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Washington University Experience | PRION DISEASES | Prion Diseases | 13A0 Case 13 History
Case 13 History ---- The patient is a 54 year old, right-handed female with a past medical history of diabetes mellitus. She suffered a decline in cognition as well as coordination and balance, and increased dyspnea with ambulation since May 2002. An MRI of the head and 2 LPs were all reportedly negative. She had a wide-based gait with a somewhat ataxic quality and decreased arm swing. The neurologic examination showed mild global cognitive deficit and hesitancy to questioning. Her speech had a scanning quality. Her cranial nerve examination was notable for sustained nystagmus, especially on right lateral gaze. There was increased tone in all extremities, and mild cog-wheeling was noted. Sensory examination was notable for decreased vibration and proprioceptive sense in her feet bilaterally. Coordination testing was notable for mild dysmetria in the right upper extremities and moderate dysmetria in the left upper extremities as well as difficulty with heel-to-shin test bilaterally. Deep tendon reflexes were 1/4 in the upper extremities, 2/4 in the knees bilaterally, 0/4 in the ankle and plantar reflexes were downgoing bilaterally. ---- In summary, the patient was found to have rapidly progressing ataxia and cognitive decline over the past month. EEG was performed and showed excessive slowing, compatible with toxic-metabolic encephalopathy. No paroxysmal features were identified. A paraneoplastic syndrome was suspected, although a primary neoplasm was not identified. Her mental status continued to decline, and the patient began to show signs of decreased orientation and attention to task. The patient passed away in July 2002.
