Disease Information for Striatonigral degeneration

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Clinical Manifestations
Signs & Symptoms
Basal ganglion signs
Disease Progression
Course/Chronic disorder
Course/Chronic only
Parkinsonism plus Other CNS issues
Demographics & Risk Factors
Population Group
Aged Adult
Sex & Age Groups
Population/Adult Aged Only
Population/Adult/all
Associated Diseases & Rule outs
Rule Outs
Parkinsons disease
Associated Disease & Complications
Parkinsonism, secondary
Striatonigral degeneration
Disease Mechanism & Classification
Class
CLASS/Basal ganglia lesion/involvement/disorders (ex)
CLASS/Meninges/pia/arachnoid involvement (ex)
CLASS/Neurologic (category)
Pathophysiology
Pathophysiology/CNS degeneration
Pathophysiology/Neurologic degenerative disorder (ex)
Process
PROCESS/Reference organ/system (category)
PROCESS/Use/Age/atrophic disorder (category)
Definition

A sporadic neurodegenerative disease with onset in middle-age characterized clinically by Parkinsonian features (eg, MUSCLE RIGIDITY; HYPOKINESIA; stooped posture) and HYPOTENSION; This condition is considered a clinical variant of MULTIPLE SYSTEM ATROPHY; Pathologic features include a prominent loss of neurons in the zona compacta of the SUBSTANTIA NIGRA and PUTAMEN; (From Adams et al, Principles of Neurology, 6th ed, p1075-6)--------------------------------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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Harrison's Online (accessmedicine)
NEJM (The New England Journal of Medicine)
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