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Washington University Experience | PRION DISEASES | Prion Diseases | 4A0 Case 4 History
Case 4 History ---- This 64-year-old woman had a medical history significant for breast cancer in 2003 (with Chemo and Radiation Rx). There is no family history of dementia. She presented with a one-year history of rapidly progressive mental confusion and deterioration. Initially, she was treated with medications, then by ECT in July 2005 but was more confused after two weeks of ECT. On psychometric testing, she scored poorly globally. An MRI in June 2005 showed diffuse cerebral atrophy and a few scattered white matter lesions. The differential diagnoses included severe clinical depression and possible intervening confusion during ECT, CJD, inflammation or infection. An EEG was ordered, which showed no focal findings. CSF analysis in January 2006 showed a normal 14-3-3 protein level. In 1/2006 she still had severe depression even with psychiatric therapy. Her neurological exam revealed a flat affect, tearfulness, slowed psychomotor function and mild gegenhalten. Her psychiatrist discontinued all antidepressants with no major changes in cognition. Namenda and Aricept were gradually stopped. By April 2006, she required full assistance with personal care and was attending Adult Day Care five days/week. In May 2006, she was placed in a nursing home. She had no spontaneous speech and was resistive to commands. No extrapyramidal signs were noted and there were no abnormal movements other than an increased startle reflex. Differential diagnoses included: 1) rapid atypical dementia including CJD or other prion disease; 2) unusual diffuse neoplastic or infectious process not diagnosed; 3) severe depression; 4) highly atypical Alzheimer’s disease. She was having frequent falls at the nursing home. In summary, she had longstanding confusion/depression (3-4 years) which culminated in rapidly progressing dementia over two years to the point of being wheelchair bound, mostly mute, and needing assistance with all ADLs, including feeding. She died in 7/2008 at age 66 due to pneumonia.
