Case of the Week
Section Editors: Matylda Machnowska1 and Anvita Pauranik2
1University of Toronto, Toronto, Ontario, Canada
2BC Children's Hospital, University of British Columbia, Vancouver, British Columbia, Canada
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July 2, 2012
Multiple System Atrophy-Parkinson Type (MSA-P)
- One of the "Parkinson plus syndromes"
- Cause unknown. More common in men (55%), 5th - 6th decade.
- Symptoms include autonomic dysfunction, parkinsonism (rigidity, tremors, akinesia), and ataxia (unsteady gait, poor co-ordination).
- Key Diagnostic Features: Cerebral volume loss more than expected for patient's stated age. Presence of posterior lateral putaminal atrophy, and peripheral slit-like T2 hypointensity is more specific (85% specificity).
- DDx: Multiple system atrophy-cerebellar type, Parkinson's disease
- No Rx. Treatment directed toward controlling symptoms: Levo-dopa. Poor prognosis. Eighty percent are disabled within 5 years of onset of motro symtoms, and only 20% survive beyond 12 years of disease onset.