Sporadic Creutzfeldt-Jakob disease presenting with nonconvulsive status epilepticus.
Creutzfeldt-Jakob disease (CJD) is a rare prion disease characterized by a spongiform encephalopathy in humans. Although the characteristic triad of myoclonus, dementia, and periodic EEG activity is easy to recognize, unusual manifestations of the disease may be challenging and create a diagnostic dilemma. We report a case of CJD that occurred in a 26-year-old patient who presented with a receptive (Wernicke's) aphasia secondary to nonconvulsive status epilepticus.
Cohen
David
D
Department of Neurology, University of Michigan Health System, UH1B300/0036 Ann Arbor, MI 48109, USA.
Kutluay
Ekrem
E
Edwards
Jonathan
J
Peltier
Amanda
A
Beydoun
Ahmad
A
D000328:Adult; D001041:Aphasia, Wernicke / Q000150:complications / Q000523:psychology; D001921:Brain / Q000473:pathology; D007562:Creutzfeldt-Jakob Syndrome / Q000150:complications* / Q000175:diagnosis / Q000473:pathology; D004569:Electroencephalography; D017809:Fatal Outcome; D005625:Frontal Lobe / Q000473:pathology; D006801:Humans; D008279:Magnetic Resonance Imaging; D008297:Male; D013226:Status Epilepticus / Q000175:diagnosis / Q000209:etiology* / Q000473:pathology