Creutzfeldt-Jakob Disease: A First Case Series from a Tertiary Hospital in Malaysia and Review of Literature in Southeast Asia
نویسندگان
چکیده
Creutzfeldt-Jakob disease is a rare, human transmissible prion disease which carries a grave prognosis with no specific treatment. Although widely reported in English literature, reports of Creutzfeldt-Jakob disease in Southeast Asia are scarce. The incidence of Creutzfeldt-Jakob disease in this region is unknown. This raises the question of whether Creutzfeldt-Jakob disease is actually rarer in Southeast Asia or under-reported. We retrospectively reviewed the case-mix records of a tertiary hospital in Malaysia from 2009-2013 and found only 4 cases of probable CreutzfeldtJakob disease. Here we provide an illustrated report of 2 patients with probable Creutzfeldt-Jakob disease presenting initially with non-specific symptoms of lethargy. Subsequently both patients developed rapidly progressive dementia, myoclonic jerks and various neurological features including visual, extrapyramidal and neuropsychiatric manifestations. Magnetic resonance imaging of brain, electroencephalogram and cerebrospinal fluid 14-3-3 protein provided supportive evidence for the diagnosis. Both patients deteriorated rapidly and succumbed within 3 months of presentation.
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