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.
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.