Multiple System Atrophy

Multiple system atrophy is a rare neurodegenerative disorder combining parkinsonism (tremor, slow movement, muscle rigidity, and postural instability) with autonomic nervous system failure and loss of coordination, caused by progressive degeneration of neurons in the basal ganglia, inferior olivary nucleus, and cerebellum. It was first described in 1960 by Milton Shy and Glen Drager and is also known as Shy-Drager syndrome; autonomic symptoms commonly include a sudden drop in blood pressure on standing, erectile dysfunction, reduced sweating, dry mouth, urinary problems, and vocal cord paralysis, and the disease is often mistaken for Parkinson's disease, though patients typically respond poorly to the dopamine drugs used for it. Research points to a misfolded, prion-like form of the protein alpha-synuclein within affected neurons as a likely cause, though the exact mechanism is unknown; the disease affects men somewhat more than women, usually begins between ages 50 and 60, and carries a life expectancy of about 6 to 10 years from symptom onset, with roughly 60 percent of patients needing a wheelchair within five years.