Multiple systems atrophy (MSA)/Shy Drager Syndrome

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Multiple systems atrophy (MSA)/Shy Drager Syndrome:
Sporadic progressive neurodegenerative group of diseases that produces various combinations of Parkinsonism, ataxia, autonomic dysfunction and spasticity. Often misdiagnosed (differential from Parkinsons disease may not be made until autopsy). Usually > 50 yrs. M>F.
Worse prognosis than Parkinsons disease. Cause unknown.
Wide range of variable clinical features – stiffness, rigidity, slowed movements, postural instability, ataxia, orthostatic hypotension, impotence in males, urinary and bowel complications, blurred visions, speech and swallowing difficulties. Autonomic dysfunction usually occurs first, but the movement disorder(s) cause greater disability.
Management – levadopa sometimes effective, but usually get a poor or transient effect; drugs to raise blood pressure for orthostatic hypotension (eg midodrine); speech therapy; lifestyle modifications due to orthostatic hypotension

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