loading...| Preferred Name |
multiple system atrophy |
|
| Synonyms |
Shy-Drager Syndrome Shy-McGee-Drager syndrome Shy-Drager syndrome orthostatic hypotension syndrome Multiple system atrophy (disorder) multisystem atrophy MSA multiple system atrophy susceptibility to multiple system atrophy 1 Shy-dragger syndrome (formerly) autonomic failure, Pure hypotension, orthostatic |
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| Definitions |
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. 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) is a neurodegenerative disorder characterized by autonomic failure (cardiovascular and/or urinary), parkinsonism, cerebellar impairment and corticospinal signs with a median survival of 6-9 years. |
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| ID |
http://www.ebi.ac.uk/efo/EFO_1001050 |
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| closeMatch | ||
| database_cross_reference |
MeSH:D012791 UMLS:C0393571 MedDRA:10064060 MeSH:D019578 SNOMEDCT:230297002 NCIt:C84909 MONDO:0007803 NCIT:C84909 GARD:7079 DOID:4752 Orphanet:102 MESH:D019578 NORD:1472 |
|
| 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. 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) is a neurodegenerative disorder characterized by autonomic failure (cardiovascular and/or urinary), parkinsonism, cerebellar impairment and corticospinal signs with a median survival of 6-9 years. |
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| disease arises from feature | ||
| exactMatch |
http://purl.obolibrary.org/obo/NCIT_C84909 http://purl.obolibrary.org/obo/Orphanet_102 http://identifiers.org/mesh/D019578 |
|
| gwas_trait |
true |
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| has_exact_synonym |
Shy-Drager Syndrome Shy-McGee-Drager syndrome Shy-Drager syndrome orthostatic hypotension syndrome Multiple system atrophy (disorder) multisystem atrophy MSA multiple system atrophy |
|
| has_related_synonym |
susceptibility to multiple system atrophy 1 Shy-dragger syndrome (formerly) autonomic failure, Pure hypotension, orthostatic |
|
| id |
EFO:1001050 |
|
| in_subset |
http://purl.obolibrary.org/obo/mondo#ordo_disease http://purl.obolibrary.org/obo/mondo#rare http://purl.obolibrary.org/obo/mondo#nord_rare |
|
| label |
multiple system atrophy |
|
| notation |
EFO:1001050 |
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| prefLabel |
multiple system atrophy |
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| term editor |
Sirarat Sarntivijai Gautier Koscielny |
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| excluded_subClassOf | ||
| subClassOf |
http://www.ebi.ac.uk/efo/EFO_0004280 |