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Washington University Experience | NEURODEGENERATION | Multiple Systems Atrophy (MSA) | 2A0 Case 2 History
Case 2 History ---- At 55yo this male patient finished treatment for hepatitis C and had the gradual onset of difficulty rising from low chairs, smaller steps when walking with an unsteady gait along with urinary frequency, urgency, incontinence and erectile dysfunction. He had his first fall at age 57. He developed trouble swallowing and over the next year developed vertical diplopia. His handwriting worsened becoming slower, shakier, micrographic and less legible. Crying and laughing seemed much easier and walking worsened resulting in use of a walker by age 59. He continued to fall, usually backward. His speech became softer and more monotone. His face was less expressive but intellect was relatively well preserved. MMSE was 28/30 at age 59. His gait was wide based with hesitation on initiation and turning en bloc. He had short steps with bilaterally decreased arm swing. He had mild facial and bilateral upper extremity resting and action tremors. His UPDRS subscale III was 58/108 with symmetric measurements. Carbidopa/levodopa 25/100 4 tabs TID provided modest benefit at best. He had vivid dreaming without any waking hallucinations or delusions. The symmetry of his symptoms and signs at onset, the rapidity of progression, the presence of atypical features early in the illness and relatively modest response to levodopa supported the clinical diagnosis of multiple system atrophy (striatonigral degeneration type) and made the diagnosis of idiopathic Parkinson disease unlikely. Progressive supranuclear palsy remained in the differential diagnosis due to the presence of frontal muscle dystonia and abnormal eye movements. He died at the age of 60 of respiratory failure secondary to aspiration pneumonitis.
