All of the following statements about CreutzfeldtJakob disease are true, EXCEPT :
Answer is C (Myoclonus is rarely seen): `Myoclonus is a very common manifestation of C-.1 disease. 'Most patient (90%) with CJD exhibit myoclonus that appears at various times throughout the illness' - Harrisons Creutzfeldt-Jacob disease :Review Nature of illness : Heritable, degenerative, infectious disease. Creutzfeldt- Jacob disease is the most common prion disease (infectious) in humans Q It is a neurodegenerative disease with major pathological changes confined to CNS Q -Harrisons 16th/ 2496 The prion concept explains how this disease can manifest both a heritable Q as well as an infectious illness. Q Clinical manifestations : Non specific prodromal symptoms : Fatigue, sleep disturbance, weight loss, headache, malaise, ill defined pain Dementia e (deficit in higher coical functions) : Memory loss, impaired judgement, decline in intellectual function Myoclonus Q:Most patients (90%) exhibit myoclonus at some time throughout illness. Cerebellar signs visual impairment may occur Characteristic ECG finding Q: * Paroxysm of high voltage and slow waves Treatment * There is no specific treatment.
It is a neurodegenerative disease
It is caused by infectious proteins
Myoclonus is rarely seen
Brain biopsy is specific for diagnosis