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Washington University Experience | PRION DISEASES | Prion Diseases | 8A0 Case 8 History
Case 8 History ---- This retired teacher was seen at age 59 who had several family members with a history of dementia that started in their 80s - her mother, a sister, and a brother. Her husband stated there was a 5-8 month history of decline in memory and thinking. Her short- and long-term memory had progressively worsened over 5 months She was more withdrawn, and she stated she was more depressed due to her difficulties. Her medicines included Lexapro, Trazodone and Alprazolam. Her neurologic exam was unremarkable. Psychometric test scores were in the moderately impaired range in semantic memory and mildly impaired in episodic memory. The clinician rated her CDR 1, early onset dementia of the Alzheimer's type (DAT). Several lab tests were ordered as well as a lumbar puncture to rule out CJD. The result of the CSF 14-3-3 test was at a level that was only slightly above normal. She was disoriented to place and was unable to carry out daily activities such as cooking. She could follow simple 2-step commands but was unable to remember a recent event. The clinician rated her CDR 2, DAT, and stated that this was clearly a rapidly progressing course that could conceivably represent CJD or FTD. Her last visit was on 4/09 at age 60. Her memory and thinking problems continued, with a marked decline in her language ability. She had little speech output and could only understand simple commands. She died on 6/2011 at age 62 of end stage dementia and inanition. She was mute, unable to walk, and required total nursing care. Her husband stated she became very stiff and could not move any muscles in the last 10-12 months. Her arms hung down at her sides and, if she tried to reach out, she would become jerky. There was no motor impairment, no myoclonus, no frontal signs, and a language disorder that was mostly semantic. There was a strong family history of dementia with one other known case with an early onset and a diagnosis of Alzheimer’s disease was thought most likely.
