Diseases » Shy-Drager Syndrome » Summary

What is Shy-Drager Syndrome?

Contents

What is Shy-Drager Syndrome?

Shy-Drager Syndrome: A condition which is characterized by a progressive disease of the brain and spinal cord affecting the autonomic nervous system.
Shy-Drager Syndrome: A progressive neurodegenerative condition of the central and autonomic nervous systems characterized by atrophy of the preganglionic lateral horn neurons of the thoracic spinal cord, which differentiates this condition from other forms of idiopathic orthostatic hypotension (HYPOTENSION, ORTHOSTATIC). This disease is generally considered a clinical variant of MULTIPLE SYSTEM ATROPHY. Affected individuals present in the fifth or sixth decade with orthostasis and bladder dysfunction; and later develop FECAL INCONTINENCE; anhidrosis; ATAXIA; IMPOTENCE; and alterations of tone suggestive of basal ganglia dysfunction. (From Adams et al., Principles of Neurology, 6th ed, p536)
Source - Diseases Database

Shy-Drager Syndrome is listed as a "rare disease" by the Office of Rare Diseases (ORD) of the National Institutes of Health (NIH). This means that Shy-Drager Syndrome, or a subtype of Shy-Drager Syndrome, affects less than 200,000 people in the US population.
Source - National Institutes of Health (NIH)

Shy-Drager Syndrome: Introduction

Types of Shy-Drager Syndrome:

Types of Shy-Drager Syndrome:

Olivopontocerebellar atrophy (OPCA)
MSA parkinsonian form (striatonigral degeneration) - similar to Parkinson's disease
MSA mixed cerebellar and parkinsonian form
more types...»

How serious is Shy-Drager Syndrome?

Prognosis of Shy-Drager Syndrome: Poor. Degenerative. Progressive. Death commonly within 7-10 years.
Complications of Shy-Drager Syndrome: see complications of Shy-Drager Syndrome
Prognosis of Shy-Drager Syndrome: Shy-Drager syndrome usually ends in the patient’s death by 7 to 10 years after diagnosis. Breathing problems such as aspiration, stridor (high-pitched breathing sounds due to airway obstruction), or cardiopulmonary arrest are common causes of death. (Source: excerpt from NINDS Shy-Drager Syndrome Information Page: NINDS)