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Demyelinating disease

MedGen UID:: 4189
•Concept ID:: C0011303
•: Disease or Syndrome

Synonyms:	Demyelinating Disease; Demyelinating Diseases; Demyelinating Disorder; Demyelinating Disorders

Monarch Initiative:	MONDO:0002562

Definition

A broad group of disorders that affect the myelin sheaths that cover the neurons. Myelin sheathes cover neuronal axons in the central and peripheral nervous system and function to increase travelling impulse speeds. Disruption of this sheath impairs neuronal transmission and can result in disorders such as multiple sclerosis and Guillain-Barre syndrome, among others. [from NCI]

Term Hierarchy

GTR
MeSH

CClinical test, RResearch test, OOMIM, GGeneReviews, VClinVar

CROGVDemyelinating disease

Pathological Conditions, Signs and Symptoms
- Pathological process
  - Disease
    - Non-Neoplastic Disorder
      - Non-Neoplastic Disorder by Special Category
        Degenerative disorder
        Demyelinating disease
        Central pontine myelinolysis
        CNS demyelinating autoimmune disease
        Acute disseminated encephalomyelitis
        Acute disseminated encephalomyelitis with anti-MOG antibodies
        Acute disseminated encephalomyelitis without anti-MOG antibodies
        Acute hemorrhagic leukoencephalitis
        Multiple sclerosis
        Chronic progressive multiple sclerosis
        Relapsing remitting multiple sclerosis
        Neuromyelitis optica
        Neuromyelitis optica spectrum disorder with anti-AQP4 antibodies
        Neuromyelitis optica spectrum disorder with anti-MOG antibodies
        Neuromyelitis optica spectrum disorder without anti-MOG and without anti-AQP4 antibodies
        Schilder disease
        Transverse myelitis
        Demyelinating disease of central nervous system
        Demyelinating Encephalopathy
        Demyelinating peripheral neuropathy
        Acute demyelinating polyneuropathy
        Chronic inflammatory demyelinating polyradiculoneuropathy
        Lewis-Sumner syndrome
        Demyelinating sensory neuropathy
        Hereditary Central Nervous System Demyelinating Diseases
        Adrenoleukodystrophy
        Adrenomyeloneuropathy
        Cerebral Adrenoleukodystrophy
        X-linked cerebral adrenoleukodystrophy
        Alexander disease
        Alexander disease type I
        Alexander disease type II
        Galactosylceramide beta-galactosidase deficiency
        Adult Krabbe disease
        Infantile Krabbe disease
        Late-infantile/juvenile Krabbe disease
        Metachromatic leukodystrophy
        Metachromatic leukodystrophy, adult type
        Metachromatic leukodystrophy, juvenile type
        Metachromatic leukodystrophy, late infantile form
        Pelizaeus-Merzbacher disease
        Null syndrome
        Pelizaeus-Merzbacher disease in female carriers
        Pelizaeus-Merzbacher disease, classic form
        Pelizaeus-Merzbacher disease, connatal form
        Pelizaeus-Merzbacher disease, transitional form
        Spongy degeneration of central nervous system
        Mild Canavan disease
        Severe Canavan disease
        Marchiafava-Bignami disease
        Polyradiculoneuropathy
        Cauda equina syndrome
        Cauda equina syndrome with neurogenic bladder
        Guillain-Barre syndrome
        Acute inflammatory demyelinating polyradiculoneuropathy
        Acute motor and sensory axonal neuropathy
        Acute motor axonal neuropathy
        Miller Fisher syndrome
        Polyradiculopathy
        Progressive multifocal leukoencephalopathy
        Recurrent painful ophthalmoplegic neuropathy
        Subacute combined degeneration of spinal cord

Professional guidelines

PubMed

Assessment and Management of Acute Disseminated Encephalomyelitis (ADEM) in the Pediatric Patient.

Wang CX
Paediatr Drugs 2021 May;23(3):213-221. Epub 2021 Apr 8 doi: 10.1007/s40272-021-00441-7. PMID: 33830467 Free PMC Article

Peripheral Neuropathy: Evaluation and Differential Diagnosis.

Castelli G, Desai KM, Cantone RE
Am Fam Physician 2020 Dec 15;102(12):732-739. PMID: 33320513

An update on immunopathogenesis, diagnosis, and treatment of multiple sclerosis.

Garg N, Smith TW
Brain Behav 2015 Sep;5(9):e00362. Epub 2015 Aug 3 doi: 10.1002/brb3.362. PMID: 26445701 Free PMC Article

See all (177)

Recent clinical studies

Etiology

Ineffective control of Epstein-Barr-virus-induced autoimmunity increases the risk for multiple sclerosis.

Vietzen H, Berger SM, Kühner LM, Furlano PL, Bsteh G, Berger T, Rommer P, Puchhammer-Stöckl E
Cell 2023 Dec 21;186(26):5705-5718.e13. Epub 2023 Dec 12 doi: 10.1016/j.cell.2023.11.015. PMID: 38091993

Autoimmune Neurologic Emergencies.

Raibagkar P, Ramineni A
Neurol Clin 2021 May;39(2):589-614. Epub 2021 Mar 31 doi: 10.1016/j.ncl.2021.01.006. PMID: 33896534

Tumefactive demyelinating lesions: A comprehensive review.

Algahtani H, Shirah B, Alassiri A
Mult Scler Relat Disord 2017 May;14:72-79. Epub 2017 Apr 9 doi: 10.1016/j.msard.2017.04.003. PMID: 28619436

Asymptomatic MS.

Siva A
Clin Neurol Neurosurg 2013 Dec;115 Suppl 1:S1-5. doi: 10.1016/j.clineuro.2013.09.012. PMID: 24321147

Acute disseminated encephalomyelitis.

Marchioni E, Tavazzi E, Minoli L, Del Bue S, Ferrante P, Piccolo G, Bastianello S, Ravaglia S
Neurol Sci 2008 Sep;29 Suppl 2:S286-8. doi: 10.1007/s10072-008-0966-6. PMID: 18690521

See all (1027)

Diagnosis

To be, or not to be… Guillain-Barré Syndrome.

Florian IA, Lupan I, Sur L, Samasca G, Timiș TL
Autoimmun Rev 2021 Dec;20(12):102983. Epub 2021 Oct 28 doi: 10.1016/j.autrev.2021.102983. PMID: 34718164

Autoimmune Neurologic Emergencies.

Raibagkar P, Ramineni A
Neurol Clin 2021 May;39(2):589-614. Epub 2021 Mar 31 doi: 10.1016/j.ncl.2021.01.006. PMID: 33896534

Update on Pediatric Optic Neuritis.

Gise RA, Heidary G
Curr Neurol Neurosci Rep 2020 Mar 3;20(3):4. doi: 10.1007/s11910-020-1024-x. PMID: 32124097

Tumefactive demyelinating lesions: A comprehensive review.

Algahtani H, Shirah B, Alassiri A
Mult Scler Relat Disord 2017 May;14:72-79. Epub 2017 Apr 9 doi: 10.1016/j.msard.2017.04.003. PMID: 28619436

An update on immunopathogenesis, diagnosis, and treatment of multiple sclerosis.

Garg N, Smith TW
Brain Behav 2015 Sep;5(9):e00362. Epub 2015 Aug 3 doi: 10.1002/brb3.362. PMID: 26445701 Free PMC Article

See all (1427)

Therapy

The Effect of Peripheral Immune Cell Counts on the Risk of Multiple Sclerosis: A Mendelian Randomization Study.

He D, Liu L, Shen D, Zou P, Cui L
Front Immunol 2022;13:867693. Epub 2022 May 10 doi: 10.3389/fimmu.2022.867693. PMID: 35619713 Free PMC Article

Creatine and multiple sclerosis.

Ostojic SM
Nutr Neurosci 2022 May;25(5):912-919. Epub 2020 Sep 11 doi: 10.1080/1028415X.2020.1819108. PMID: 32912105

Advances in Treatment of Progressive Multifocal Leukoencephalopathy.

Bernard-Valnet R, Koralnik IJ, Du Pasquier R
Ann Neurol 2021 Dec;90(6):865-873. Epub 2021 Sep 7 doi: 10.1002/ana.26198. PMID: 34405435 Free PMC Article

Thyroid Hormone Analogues: An Update.

Zucchi R
Thyroid 2020 Aug;30(8):1099-1105. Epub 2020 Apr 7 doi: 10.1089/thy.2020.0071. PMID: 32098589 Free PMC Article

Tumefactive demyelinating lesions: A comprehensive review.

Algahtani H, Shirah B, Alassiri A
Mult Scler Relat Disord 2017 May;14:72-79. Epub 2017 Apr 9 doi: 10.1016/j.msard.2017.04.003. PMID: 28619436

See all (878)

Prognosis

Creatine and multiple sclerosis.

Ostojic SM
Nutr Neurosci 2022 May;25(5):912-919. Epub 2020 Sep 11 doi: 10.1080/1028415X.2020.1819108. PMID: 32912105

Advances in Treatment of Progressive Multifocal Leukoencephalopathy.

Bernard-Valnet R, Koralnik IJ, Du Pasquier R
Ann Neurol 2021 Dec;90(6):865-873. Epub 2021 Sep 7 doi: 10.1002/ana.26198. PMID: 34405435 Free PMC Article

Update on Pediatric Optic Neuritis.

Gise RA, Heidary G
Curr Neurol Neurosci Rep 2020 Mar 3;20(3):4. doi: 10.1007/s11910-020-1024-x. PMID: 32124097

Multiple sclerosis - etiology and diagnostic potential.

Kamińska J, Koper OM, Piechal K, Kemona H
Postepy Hig Med Dosw (Online) 2017 Jun 30;71(0):551-563. doi: 10.5604/01.3001.0010.3836. PMID: 28665284

The spectrum of neuromyelitis optica.

Wingerchuk DM, Lennon VA, Lucchinetti CF, Pittock SJ, Weinshenker BG
Lancet Neurol 2007 Sep;6(9):805-15. doi: 10.1016/S1474-4422(07)70216-8. PMID: 17706564

See all (878)

Clinical prediction guides

Polygenic risk score association with multiple sclerosis susceptibility and phenotype in Europeans.

Shams H, Shao X, Santaniello A, Kirkish G, Harroud A, Ma Q, Isobe N; University of California San Francisco MS-EPIC Team, Schaefer CA, McCauley JL, Cree BAC, Didonna A, Baranzini SE, Patsopoulos NA, Hauser SL, Barcellos LF, Henry RG, Oksenberg JR
Brain 2023 Feb 13;146(2):645-656. doi: 10.1093/brain/awac092. PMID: 35253861 Free PMC Article

Long-term safety of satralizumab in neuromyelitis optica spectrum disorder (NMOSD) from SAkuraSky and SAkuraStar.

Yamamura T, Weinshenker B, Yeaman MR, De Seze J, Patti F, Lobo P, von Büdingen HC, Kou X, Weber K, Greenberg B
Mult Scler Relat Disord 2022 Oct;66:104025. Epub 2022 Jul 5 doi: 10.1016/j.msard.2022.104025. PMID: 36007339

Targeted Complement Inhibition at Synapses Prevents Microglial Synaptic Engulfment and Synapse Loss in Demyelinating Disease.

Werneburg S, Jung J, Kunjamma RB, Ha SK, Luciano NJ, Willis CM, Gao G, Biscola NP, Havton LA, Crocker SJ, Popko B, Reich DS, Schafer DP
Immunity 2020 Jan 14;52(1):167-182.e7. Epub 2019 Dec 26 doi: 10.1016/j.immuni.2019.12.004. PMID: 31883839 Free PMC Article

The radiologically isolated syndrome.

Lebrun C
Rev Neurol (Paris) 2015 Oct;171(10):698-706. Epub 2015 Sep 26 doi: 10.1016/j.neurol.2015.05.001. PMID: 26410363

Natalizumab.

Berger JR
Drugs Today (Barc) 2006 Oct;42(10):639-55. doi: 10.1358/dot.2006.42.10.1042190. PMID: 17136224

See all (798)

Recent systematic reviews

Alemtuzumab for multiple sclerosis.

Riera R, Torloni MR, Martimbianco ALC, Pacheco RL
Cochrane Database Syst Rev 2023 Jun 5;6(6):CD011203. doi: 10.1002/14651858.CD011203.pub3. PMID: 37272540 Free PMC Article

Worldwide prevalence of neuromyelitis optica spectrum disorder (NMOSD) and neuromyelitis optica (NMO): a systematic review and meta-analysis.

Bagherieh S, Afshari-Safavi A, Vaheb S, Kiani M, Ghaffary EM, Barzegar M, Shaygannejad V, Zabeti A, Mirmosayyeb O
Neurol Sci 2023 Jun;44(6):1905-1915. Epub 2023 Feb 6 doi: 10.1007/s10072-023-06617-y. PMID: 36745300

Sexual dysfunction therapeutic approaches in patients with multiple sclerosis: a systematic review.

Giannopapas V, Kitsos D, Tsogka A, Tzartos JS, Paraskevas G, Tsivgoulis G, Voumvourakis K, Giannopoulos S, Bakalidou D
Neurol Sci 2023 Mar;44(3):873-880. Epub 2022 Dec 31 doi: 10.1007/s10072-022-06572-0. PMID: 36585597 Free PMC Article

Prevalence and burden of multiple sclerosis-related fatigue: a systematic literature review.

Oliva Ramirez A, Keenan A, Kalau O, Worthington E, Cohen L, Singh S
BMC Neurol 2021 Dec 2;21(1):468. doi: 10.1186/s12883-021-02396-1. PMID: 34856949 Free PMC Article

Dietary interventions for multiple sclerosis-related outcomes.

Parks NE, Jackson-Tarlton CS, Vacchi L, Merdad R, Johnston BC
Cochrane Database Syst Rev 2020 May 19;5(5):CD004192. doi: 10.1002/14651858.CD004192.pub4. PMID: 32428983 Free PMC Article

See all (42)

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Demyelinating disease

Definition

Term Hierarchy

Professional guidelines

PubMed

Recent clinical studies

Etiology

Diagnosis

Therapy

Prognosis

Clinical prediction guides

Recent systematic reviews

Supplemental Content

Table of contents

Clinical resources

Practice guidelines

Consumer resources

Reviews

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