Multiple system atrophy
| Multiple system atrophy | |
|---|---|
 |
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| Alpha synuclein immunohistochemistry showing many glial inclusions | |
| Classification and external resources | |
| Specialty | neurology |
| ICD-10 | G90.3 |
| ICD-9-CM | 333.0 |
| OMIM | 146500 |
| DiseasesDB | 8441 |
| MedlinePlus | 000757 |
| eMedicine | neuro/671 |
| Patient UK | Multiple system atrophy |
| MeSH | D019578 |
Multiple system atrophy (MSA) is a degenerativeneurological disorder that depicts a group of disorders characterised by the neuronal degeneration mainly in the substantia nigra, striatum, autonomic nervous system and cerebellum. Following a report in 1964 of what was then called striatonigral degeneration, many patients were recognised in whom the changes of striatonigral and olivopontocerebellar degeneration were combined and who had symptoms and signs of cerebellar ataxia and parkinsonian manifestations. More than half of the patients with striatonigral degeneration have orthostatic hypotension, which proves at autopsy to be associated with loss of intermediolateral horn cells (origin of the presynaptic cholinergic sympathetic neurones) and of pigmented nuclei of the brainstem.
This combined parkinsonian and autonomic disorder is referred to as the Shy–Drager syndrome. In addition to orthostatic hypotension, other features of autonomic failure include impotence, loss of sweating, dry mouth and urinary retention and incontinence. Vocal cord palsy is an important and sometimes initial clinical manifestation of the disorder.
Both MRI and CT scanning frequently show atrophy of the cerebellum and pons in those with cerebellar features. The putamen is hypodense on T2-weighted MRI and may show an increased deposition of iron in Parkinsonian form. In cerebellar form, a "hot cross" sign has been emphasised; it reflects atrophy of the pontocereballar fibres that manifest in T2 signal intensity in atrophic pons.
The cause of MSA is unclear and no specific risk factors have been identified, although research indicates that a prion form of the alpha-synuclein protein may be the cause of the disease.
Approximately 55% of MSA cases occur in men, with typical age of onset in the late 50s to early 60s. MSA often presents with some of the same symptoms as Parkinson's disease. However, those with MSA generally show minimal if any response to the dopamine medications used for Parkinson's disease.
MSA is distinct from the more common syndrome multisystem proteinopathy. It should also not be confused with the two terms multiple organ dysfunction syndrome or multiple organ system dysfunction syndrome, which are the more modern and accurate terms for multiple organ failure or multiple organ system failure, which is an often-fatal complication of septic shock (due to severe sepsis, a systemic infection that has spread to the bloodstream) or other very severe illnesses or injuries.
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