Question Title: Shy Drager

Forum: Neurology Forum
Topic: Shy Drager

Posted by CCF Neuro MD on July 13, 1997 at 12:24:12:

In Reply to: Shy Drager posted by Joan on July 04, 1997 at 20:51:06:

: What is Shy Drager? Is it a form of Parkinson's disease? If so, how does it differ from Parkinson's?

I have little to add to what Marie wrote. To have some understanding of this area of neurology, one needs to remember and define various terms: Parkinsonism, Parkinson's disease (PD), "Parkinson's plus", Multiple system atrophy (MSA), Striatonigral degeneration, sporadic olivopontocerebellar atrophy (OPCA), and the Shy-Drager syndrome. Parkinsonism has a clinical definition-the presence a varying combination of the cardinal motor signs of Parkinson's disease, namely, akinesia (slowness of movement), rigidity, tremor, and abnormalities of posture and gait. The majority of adult patients with this picture have PD, which is a specific degenerative disease defined by the presence of specific pathology in specific regions of the brain at autopsy. A minority of patients with parkinsonism, that superficially look like they have PD, have another degenerative disease instead. The more common possible mimics of PD are MSA, Progressive supranuclear palsy (PSP), and Cortical-basal ganglionic degeneration (CBGD). Each of these, again, is believed to be a specific disease with specific pathology (at autopsy) by the majority of experts.They can often be distinguished clinically from PD because they have some additional clinical findings ("Parkinson's plus"). Amongst these, MSA (which is the commonest) is a heterogenous disease. Some patients with MSA have prominent parkinsonian features, and striatonigral degeneration pathologically, while others have imbalance and incoordination (ataxia) clinically, and OPCA pathologically. A large proportion of patients with MSA have autonomic nervous system abnormalities such as a drop in blood pressure on standing, urinary retention/incontinence, impotence, constipation, dry mouth, and decreased sweating, in addition to the parkinsonian symptomatology. These are included under the term "Shy-Drager syndrome" (after the people who first described the condition in 1960). There are several "red flags" that a neurologist uses to help identify the few MSA/Shy-Drager cases from among the majority of PD cases. The two important ones are the autonomic abnormalities, and the lack of sustained responsiveness to levodopa/Sinemet.
It is generally believed that MSA has a worse prognosis than PD.
I hope this answers your question.
In case you (or your relative who has MSA) live in the Cleveland area, and wish a second opinion, I strongly recommend a consultion with Dr. R. Stanley Burns, who is a movement disorder specialist it the Cleveland Clinic. An appointment can be made by calling (216)444-5559, or (800)223-2273 and asking for the Neurology appointments desk.
This information is provided for general medical educational purposes only. Please consult your physician for diagnostic and treatment options of your specific medical condition.

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