Background: Multiple system atrophy is a sporadic, progressive, neurodegenerative disease of undetermined etiology characterized clinically by extra pyramidal, pyramidal, cerebellar, and autonomic dysfunction in any combination. It is recognized by various names as Shy-Dragger syndrome, Striato-niagral degeneration and sporadic Olivopontocerebellar atrophy depending upon the predominant presentation. Objective: To study the variability in initial clinical presentation and progression of patients of multiple system atrophy. Material and Methods: Six patients of MSA diagnosed during December 2008 to March 2010 in a tertiary care teaching hospital were analyzed. Bedside autonomic function testing and MRI Brain was done for all these patients. Results: Five patients satisfied the criteria for probable MSA, one patient for possible MSA. Four patients were sub categorized as MSA-C and two as MSA-P. All patients had remarkably different modes of presentation and findings at onset. Duration between initial symptoms and combined motor and autonomic dysfunction varied in all of these patients. Conclusion: The clinical presentation of MSA is variable even within a particular subtype. Detailed clinical evaluation including Autonomic functions (and close follow-up) is essential for diagnosis.

Keywords: Multiple System Atrophy; Variable Presentation; Autonomic Dysfunction.