multiple-system atrophy

multiple-system atrophy

An uncommon (5/105) neurodegenerative disorder (”tauopathy”) with incomplete penetrance, characterised by:
(1) Parkinsonism,
(2) Ataxia, and
(3) appearance in those aged 55 to 65.

Clinical findings
Parkinsonism, cerebellar dysfunction (e.g, ataxia), incoordination, autonomic dysfunction (e.g, orthostatic hypotension), urinary incontinence, impotence, constipation, dry mouth, temperature dysregulation.

Management
Symptomatic and supportive.

Prognosis
Slow, inexorable deterioration; death commonly within 10 years.

multiple-system atrophy

Neurology A heterogenous group of neurologic syndromes– corticobasal ganglionic degeneration, olivopontine cerebellar atrophy, progressive supranuclear palsy, Shy-Drager syndrome, striatonigral degeneration–which share features caused by lesions of interrelated groups of neurons

mul·ti·ple-sys·tem at·ro·phy

(mŭl'ti-pĕl sis'tĕm at'rŏ-fē)
Nonhereditary, neurodegenerative disease of unknown cause, characterized clinically by the development of parkinsonism, ataxia, autonomic failure, or pyramidal tract signs, in various combinations. Pathologically there are nerve cell loss, gliosis, and the accumulation of abnormal tubular structures in the cytoplasm and nucleus of oligodendrocytes and neurons in the basal ganglion, cerebellum,and intermediolateral columns of the spinal cord; can present as predominantly parkinsonism, as predominantly ataxia, or as a combination of parkinsonism, ataxia, and autonomic failure; it is a relatively rapidly progressive and fatal disorder.

mul·ti·ple-sys·tem at·ro·phy

(MSA) (mŭl'ti-pĕl sis'tĕm at'rŏ-fē)
Nonhereditary, neurodegenerative disease of unknown cause, characterized clinically by the development of parkinsonism, ataxia, autonomic failure, or pyramidal track signs, in various combinations.
References in periodicals archive ?
NOH is a rare, chronic and often debilitating drop in blood pressure upon standing that is associated with Parkinson's disease, multiple-system atrophy, and pure autonomic failure.
They found that "among [the 170] neurodegenerative disorders associated with RBD, 160 (94%) were synucleinopathies." Among them were 136 patients with Lewy bodies and 19 with multiple-system atrophy The remaining few had findings consistent with Alzheimer's disease or other nonsynucleinopathies.
Early or Predominant Neurologic Sign Suggested Diagnoses Tremor Parkinson's disease Gait disorder Multiple-system atrophy, progressive supranuclear palsy (PSP) Dementia Alzheimer's disease, multi-infarct state, normal pressure hydrocephalus (NPH) Autonomic instability Murinary Multiple-system atrophy urgency, constipation, loss (Shy-Drager syndrome) of libido) Visual complaints (diplopia, PSP, multiple-system atrophy ophthalmoparesis) Ataxia Olivopontocerebellar atrophy (OPCA), multiple-system atrophy Apraxia NPH, senile gait apraxia Peripheral neuropathy OPCA, multiple-system atrophy, spinocerebellar-nigral degeneration Pyramidal tract signs Multi-infarct state, multiple-system atrophy, cervical myelopath Adapted, with permission, from Stern MB, Hurtig HI, eds.

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