multiple-system atrophy

(redirected from Multisystem 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 ?
Diagnosing patients only on the basis of clinical findings is a challenge, and some patients have been misdiagnosed as multiple sclerosis, primary lateral sclerosis, amyotrophic lateral sclerosis, myasthenia gravis, multisystem atrophy spinocerebellar ataxia, and cervical myelopathy (8).
Parkinson's Disease, Parkinson Plus Syndromes like Progressive Supranuclear Palsy (PSP), Multisystem Atrophy Parkinson's type (MSA-P), Corticobasal Degeneration (CBD), Huntington's Chorea, Wilson's Disease, Hallervorden-Spatz Disease and Normal Pressure Hydrocephalus (NPH).
Olivopontocerebellar atrophy: MR diagnosis and relationship to multisystem atrophy.
Shy-Drager syndrome: multisystem atrophy with comorbid depression.
Pick disease is a typical 3R tauopathy; CBD, PSP, AGD, and multisystem atrophy with globular inclusions belong to 4R tauopathies; and diffuse neurofibrillary tangle dementia with calcifications, to 3R + 4R tauopathies.
In Alzheimer's, as well as in progressive supranuclear palsy and multisystem atrophy, you do have nice visualization of these fibers, and this is a very useful test for differentiating Lewy body disorders from these other diseases.
Abstract: Shy-Drager syndrome, one type of multisystem atrophy, is considered a Parkinson's-plus syndrome.
The false-positive diagnosis is generally due to vascular parkinsonism, essential tremor, drug-induced parkinsonism, or other neurodegenerative disorders that include parkinsonism as one of the clinical features, such as multisystem atrophy, progressive sopranuclear palsy, and dementia.