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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