Table of Contents
Washington University Experience | NEURODEGENERATION | NBIA1 (PKAN, Hallervorden-Spatz Dz) | 9A0 Case 9 History
Case 9 History ---- This patient was seen first when he was 84 years old with a past history of Parkinson's disease (PD), with symptoms beginning 4 years previously with increasing difficulty walking. Symptoms improved somewhat on Sinemet. Gradual progressive changes in his memory were noted beginning approximately 3 years prior to a subdural hemorrhage sustained in a fall requiring surgical evacuation. He had bradykinesia, difficulty arising from a chair, walked with short steps and had a flexed stooped posture, difficulty turning en bloc and diminished tandem gait. He had a positive snout reflex, a mild jaw jerk, bilateral palmomental reflexes and positive perseveration. The attending clinician diagnosed an affective disorder with parkinsonism preceding an Alzheimer type dementia. He entered a nursing home a year prior to his death due to increased severity of his parkinsonism and memory issues. He exhibited diminished vertical upgaze and downgaze and extraocular movements were described as being slow and with saccades. His clinician believed the eye movement difficulty coupled with his gait disturbance and only some improvement noted with Sinemet, was consistent with the specific diagnosis of progressive supranuclear palsy and not Alzheimer disease. ---- At autopsy the unfixed brain weighed 1280g.
