Shy-Drager

[Mike Usman <MikeUsman@xxxxxxx>]

These questions came from two separate lists yesterday:

>Dear Barbara,
>I have a friend whose husband has been diagnosed with Parkinson's Disease
>and has also developed Shy Drager Syndrome.  Would you have any up to
>date information on this subject.
>Trudy Mackrory.

>I know you are busy, but what is Shy-Dreger syndrome...

Here is probably the most concise (medical) description I've come across:

"Shy-Drager syndrome is an illness presenting with features of autonomic
dysfunction including impotence, incontinence, and orthostatic hypotension.
 Anhidrosis, iris atrophy, and decreased tearing may also occur.  Several
years after onset of the autonomic dysfunction, an akinetic rigid form of
parkinsonian syndrome develops.  In some patients there may be associated cere
bellar, upper motor neuron, and/or lower motor neuron findings.  Dementia
rarely occurs.  Pathologic examination reveals cell loss and gliosis in the
pigmented brainstem nuclei, striatum, pontine structures, and the intermediola
teral cell column of the spinal cord. (4 references given.)"
[From: Dementia: A Clinical Approach, Second Edition.  Cummings & Benson,
eds. Butterworth-Heinemann Publishing]

In laymans terms, patients usually first have trouble with dropping blood
pressure and later develop a type of parkinsonism.  All of the 4 or 5 cases
I've seen have had dementia, but that could a selection bias (I run a
dementia program.)  What seems unclear is if the syndrome is a disease itself
or rather a syndrome that can be seen as part of several different diseases.

I hope this helps.

mike

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Mahmood (Mike) A. Usman, M.D.
Director, Memory and Movement Disorder Programs
Medical College of PA & Hahnemann University, Allegheny Campus
e-mail: mikeusman@xxxxxxx  usman@xxxxxxxxxxxxxxx
Phone: (412) 330-4005   Fax: (412) 330-4089
4 Allegheny Center      Pittsburgh, PA 15212 USA
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