Disease Information for Shy-Drager syndrome: Definition

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)-------------------------------------------.

Multiple System Atrophy; MSA; Progressive Autonomic Failure with Multiple System Atrophy; Shy-Drager Syndrome; (SDS); Sporadic OPCA; Sporadic Olivopontocerebellar Atrophy; Striatonigral Degeneration (SND); Multiple system atrophy (MSA) is a rare progressive neurological disorder characterized by a varying combination of symptoms; Affected individuals may experience symptoms similar to those found in Parkinson’s disease (parkinsonism); cerebellar signs such as progressive impairment of the ability to coordinate voluntary movements (cerebellar ataxia); and impaired functioning of the portion of the nervous system (autonomic nervous system) that regulates certain involuntary body functions (autonomic failure) such as heart rate, blood pressure, sweating, and bowel and bladder control; The exact cause of multiple system atrophy is unknown; The term multiple system atrophy has generated significant controversy and confusion in the medical literature; The term now encompasses three conditions once thought to be separate disorders, specifically Shy-Drager syndrome, striatonigral degeneration, and sporadic olivopontocerebellar atrophy; Additionally, there is a hereditary form of olivopontocerebellar atrophy that is not part of the multiple system atrophy spectrum; [NORD]

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