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Washington University Experience | PRION DISEASES | Prion Diseases | 7A0 Case 7 History
Case 7 History ---- The patient was a 68-year-old male with a history of hypertension and hyperlipidemia, who presented in early February 2010 with a four month history of unsteady gait with progressive symptoms, including recurrent falls. During that admission, he was noted to have significant cerebellar dysfunction, including nystagmus, ataxia, dysmetria and ataxic speech. An MRI showed mild global atrophy and microvascular leukoencephalopathy, but otherwise was normal. An anti-potassium channel antibody and paraneoplastic screen were negative. His CSF studies showed mild pleocytosis, lymphocytic dominant with normal glucose and protein, and normal cytology. A 14-3-3 was also sent (NPDPSC) which was positive with markedly elevated total tau protein of 5,760 pg/ml (normal <1,200 pg/ml). Within a month he had worsening symptoms involving gait and significant rapidly progressive cognitive decline. His memory was very poor with recall zero out of three at one minute and very poor attention span. He had severe dysarthria, and his speech was ataxic. His extraocular movements were full, however, with persistent intermittent rotatory nystagmus. Motor examination revealed 5/5 strength throughout. He had choreiform movements of his upper extremities, with significant proprioceptive problems, in addition to severe ataxia. His tone was increased throughout. Coordination demonstrated severe ataxia and severe dysmetria. The patient was unable to ambulate without support due to significant ataxia. An EEG showed moderate generalized slowing. Repeated MRI scan of the brain, however, showed diffusion signal abnormalities, over the right caudate nucleus and right lobe. The patient was discharged to an extended care facility with a likely diagnosis of CJD. The patient expired in 4/2010, 8 months after his first symptoms.
