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Washington University Experience | PRION DISEASES | Prion Diseases | 9A0 Case 9 History
Case 9 History ---- The patient was a 73 year old woman with a history of atrial fibrillation in her usual state of health when she presented with approximately two weeks of altered mental status, dysarthria, dizziness, and falls. Workup testing showed an unremarkable non-contrast head computed tomography (CT) scan and CSF with no white cells. MRI in December 2012 showed diffusion-weighted and FLAIR sequence abnormalities in the cerebral cortex with a differential diagnosis that included CJD, viral encephalitis, metabolic encephalopathy, and paraneoplastic syndrome. Testing for metabolic, infectious, autoimmune, and paraneoplastic etiologies were all negative. CSF analysis at the NPDPSC showed the abnormal presence of tau protein and an ambiguous 14-3-3 level; these findings are consistent with a diagnosis of prion disease. During her hospitalization she was noted to have myoclonic jerks. Given the clinical picture of rapidly progressive dementia with myoclonic jerks, MRI abnormalities, and CSF tau and 14-3-3 levels, CJD was thought most likely. She died in 9/2013, a course of less than one year duration.
