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Washington University Experience | NEURODEGENERATION | Progressive Supranuclear Palsy (PSP) | 8A0 Case 8 History
Case 8 History ---- The patient was a 74yo male who initially noticed gradual humping of the shoulders and slower walking around 1984. He noted the insidious onset of smaller, less legible handwriting in 1990. At that time, it was also hard for him to button collars on his shirts. In 1991, he noticed that it was harder to brush his teeth and shave, and there was a report of more emotional lability, specifically described as crying spells. He gradually developed softer speech. By 1992, he began falling backwards and sideways, retropulsing while he walked. It was harder for him to rise from chairs. Freezing with starts, turns and at doorways and tight spaces also became more prominent. He began to develop swallowing problems and subtle signs of aspiration, including coughing while eating and drinking. He continually felt as if food was “hung up” in his mid-throat.” Within the next two years, his voice and walking worsened and he was falling up to 3-4 times per week. He did not have significant orthostasis initially and he had little constipation with no urinary dysfunction. He did have difficulty with erections by 1990. By 1994, he occasionally had double vision on vertical gaze. His first visit to the MDC was in 3/1994. He had already been started on Selegiline, and he had been taking Sinemet CR. His past medical history was significant for malaria and fever with delirium for 7 days at age 12-13; appendectomy in 1989; cervical spinal stenosis and myelopathy in 1998; and, B12 deficiency requiring parental supplementation since 1997. Initial neurologic exam showed no orthostasis, orientation was slightly affected, memory was 2/3 at 3 min, and he had trouble naming the presidents and doing serial 7’s. He had decreased upgaze with normal downgaze, and “ratchety” smooth pursuits. No Kayser-Fleischer rings were found. He had 1(+) facial masking. 1+ rigidity was present in the left upper extremity and the right upper extremity, and he had bilateral 1+bradykinesia. He had no dystonic posturing nor choreiform movements. Gait was remarkable for initial hesitation, small steps, moderate retropulsion, decreased left > right arm swing, and occasional freezing on turning. He had a 3 step en bloc turn, and his postural stability was poor on the pull test. Despite a trial of over 1 gram of Sinemet a day over the next few years, he never had dramatic improvement in his exam. He developed further emotional lability and a supranuclear gaze palsy manifested by inability to gaze vertically, either up or down. He developed significant gait dysfunction so that he was falling every day. He also manifested severe dysphagia by 1997, choking on his saliva and on food. He rarely spoke by 6/99, and developed blepharospasm requiring botulinum toxin injections by 2002. He also had poor sleep and visual hallucinations which occurred only after starting Sinemet and Pramipexole. In his last office visit in 4/2003, a note stated that his memory was good and he required assistance with bathing and dressing, but not with eating. He still would fall when he would try to walk. He had poor control of bowel and bladder function. Action or postural or at rest tremors were absent. Rigidity was substantial in the neck and less at other sites. He passed away in 4/2003 of unknown causes.
