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Shy Drager Syndrome

 

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  Shy Drager Syndrome Review

 

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: This is the reason why this syndrome was first described as a Parkinsonism or related to Parkinson Plus

  • Orthostatic hypotension: The first and most important sign of autonomic nervous system failure. An important reflex that rises blood pressure as a response to postural changes is lost.  Blood is forced towards the legs by gravity, but this reflex ensures that blood flow to the brain is conserved even with postural changes by blood vessel constriction.

  • Erectile dysfunction: Erection is directly related to the parasympathetic system (part of the autonomic nervous system)

  • Fecal and urinary Incontinence

  • Alterations of muscle tone (stiffness): This is the other sign that made this syndrome parkinson like

  • Anhidrosis: This is the loss of function of the sweat glands secondary to the lack of activation by the autonomic nervous system.

  • Progressive dementia: Secondary to the degenerative changes that occur in the central nervous system that will be explained later.

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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.

Case Description (Real Case)

A 50 year old man is brought to the  emergency room, clear signs of prostration, with infected pressure wounds on his lower back, a septic look on his face.
After a quick examination there were signs of spasticity, he could not move his limbs. The only movement that could be discerned was the tremor of his hands and head. At first glace he looked like a stroke patient or a patient suffering from Parkinson in its late stages.

A complete clinical assessment showed the following:


BP 100/60mmHg (when brought to a sitting position with his legs hanging, BP dropped more than 30 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 marmoreal skin, infected pressure wound on his lower back, typical anaerobic bacteria smell came from the wound.
His face wore an inexpressive septic look.
On his cardiac and respiratory examination rales could be heard on both lungs, probably due to frequent aspiration of the content of the mouth (aspiration pneumonia).
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.
A Parkinson like syndrome was diagnosed, and then his signs were cross-referenced on the net. The association of orthostatic hypotension and parkinson like symptoms was the best description. Some rare syndromes that could be associated with this symptoms: Shy Drager Syndrome (SDS) and Multiple System Atrophy (MSA) were the best candidates for his disease.
Treatment with antibiotics improved patient state but later that month he died from a massive aspiration of gastric content to his lungs.
The autopsy revealed the typical histological findings of Shy Drager Syndrome.

Other Shy Drager Syndrome Resources

Shy Drager Syndrome No Longer Exists

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.

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By Francisco Santander.

M.D.  Internal Medicine

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