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Washington University Experience | PRION DISEASES | Prion Diseases | 22A0 Case 22 History
Case 22 History ---- This 57 year old female was in good health until March of 1977 when she began to notice dizziness, vertigo, and blurred vision. Her family noted that she had some problems with memory loss and she continued to deteriorate steadily with these complaints. She eventually developed a gait disturbance, aphasia, inappropriate affect, posturing of her right arm, and myoclonic jerks. In July of 1977 she was admitted for an evaluation of her progressing dementia. She was oriented to name and place, but had marked perseveration. She had startle myoclonus. She had athetoid posturing of her right upper extremity. She had bilateral paratonia and numerous frontal release signs. Her gait was ataxic. She had normal CSF studies. The patient had an abnormal EEG in that there were multi-focal spikes and sharp wave complexes. In September again she was readmitted for possible pneumonia. Her mental status exam at that time had changed remarkably from her initial evaluation in the hospital. She was unresponsive to verbal stimuli and only winced in response to noxious stimuli. She had constant dystonic movements of the face and intermittent myoclonic jerks of all 4 extremities. She had almost constant "sundown" appearance of her eyes. She had tonic rigidity of the head and neck with flexion contraction of both hands. The EEG done at this admission showed periodic triphasic sharp waves with bifrontal predominance, left greater than right. The last admission was in November when the patient was readmitted for GI bleed. She was found to have a serum sodium of 113 at that time with a serum osmolarity of 229 . Her diagnosis at that time was that of inappropriate ADH in addition to the GI bleed and numerous decubitus ulcers. The patient died on December of 1977 in a nursing home.
