Document Type

Case Report


Creutzfeldt-Jakob disease is lethal and most common spongiform encephalopathy worldwide and is rarely encountered in the Emergency department. For the benefit of patients and healthcare workers, rapid consideration of this condition needs to be sought in the setting of rapid neurological decline and myoclonic jerky movements. We present a case of a 54-year old male with behavioral changes, jerky body movements, cognitive dysfunction and left sided weakness developing over the course of few months and presented to our Emergency Department for the evaluation for stroke. Due to the non-specific nature of psychiatric and neurologic complaints, the initial diagnosis remained unclear. Magnetic Resonance Imaging of Brain demonstrated global atrophy with periventricular and subcortical T2 hyperintensities. Electroencephalogram showed continuous generalized periodic sharp wave activity with triphasic configuration. Investigations were highly supportive of probable Creutzfeldt-Jakob disease. Brain biopsy was planned but was not an acceptable option for suffering family. Our report showcased the symptoms, investigations, diagnostic challenges and raising concerns regarding this condition. It is advantageous for the emergency physician to be aware of clinical presentation of this rare condition. The prognosis for this disease is very poor and there is currently no cure.

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