Multiple system atrophy

I mentioned to you before. A constellation of three entities; Shy-Drager, which in the older nomenclature was going by the term idiopathic orthostatic hypertension, or pure anatomic failure. Olivopontocerebellar atrophy, or PCA, striatal nigral degeneration. The clinical picture of these things varies of course. There’s all kinds of issues. Like an OPCA can start off with a little bit of appendicular dysmetria, truncal ataxia, not much to write home about. “Gee, I don’t know why you are ataxic. Please don’t drink so much.” They come back two years later, they are a little bit stiffer. You don’t know, they are still ataxic, maybe even more so. It’s a slow progressive course, not really good levodopa response. Highly variable. Striatal nigral degeneration is usually a little bit more aggressive, very “parky”, very rigid, not levodopa responsive, go south in a hurry, that kind of stuff. In a hurry meaning over a one to two years kind of story. The reason why all these things come under one rubric is because of these oligodendrial cytoplasmic inclusions in virtually all cases of multiple system atrophy, suggesting that this process of degeneration may originate in the myelin sheath of axons.

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