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Washington University Experience | NEURODEGENERATION | Progressive Supranuclear Palsy (PSP) | 17A0 Case 17 History
Case 17 History ---- The patient was a 79 year old female with a gait disorder that began in 1996. She had frequent falls and declined rapidly over 1 year to the point of requiring a wheelchair. She developed left arm elevation and inability to move the right arm or leg secondary to freezing. She had an intermittent left action tremor. Her speech was soft. She showed no response to Sinemet. A brain MRI revealed nonspecific periventricular white matter disease. She had a history of coronary artery disease with prior coronary bypass surgery. Her neurological exam revealed dementia, moderate language impairment, saccadic smooth pursuits, a paucity of facial expression, asymmetric increased clasp-knife tone (right greater than left), bradykinesia, apraxia, akinesia, dystonia with continuous elevation of the right arm at the shoulder, flexion at the elbow, and extension of the elbow. She had flexion of the right leg at the hip and dorsiflexion of the right foot. She had marked stooped posture. She had very short steps, freezing and postural instability. She was diagnosed with cortico-basal ganglia degeneration with parkinsonism and alien hand syndrome. She was treated with Botox injections. She progressively declined with fecal impaction and poor oral intake. She developed deep vein thrombosis and pulmonary embolus and passed away.
