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Washington University Experience | NEURODEGENERATION | NBIA1 (PKAN, Hallervorden-Spatz Dz) | 2A0 Case 2 History
Case 2 History ---- The patient was a 67 year old man who carried a diagnosis of probable multisystem atrophy (MSA) and transient left hemiparesis. At presentation at age 57 years, the patient noted initially resting tremor of his right thumb and relatives commented on his decreased facial expression. One year later he was unstable with a stooped posture, stumbling and falling. Sinemet and deprenyl provided no benefit. On neurologic examination, he had normal mentation, moderate to severe hypophonia, dysarthria, masked facies and appropriate affect. His cranial nerve functions were normal. Motor examination showed normal strength with decreased fine finger movements bilaterally. He had bradykinesia during tapping movements and there was no ballismus or chorea; no dystonia, tics or myoclonus. He had a 3 Hz resting tremor. He had a normal gait and base with no ataxia, propulsion or retropulsion detected. There was moderately decreased arm swing bilaterally. He had normal deep tendon reflexes except for an absent left ankle reflex. The clinical impression was stage 3 parkinsonism with slightly asymmetrical resting tremor, relatively symmetric bradykinesia and rigidity. The likely etiology was thought to be striatonigral degeneration or idiopathic Parkinson disease. The patient developed blepharospasm over the next years. Both eyes remained closed most of the time but responded to treatment with Botox injections. The patient died after prolonged hospitalization resulting from other medical conditions.
