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Washington University Experience | NEURODEGENERATION | Progressive Supranuclear Palsy (PSP) | 7A0 Case 7 History
Case 7 History ---- This 75yo male first had soft voice, daytime fatigue, gradually slowing walking and right slower than left upper extremity starting at age 67. At age 68, pramipexole reduced slowness and increased gait speed. By age 70, he had a shuffling gait and his first fall (on ice). He also developed RBD symptoms (with flailing and kicking) that responded to melatonin and clonazepam. He had excessive daytime sleepiness from levodopa and also developed some mild wearing off. He also had obstructive sleep apnea. He did not have tremor, freezing, depression, anxiety, apathy, or hallucinations. Levodopa provided reasonable benefit. Parkinsonism remained more severe on the right. He remained cognitively intact. He then developed myelodysplasia in the last several years and this became increasingly progressive; this became his major medical problem. He did not have RLS, impulse control problems, depression, anxiety, apathy, hallucinations, or supranuclear gaze palsies. He needed a walker in the last year. Then, in the last few months, he needed a wheelchair. Speech was increasingly impaired. He did have freezing at that time without clear dopa benefit. He had some tremor in the last year in the right hand. He remained very bright and continued to handle all of his finances. He did develop some apathy. He developed myelodysplasia for the last 3 years without leukemic conversion. His WBC dropped in his last year. Blood counts dropped in the last few months. He also had more trouble with diabetes mellitus but his blood counts with WBC, RBCs and platelets were also very low. He was becoming weaker and weaker in the last several months. The clinical cause of death was myelodysplastic syndrome with respiratory arrest and on hospice
