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Gerstmann Straussler Scheinker disease
1. General outline
Familial disease due to specific mutations on prion gene. Similar to Creutzfeld-Jacob
disease, but is exclusively wit a genetic origin, and is distinguished by
the characteristic multicentric amyloid plaques in the brain.
2. Synonyms GSS
3. Course (Evolution of the disease):Dementia first, followed
by inconstant myoclonia, ataxia, epilepsy, moto neuron disease.
4. Caregiver problems: similar to CJD
5. Aetiology (Causes and risk factors)
6. Diagnostic procedures
Clinical symptoms + IRM , EEG, genetic tests
7. Treatment and rehabilitation: similar to CJD
8. Ongoing research
Vaccination against aggregated prion protein has been suggested
9. Available services
10. Further reading
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