loading...| Preferred Name |
Marchiafava-Bignami Disease |
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| Synonyms |
acute Marchiafava-Bignami disease chronic Marchiafava-Bignami syndrome MBD Marchiafava-Bignami disease Marchiafava Bignami disease |
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| Definitions |
A neurodegenerative condition that is characterized by demyelination or necrosis of the CORPUS CALLOSUM. Symptoms include DEPRESSION; PARANOIA; DEMENTIA; SEIZURES; and ATAXIA which can progress to COMA and death in a few months. Marchiafava-Bignami syndrome is seen often in alcoholics but has been found in non-alcoholics as well. Marchiafava Bignami disease is defined by characteristic demyelination of the corpus callosum (erosion of the protective covering of nerve fibers joining the 2 hemispheres of the brain). The disease seems to most often affect severe and chronic alcoholics in their middle or late adult life. Early symptoms may include depression, paranoia, psychosis, or dementia. Seizures are common, and hemiparesis, aphasia, abnormal movements, and ataxia may sometimesprogress to coma and/or death. The cause of Marchiafava Bignami disease, including the potential role of nutritional deficiency, remains unknown. Improvement and recovery of some individuals has been reported. Treatment focuses on nutritional support and rehabilitation from alcoholism. |
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| ID |
http://www.ebi.ac.uk/efo/EFO_1001809 |
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| closeMatch | ||
| database_cross_reference |
MESH:D054319 SNOMEDCT:386766007 UMLS:C0238265 MeSH:D054319 SCTID:386766007 Orphanet:221074 icd11.foundation:1694687314 ORDO:Orphanet_221074 MedDRA:10026828 GARD:6971 ICD9:341.8 MONDO:0016370 |
|
| definition |
A neurodegenerative condition that is characterized by demyelination or necrosis of the CORPUS CALLOSUM. Symptoms include DEPRESSION; PARANOIA; DEMENTIA; SEIZURES; and ATAXIA which can progress to COMA and death in a few months. Marchiafava-Bignami syndrome is seen often in alcoholics but has been found in non-alcoholics as well. Marchiafava Bignami disease is defined by characteristic demyelination of the corpus callosum (erosion of the protective covering of nerve fibers joining the 2 hemispheres of the brain). The disease seems to most often affect severe and chronic alcoholics in their middle or late adult life. Early symptoms may include depression, paranoia, psychosis, or dementia. Seizures are common, and hemiparesis, aphasia, abnormal movements, and ataxia may sometimesprogress to coma and/or death. The cause of Marchiafava Bignami disease, including the potential role of nutritional deficiency, remains unknown. Improvement and recovery of some individuals has been reported. Treatment focuses on nutritional support and rehabilitation from alcoholism. |
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| exactMatch |
http://identifiers.org/snomedct/386766007 http://purl.obolibrary.org/obo/Orphanet_221074 |
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| has_exact_synonym |
Marchiafava-Bignami disease Marchiafava Bignami disease |
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| has_narrow_synonym |
acute Marchiafava-Bignami disease chronic Marchiafava-Bignami syndrome |
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| has_related_synonym |
MBD |
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| id |
EFO:1001809 |
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| in_subset |
http://purl.obolibrary.org/obo/mondo/mondo-base#rare http://purl.obolibrary.org/obo/mondo/mondo-base#otar http://purl.obolibrary.org/obo/mondo/mondo-base#gard_rare http://purl.obolibrary.org/obo/mondo/mondo-base#orphanet_rare http://purl.obolibrary.org/obo/mondo/mondo-base#nord_rare http://purl.obolibrary.org/obo/mondo/mondo-base#ordo_disease |
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| label |
Marchiafava-Bignami Disease |
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| notation |
EFO:1001809 |
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| preferred label |
Marchiafava-Bignami Disease |
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| prefLabel |
Marchiafava-Bignami Disease |
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| see also |
https://rarediseases.info.nih.gov/diseases/6971/marchiafava-bignami-disease |
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| term editor |
Sirarat Sarntivijai |
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| subClassOf |