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

Most deaths occur in seven to ten years from the onset of the symptoms mainly from respiratory infections, sepsis, etc.

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.