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Washington University Experience | PRION DISEASES | Prion Diseases | 18A0 Case 18 History
Case 18 History ---- The patient was a 51 year old man who had a clinical history of a rapidly progressive altered mental status that began in October 2012 when he began having some speech difficulties. He also had episodes of sudden loss of consciousness striking his head on the left side. After that time he began having difficulty focusing and began to show signs of being "off balance." These feelings were progressive and he was taken to an outside hospital emergency department where a CT scan was negative, but he was noted to have significant difficulty with speech and left arm numbness. At the beginning of November (2012), he was noted by a physician to have significant difficulty with his speech and shaking handwriting. His right eye was "glued shut." On his mental status examination he had difficulty spelling. An MRI performed in November 2012 was reported as negative but had concern for possible cortical ribboning and basal ganglia hyperintensity when reviewed by the neurology staff. Worsening of his speech, as measured by speech therapists, occurred in December. He was taken to the Emergency Room where there was concern about a stroke because of decrease in use of the right hand and increased stumbling. He was globally aphasic at that time following only minimal commands and his right side felt to be spastic and slightly hemiparetic. There was concern for possible CJD. The patient was admitted in late December 2012 because of his rapidly progressive dementia and clinical concern for Creutzfeldt-Jakob disease. CSF cytology and flow cytometry showed only scattered lymphocytes and acellular debris. MRI showed left cerebral cortical restriction diffusion with involvement of bilateral caudate nuclei, as well as the left anterior putamen and right putamen because differential diagnosis included CJD, paraneoplastic syndrome, and metabolic etiology. CSF tau was measured by the NPDPSC to be positive (5373 pg/ml) and 14-3-3 test was also positive. The patient died in the hospital in early January 2013.
