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Washington University Experience | NEURODEGENERATION | Multiple Systems Atrophy (MSA) | 15A0 Case 15 History
Case 15 History
This 76yo male first developed unsteady walking at age 59. He had trouble pulling on his pants when standing. By age 60, he could not walk a straight line. At age 61, he had difficulty pronouncing words clearly but the voice volume was OK. Hand coordination became impaired at that time with both hands; handwriting was sloppy but not small. He had his first fall at age 61, as gait became less steady. At age 62, he could not hammer a nail. Exam at age 62 in the MDC revealed appendicular and truncal ataxia, scanning dysarthria, ocular overshoot, but no extrapyramidal features. Over the next several years, he had increasing clumsiness of the hands, more falling, more impaired speech and depressive symptoms. By age 67, he had a clear pan-cerebellar syndrome with gait ataxia, lower greater than upper extremity dysmetria, ocular dysmetria, and scanning dysarthria but that year he also developed bradykinesia, rigidity and masked facies. However, these Parkinsonian manifestations remained modest but that year he developed freezing of gait and some orthostasis with more bladder dysfunction. Levodopa may have provided modest benefit. By age 68, orthostasis became severe requiring medication. By age 72, he had stage 5 Parkinsonism with marked ataxia, autonomic insufficiency and then developed modest supranuclear gaze palsies. He also had a right Horner’s syndrome. By age 74, he had more supranuclear gaze palsies. He also had left hand dystonia. By age 73, the left hand dystonia progressed. He had trouble with saccade and smooth pursuits either up or down with some limitation on horizontal gaze. Axial rigidity was greater than appendicular rigidity. He became nearly anarthric. He had intermittent action tremor of either hand. He was on hospice for the last 10 months. He had progressive trouble eating and swallowing. He had almost nothing to eat in the last week and minimal to drink in the last 5 days. All medications were stopped for the last 5 days. He died of dehydration and inanition at home.
