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Washington University Experience | PRION DISEASES | Prion Diseases | 23A0 Case 23 History
Case 23 History ---- The patient was a 62-year-old man with a past medical history of abdominal aortic aneurysm, status post repair in 2011, hypertension and benign prostatic hypertrophy who presents with rapidly progressive dementia. Approximately 6-7 months prior to his death, his daughter noticed that he became more angry and agitated, which was unusual for him, and at other times seem depressed and reclusive; he had difficulty with executive function and became unable to care for himself. He complained of numbness on the bottom of his feet, poor balance, required a walker, and had jerking movements of his whole body. His memory grew progressively worse and he became non-verbal. He lost 50 pounds. The patient has a history of hunting and consumption of wild game in various regions of the United States and Canada. He received blood, platelets and plasma during surgery for his abdominal aortic aneurysm. Serum laboratory studies were unremarkable with exhaustive analysis. CSF sent to the National Prion Center showed an elevated tau level (4681 pg/mL) and was positive for 14-3-3 protein. MR imaging performed September 2014 showed multifocal cortical diffusion trace with left-sided predominance, and diffusion restriction within the caudate nuclei and putamina bilaterally, consistent with CJD. The patient was placed in hospice care and died October 2014.
