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Marchiafava-Bignami disease

MedGen UID:
65955
Concept ID:
C0238265
Disease or Syndrome
Synonyms: Acute Marchiafava-Bignami disease; Chronic Marchiafava-Bignami syndrome
SNOMED CT: Marchiafava-Bignami disease (386766007); Marchiafava disease (386766007); Central demyelination of corpus callosum (386766007)
 
Monarch Initiative: MONDO:0016370
Orphanet: ORPHA221074

Definition

A rare neurologic disease most prominently characterized by progressive demyelination and necrosis of the corpus callosum. It is in most cases associated with chronic alcoholism and malnutrition. Speed of onset and clinical presentation are very variable with a range of possible symptoms, including dementia, seizures, gait abnormalities, dysarthria, aphasia, athetosis, as well as stupor and coma. [from ORDO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVMarchiafava-Bignami disease
Follow this link to review classifications for Marchiafava-Bignami disease in Orphanet.

Professional guidelines

PubMed

Hillbom M, Saloheimo P, Fujioka S, Wszolek ZK, Juvela S, Leone MA
J Neurol Neurosurg Psychiatry 2014 Feb;85(2):168-73. Epub 2013 Aug 26 doi: 10.1136/jnnp-2013-305979. PMID: 23978380Free PMC Article

Recent clinical studies

Etiology

Liu C, Wang H, Xie B, Tian S, Ding Y
BMC Neurol 2024 Oct 14;24(1):389. doi: 10.1186/s12883-024-03901-y. PMID: 39402444Free PMC Article
Li W, Ran C, Ma J
Acta Radiol 2021 Jul;62(7):904-908. Epub 2020 Jul 27 doi: 10.1177/0284185120943040. PMID: 32718180
Dong X, Bai C, Nao J
Medicine (Baltimore) 2018 Feb;97(5):e9626. doi: 10.1097/MD.0000000000009626. PMID: 29384842Free PMC Article
Logan C, Asadi H, Kok HK, Looby ST, Brennan P, O'Hare A, Thornton J
J Med Imaging Radiat Oncol 2017 Aug;61(4):435-440. Epub 2016 Dec 16 doi: 10.1111/1754-9485.12572. PMID: 27987266
Raina S, Mahesh DM, Mahajan J, Kaushal SS, Gupta D, Dhiman DS
J Assoc Physicians India 2008 Aug;56:633-5. PMID: 19051711

Diagnosis

Haralur Y, Mechtler LL
Neurol Clin 2020 Feb;38(1):149-170. Epub 2019 Oct 24 doi: 10.1016/j.ncl.2019.09.002. PMID: 31761056
Logan C, Asadi H, Kok HK, Looby ST, Brennan P, O'Hare A, Thornton J
J Med Imaging Radiat Oncol 2017 Aug;61(4):435-440. Epub 2016 Dec 16 doi: 10.1111/1754-9485.12572. PMID: 27987266
Hillbom M, Saloheimo P, Fujioka S, Wszolek ZK, Juvela S, Leone MA
J Neurol Neurosurg Psychiatry 2014 Feb;85(2):168-73. Epub 2013 Aug 26 doi: 10.1136/jnnp-2013-305979. PMID: 23978380Free PMC Article
Koeppen AH, Barron KD
Neurology 1978 Mar;28(3):290-4. doi: 10.1212/wnl.28.3.290. PMID: 415261
Hathaway BM, Ch'ien L
South Med J 1971 May;64(5):602-6. doi: 10.1097/00007611-197105000-00021. PMID: 5573080

Therapy

Kinsley S, Giovane RA, Daly S, Shulman D
BMJ Case Rep 2020 Dec 10;13(12) doi: 10.1136/bcr-2020-238187. PMID: 33303506Free PMC Article
Dong X, Bai C, Nao J
Medicine (Baltimore) 2018 Feb;97(5):e9626. doi: 10.1097/MD.0000000000009626. PMID: 29384842Free PMC Article
Kim TE, Lee EJ, Young JB, Shin DJ, Kim JH
Semin Ultrasound CT MR 2014 Apr;35(2):85-96. doi: 10.1053/j.sult.2013.09.004. PMID: 24745886
Hillbom M, Saloheimo P, Fujioka S, Wszolek ZK, Juvela S, Leone MA
J Neurol Neurosurg Psychiatry 2014 Feb;85(2):168-73. Epub 2013 Aug 26 doi: 10.1136/jnnp-2013-305979. PMID: 23978380Free PMC Article
Kohler CG, Ances BM, Coleman AR, Ragland JD, Lazarev M, Gur RC
Neuropsychiatry Neuropsychol Behav Neurol 2000 Jan;13(1):67-76. PMID: 10645739

Prognosis

Li W, Ran C, Ma J
Acta Radiol 2021 Jul;62(7):904-908. Epub 2020 Jul 27 doi: 10.1177/0284185120943040. PMID: 32718180
Zhao P, Zhang H, Zhang Y, Sun L
Clin Lab 2018 Jun 1;64(6):1055-1059. doi: 10.7754/Clin.Lab.2018.171220. PMID: 29945327
Dong X, Bai C, Nao J
Medicine (Baltimore) 2018 Feb;97(5):e9626. doi: 10.1097/MD.0000000000009626. PMID: 29384842Free PMC Article
Hillbom M, Saloheimo P, Fujioka S, Wszolek ZK, Juvela S, Leone MA
J Neurol Neurosurg Psychiatry 2014 Feb;85(2):168-73. Epub 2013 Aug 26 doi: 10.1136/jnnp-2013-305979. PMID: 23978380Free PMC Article
Heinrich A, Runge U, Khaw AV
J Neurol 2004 Sep;251(9):1050-9. doi: 10.1007/s00415-004-0566-1. PMID: 15372245

Clinical prediction guides

Zhang YL, Ran C, Xu C, Li W
Sci Rep 2023 Oct 28;13(1):18516. doi: 10.1038/s41598-023-45431-6. PMID: 37898646Free PMC Article
Dong X, Bai C, Nao J
Medicine (Baltimore) 2018 Feb;97(5):e9626. doi: 10.1097/MD.0000000000009626. PMID: 29384842Free PMC Article
Hillbom M, Saloheimo P, Fujioka S, Wszolek ZK, Juvela S, Leone MA
J Neurol Neurosurg Psychiatry 2014 Feb;85(2):168-73. Epub 2013 Aug 26 doi: 10.1136/jnnp-2013-305979. PMID: 23978380Free PMC Article
Namekawa M, Nakamura Y, Nakano I
Intern Med 2013;52(7):811-3. Epub 2013 Apr 1 doi: 10.2169/internalmedicine.52.9336. PMID: 23545681
Raina S, Mahesh DM, Mahajan J, Kaushal SS, Gupta D, Dhiman DS
J Assoc Physicians India 2008 Aug;56:633-5. PMID: 19051711

Recent systematic reviews

Deví-Bastida J, Xifré-Passols M, Oviedo-Penuela LM, Teresa Abellán-Vidal M, Dolores López-Villegas M
Rev Colomb Psiquiatr (Engl Ed) 2024 Jul-Sep;53(3):385-395. Epub 2024 Oct 28 doi: 10.1016/j.rcpeng.2024.10.004. PMID: 39472222

Supplemental Content

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