All of the following statements about Creutzfcldt-Jakob disease are true, EXCEPT
(C) Myoclonus is rarely seen # Creutzfeldt-Jakob disease is a neurodegenerative disease caused by Prions> Myoclonus is seen in 90% of patients with Creutzfeldt - Jakob disease.> Prions are infectious proteinaceous material devoid of detectable amount of nucleic acids.> Characteristics of diseases caused by Prions - Diseases are confined to central nervous system Basic pathology is - Neurodegeneration - Spongiform encephalopathy> Presence of Amyloid plaques in brain Hosts shows no inflammatory response (Disease is almost always fatal)> Clinical features (Symptoms): Usually presents in middle ages Rapidly progressive dementia Myoclonic fasciculations (seen in 90%) Ataxia Somnolence> Characteristic EEC pattern - Paroxysms with high voltage and slow waves
It is a neurodegenerative disease
It is caused by infectious proteins
Myoclonus is rarely seen
Brain biopsy is specific for diagnosis
Miscellaneous