Shy Drager Reviewed

Shy Drager Syndrome no longer exists

2006-01-30T10:44:00.000-08:00

According to the American Autonomic Society and the American Academy of Neurology, the syndrome described by Shy and Drager in 1962 now forms part a a bigger entity called Multiple System Atrophy. Review the source:

Consensus statement on the definition of orthostatic hypotension, pure autonomic failure, and multiple system atrophy. Neurology. 1996; 46:1470.

My Shy Drager Page

How I became aware of this rare disease

2006-01-29T19:09:00.000-08:00

I was on call when a woman entered my office and told me she had an emergency.
She brought her husband, a 50 year old man, postrated, with infected pressure wounds on his back, a septic look on his face.
I gave him a thorough look only to realize that he was spastic, he could not move his limbs. The only movement I could discerns was the tremor of his hands and head.
At first glace, I thought he was a stroke patient or a patient suffering from Parkinson.
A complete clinical assessment showed the following:
BP 100/60mmHg (when I tried to sit him up with his legs hanging, BP dropped more than 20 mmHg) HR 98bpm RR 29rpm Temp 36,5 (celsius)
He was lying on his back, had a diaper on with urine a fecal matter.
He looked dehydrated
Pale, dry skin, infected pressure wound on his back, an anaerobic bacteria smell came from the wound.
His face wore an inexpressive look.
On his cardiac and respiratory examination rales could be heard on both lungs.
Abdominal examination didn't show any abnormalities.
His limbs were shaky but when an active maneuver was performed, his muscles were stiff, he had areflexia.
Neurological examination showed dementia, many extrapiramidal symptoms such as tremor, and ataxia.
I diagnosed a Parkinson like syndrome and then reviewed the diagnostic possibilities on the net. I found some rare syndromes that could be associated with this symptoms: Shy Drager Syndrome (SDS) and Multiple System Atrophy (MSA). Later I foud out that they were related.
I started treatment with antibiotics and he could be discharged but later that month he died from a massive aspiration of gastric content to his lungs.
The autopsy revealed the typical histological findings of SDS.

Visit my page: Shy Drager Syndrome Review

Shy Drager Syndrome Review

2006-01-29T19:00:00.000-08:00

What is Shy dragger syndrome?
It is a disease first described by Dr. Milton Shy and Dr. Glen Drager in 1960
It is a rare disease of unknown etiology that causes a progressive degeneration of the central and autonomic nervous system.
It is characterized by the the following symptoms:
Parkinson like tremor
Orthostatic hypotension
Erectile dysfunction
Fecal and urinary Incontinence
Alterations of muscle tone (stiffness)
Anhidrosis
Progressive dementia
It was previously associated with Parkinson like syndromes, then reclassified with MSA (MULTIPLE SYSTEM ATROPHY) syndromes.
From a histological point of view, necropsies show basal ganglia degeneration, but the most commonly found sign is atrophy of the lateral horn neurons of the thoracic spinal cord.
From an epidemiological point of view, this disease is 2 to 3 times more common in men than in women, in the fifth or sixth decade of life. It is not reported to be hereditary.
Diagnostic can only be made post mortem by demonstrating the degenerative changes in the central and autonomic nervous system. However, it can be suspected after ruling out any other Parkinson like diseases and any other form of idiopathic orthostatic hypotension.
There is no known treatment up to the date this review was written.

Shy Drager Syndrome Review Article
(My Shy drager page where I got the material for this first post, the blog will get more content because it will also have personal comments on my experience with the disease, something I am not planning on adding to the web page.)