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Washington University Experience | PRION DISEASES | Prion Diseases | 26A0 Case 26 History
Case 26 History ---- This patient was a 78 year old male in his usual state of excellent health until approximately 2 weeks prior to admission when he was noted to be mildly paranoid and forgetful. This rapidly progressed to abusive and belligerent behavior accompanied by deterioration in his function within the community. In particular he became unable to drive his automobile in an appropriate way running through red lights, starting and stopping erratically, and losing his way. Investigating his apartment his family found it disorganized with remnants of food lying about. His family also noted that his gait became unsteady and that he appeared weaker and thinner than usual. There was no prior history of medical, psychiatric, or neurologic disease. The patient was admitted to Barnes Hospital in December 1985. His admitting general physical examination was remarkable an uncooperative attitude, evidence of paranoid ideation with the expressed fear that doctors were attempting to cause his death. He appeared to have little insight into the events leading up to his admission. His cranial nerves were remarkable only for a small cataract in the right eye. There was mild appendicular ataxia and wide based ataxic gait. Spontaneous mild myoclonic jerks were elicited with startle. An EEG obtained in 12/85 demonstrated persistent slowing in the absence of normal waking rhythm while a followup EEG was moderately to severely abnormal with persistent slowing in the absence of and absence of normal waking rhythm and reactivity while also containing multifocal generalized and focal left temporal sharp waves. Throughout the course of his hospitalization the patient demonstrated a continuous decline of intellectual function and the loss of self care skills such that by the time of his discharge on l/21/86 he required total nursing care and was unable to feed himself. At the time of his discharge the patient was noted to be lying in bed with his eyes open and a gaze preference to the left. He did not respond in any meaningful way to speech or touch. He did develop an exaggerated startle response both to verbal and tactile stimulation. Prominent myoclonus was present both awake and asleep and especially upon stimulation. The patient did not localize painful stimuli. His deep tendon reflexes were increased symmetrically and the platter responses were bilaterally flexor to Babinski stimulation. There was a positive snout reflex. The patient was discharged home to his family after careful instruction of gastrostomy feedings and other matters of care. The patient subsequently died of pneumonia at an OSH.
