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Washington University Experience | PRION DISEASES | Prion Diseases | 17A0 Case 17 History
Case 17 History ---- The decedent was a 46 year old man with a rapidly progressive dementia. His past medical history also included depression which had worsened in the last three years. He had a father who had a stroke at the age of 37 but lived into his 70’s. He had a maternal grandmother who was institutionalized. His immediate medical history began during the month of April when he changed his diet dramatically in response to health complaints (kidney stone, gastroesophageal reflux disease). At the end of April he had a notable change in vision affecting his right visual field (noticed in the course of a day). At the time he and his family members also noticed a change in his gait. He complained of right hand weakness. He had a fall in mid-June and was diagnosed at an OSC as a basal ganglia stroke. He was sent to a rehabilitation facility but his symptoms over the next two weeks were rapidly progressive with increasing changes in cognition. He stopped ambulating and required assistance to transfer, toilet, and keep up hygiene. He stopped eating and required first pureed then tube feeds. The decedent was admitted to Barnes-Jewish Hospital at the end of June for a workup of his dementia. His EEG was abnormal on admission showing frequent generalized periodic epileptiform discharges of left hemispheric predominance and intermittently left hemispheric slowing along with moderate generalized slowing. An EEG showed left frontal central periodic lateralized epileptiform discharges (PLEDs) as well as left hemispheric slowing and moderate generalized slowing. MRI showed diffusion restriction in the left parietal and occipital cortical grey matter, both basal ganglia, and both medial thalami with corresponding FLAIR/T2 hyperintensity. A PET showed diffuse hypometabolism involving the left putamen and caudate as well as the superior aspect of the left frontal and left parietal lobes. Hypometabolism was also seen in the occipital lobes. Workup for reversible causes of dementia were negative. A cerebrospinal fluid Tau and 14-3-3 levels were consistent with a prion disease (assayed at the NPDPSC). He died in 7/2011 with a course of 3-4 months.
