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Friday, March 7, 2014
My husband (50)has been diagnosed with MultiSystem Atrophy (Shy Drager). Do you have some sort of remission with this disease or does it just slowly deteriorate the nervous system? He was put on stalevo and it seemed to help for about 2 months and his symptoms have slowly started returning- tremors, balance problems, orthostatic hypotension, mild confusion and impotence. Our neuro doc does not think a pet scan would help (he has a pacemaker) but I so hope this is not the disease he has. He has 9 stents due to hyperlipidemia and heart disease, diabetes. so it is hard to know what causes what symptom! Thanks,
Multiple System Atrophy or MSA is a group of syndromes that share a common pathological mechanism and some common clinical features. These syndromes used to be known separately as: Shy-Drager syndrome which had prominent autonomic nervous system dysfunction such as orthostatic hypotension and urinary dysfunction; Olivo-ponto-cerebellar Atrophy (OPCA) which had prominent cerebellar features such as incoordination; and Striatal-nigral degeneration (SND) which had features that appeared more like Parkinson's disease but often with prominent speech disturbance. These were reclassed as the MSA's due to their common features. Often there is overlap clinically.
In general, MSA occurs in middle-aged individuals (6th decade of life). It affects autonomic function early in its course (blood pressure, sexual function and urinary function, for instance). These are things that are typically affected later in typical PD. MSA tends to respond less briskly to parkinson medications like levodopa (Stalevo, Sinemet). There may be some improvement with medications in some patients, but this is a progressive disorder that typically has a course that is more rapid than PD. Treatment is symptomatic, and each symptom needs to be addressed separately.
There is no specific diagnostic test to verify the diagnosis. Autonomic function testing and tilt-table testing are usually done. MRI obviously cannot be done in someone with a pacemaker. CT scan is of very limited value except to rule out another cause. Other causes might include vascular parkinsonism with autonomic dysfunction such as would be caused from diabetes. Fluorodopa PET scanning is not felt to sufficiently differentiate between MSA and PD. Other types of PET scans (FDG for instance) may show more differentiation because it is felt that in some MSA patients larger areas of the brain are involved. In many patients, following them over time with serial examinations is what clarifies the diagnosis.
Karen M Thomas, DO
College of Medicine
The Ohio State University