠 The word “kuru” means “to shiver” or “trembling in fear.”
It is a rare prion disease, now extinct, that spread among the Fore people of Papua New Guinea through ritual cannibalism.
Thought to have begun from a sporadic prion mutation (like sporadic CJD) which became transmissible through ingestion of infected brain tissue.
📌 About
- Rare, fatal, progressive neurological disease, mainly affecting the cerebellum (ataxia dominant).
- Classified as a transmissible spongiform encephalopathy (TSE).
- Historical epidemic in the 1950s–60s among the Fore people of New Guinea.
🧬 Aetiology
- Spread through ritual cannibalism, especially ingestion of infected brain tissue.
- Women and children were most affected since they were more involved in funerary cannibal practices.
🚶 Clinical Stages
- Ambulant stage: Headache, joint pain, shivering, gait ataxia, dysarthria, tremor, unsteadiness.
- Ambulant with help: Severe tremors, worsening ataxia, frequent falls.
- Chair/Bedbound: Advanced frailty, immobility, high risk of secondary infections.
- Unlike many other encephalopathies: no early fever, convulsions, or coma.
🔎 Investigations (historical)
- Routine bloods (FBC, U&E, LFTs) – non-specific.
- EEG: non-specific changes.
- CT/MRI: cerebral and cerebellar atrophy in late stages.
- CSF analysis: non-diagnostic.
🧩 Pathology
- Marked spongiform change with widespread plaques.
- Neuronal shrinkage, vacuolation (“moth-eaten” appearance).
- Purkinje cell degeneration in the cerebellum.
- Similar histology to scrapie and other prion diseases.
💊 Management
- No treatment or cure existed.
- Public health measure: stopping ritual cannibalism ended the epidemic.
📚 References
