Full Report

Format

Full Report
Summary (Text)
Summary (XML)

Send to:

Choose Destination

File
Clipboard
Collections

Autoimmune disorder of the nervous system

MedGen UID:: 155946
•Concept ID:: C0751871
•: Disease or Syndrome

Synonym:	Autoimmune disease of the nervous system

Monarch Initiative:	MONDO:0002977

Definition

A disorder characterized by the degeneration of the nervous system due to autoimmunity. Representative examples include multiple sclerosis, Guillain-Barre syndrome, and myasthenia gravis. [from NCI]

Term Hierarchy

GTR
MeSH

CClinical test, RResearch test, OOMIM, GGeneReviews, VClinVar

CROGVAutoimmune disorder of the nervous system

Pathological process
- Disease
  - Disorder by Site
    - Immune System and Related Disorders
      - Immune system disorder
        Autoimmune disease
        Autoimmune disorder of the nervous system
        Anti-NMDA receptor encephalitis
        Central nervous system lupus
        Central nervous system vasculitis
        Central nervous system AIDS arteritis
        Giant cell arteritis
        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
        Guillain-Barre syndrome
        Acute inflammatory demyelinating polyradiculoneuropathy
        Acute motor and sensory axonal neuropathy
        Acute motor axonal neuropathy
        Miller Fisher syndrome
        Myasthenia gravis
        Adult-onset myasthenia gravis
        Eaton-Lambert syndrome
        Juvenile myasthenia gravis
        Neonatal myasthenia gravis
        Transient neonatal myasthenia gravis
        Polyradiculoneuropathy
        Cauda equina syndrome
        Cauda equina syndrome with neurogenic bladder
        Chronic inflammatory demyelinating polyradiculoneuropathy
        Lewis-Sumner syndrome
        Polyradiculopathy
        Steroid-responsive encephalopathy associated with autoimmune thyroiditis
        Stiff-man syndrome
        Classic stiff person syndrome
        Focal stiff limb syndrome
        Progressive encephalomyelitis with rigidity and myoclonus
        Vogt-Koyanagi-Harada disease

Professional guidelines

PubMed

Diagnosis and Treatment of Multiple Sclerosis: A Review.

McGinley MP, Goldschmidt CH, Rae-Grant AD
JAMA 2021 Feb 23;325(8):765-779. doi: 10.1001/jama.2020.26858. PMID: 33620411

Update οn the diagnosis and management of systemic lupus erythematosus.

Fanouriakis A, Tziolos N, Bertsias G, Boumpas DT
Ann Rheum Dis 2021 Jan;80(1):14-25. Epub 2020 Oct 13 doi: 10.1136/annrheumdis-2020-218272. PMID: 33051219

Diagnosis of multiple sclerosis: 2017 revisions of the McDonald criteria.

Thompson AJ, Banwell BL, Barkhof F, Carroll WM, Coetzee T, Comi G, Correale J, Fazekas F, Filippi M, Freedman MS, Fujihara K, Galetta SL, Hartung HP, Kappos L, Lublin FD, Marrie RA, Miller AE, Miller DH, Montalban X, Mowry EM, Sorensen PS, Tintoré M, Traboulsee AL, Trojano M, Uitdehaag BMJ, Vukusic S, Waubant E, Weinshenker BG, Reingold SC, Cohen JA
Lancet Neurol 2018 Feb;17(2):162-173. Epub 2017 Dec 21 doi: 10.1016/S1474-4422(17)30470-2. PMID: 29275977

See all (1776)

Recent clinical studies

Etiology

Autonomic neuropathies.

Kaur D, Tiwana H, Stino A, Sandroni P
Muscle Nerve 2021 Jan;63(1):10-21. Epub 2020 Sep 14 doi: 10.1002/mus.27048. PMID: 32926436

Clinical approach to the diagnosis of autoimmune encephalitis in the pediatric patient.

Cellucci T, Van Mater H, Graus F, Muscal E, Gallentine W, Klein-Gitelman MS, Benseler SM, Frankovich J, Gorman MP, Van Haren K, Dalmau J, Dale RC
Neurol Neuroimmunol Neuroinflamm 2020 Mar;7(2) Epub 2020 Jan 17 doi: 10.1212/NXI.0000000000000663. PMID: 31953309 Free PMC Article

Autoimmune seizures and epilepsy.

Geis C, Planagumà J, Carreño M, Graus F, Dalmau J
J Clin Invest 2019 Mar 1;129(3):926-940. Epub 2019 Feb 4 doi: 10.1172/JCI125178. PMID: 30714986 Free PMC Article

Multiple sclerosis.

Cotsapas C, Mitrovic M, Hafler D
Handb Clin Neurol 2018;148:723-730. doi: 10.1016/B978-0-444-64076-5.00046-6. PMID: 29478610

Vaccination and Induction of Autoimmune Diseases.

Toussirot É, Bereau M
Inflamm Allergy Drug Targets 2015;14(2):94-8. doi: 10.2174/1871528114666160105113046. PMID: 26728772

See all (13270)

Diagnosis

Diagnostic criteria for autoimmune encephalitis: utility and pitfalls for antibody-negative disease.

Dalmau J, Graus F
Lancet Neurol 2023 Jun;22(6):529-540. doi: 10.1016/S1474-4422(23)00083-2. PMID: 37210100

Anti-MAG neuropathy: From biology to clinical management.

Steck AJ
J Neuroimmunol 2021 Dec 15;361:577725. Epub 2021 Sep 28 doi: 10.1016/j.jneuroim.2021.577725. PMID: 34610502

Neurological Syndromes Associated with Anti-GAD Antibodies.

Dade M, Berzero G, Izquierdo C, Giry M, Benazra M, Delattre JY, Psimaras D, Alentorn A
Int J Mol Sci 2020 May 24;21(10) doi: 10.3390/ijms21103701. PMID: 32456344 Free PMC Article

Autoimmune Epilepsy.

Husari KS, Dubey D
Neurotherapeutics 2019 Jul;16(3):685-702. doi: 10.1007/s13311-019-00750-3. PMID: 31240596 Free PMC Article

Autoimmune Glial Fibrillary Acidic Protein Astrocytopathy: A Review of the Literature.

Shan F, Long Y, Qiu W
Front Immunol 2018;9:2802. Epub 2018 Dec 5 doi: 10.3389/fimmu.2018.02802. PMID: 30568655 Free PMC Article

See all (17665)

Therapy

Lactate limits CNS autoimmunity by stabilizing HIF-1α in dendritic cells.

Sanmarco LM, Rone JM, Polonio CM, Fernandez Lahore G, Giovannoni F, Ferrara K, Gutierrez-Vazquez C, Li N, Sokolovska A, Plasencia A, Faust Akl C, Nanda P, Heck ES, Li Z, Lee HG, Chao CC, Rejano-Gordillo CM, Fonseca-Castro PH, Illouz T, Linnerbauer M, Kenison JE, Barilla RM, Farrenkopf D, Stevens NA, Piester G, Chung EN, Dailey L, Kuchroo VK, Hava D, Wheeler MA, Clish C, Nowarski R, Balsa E, Lora JM, Quintana FJ
Nature 2023 Aug;620(7975):881-889. Epub 2023 Aug 9 doi: 10.1038/s41586-023-06409-6. PMID: 37558878 Free PMC Article

Immunotherapy for Refractory Autoimmune Encephalitis.

Yang J, Liu X
Front Immunol 2021;12:790962. Epub 2021 Dec 16 doi: 10.3389/fimmu.2021.790962. PMID: 34975890 Free PMC Article

Anti-MAG neuropathy: From biology to clinical management.

Steck AJ
J Neuroimmunol 2021 Dec 15;361:577725. Epub 2021 Sep 28 doi: 10.1016/j.jneuroim.2021.577725. PMID: 34610502

Autonomic neuropathies.

Kaur D, Tiwana H, Stino A, Sandroni P
Muscle Nerve 2021 Jan;63(1):10-21. Epub 2020 Sep 14 doi: 10.1002/mus.27048. PMID: 32926436

Critical review: Typical and atypical optic neuritis.

Abel A, McClelland C, Lee MS
Surv Ophthalmol 2019 Nov-Dec;64(6):770-779. Epub 2019 Jun 20 doi: 10.1016/j.survophthal.2019.06.001. PMID: 31229520

See all (10391)

Prognosis

Neurological Syndromes Associated with Anti-GAD Antibodies.

Dade M, Berzero G, Izquierdo C, Giry M, Benazra M, Delattre JY, Psimaras D, Alentorn A
Int J Mol Sci 2020 May 24;21(10) doi: 10.3390/ijms21103701. PMID: 32456344 Free PMC Article

Autoimmune Epilepsy.

Husari KS, Dubey D
Neurotherapeutics 2019 Jul;16(3):685-702. doi: 10.1007/s13311-019-00750-3. PMID: 31240596 Free PMC Article

Immune-mediated Cerebellar Ataxias: Practical Guidelines and Therapeutic Challenges.

Mitoma H, Manto M, Hampe CS
Curr Neuropharmacol 2019;17(1):33-58. doi: 10.2174/1570159X16666180917105033. PMID: 30221603 Free PMC Article

Autoimmune Glial Fibrillary Acidic Protein Astrocytopathy: A Review of the Literature.

Shan F, Long Y, Qiu W
Front Immunol 2018;9:2802. Epub 2018 Dec 5 doi: 10.3389/fimmu.2018.02802. PMID: 30568655 Free PMC Article

GAD65 neurological autoimmunity.

McKeon A, Tracy JA
Muscle Nerve 2017 Jul;56(1):15-27. Epub 2017 Apr 11 doi: 10.1002/mus.25565. PMID: 28063151

See all (9068)

Clinical prediction guides

Neurological Syndromes Associated with Anti-GAD Antibodies.

Dade M, Berzero G, Izquierdo C, Giry M, Benazra M, Delattre JY, Psimaras D, Alentorn A
Int J Mol Sci 2020 May 24;21(10) doi: 10.3390/ijms21103701. PMID: 32456344 Free PMC Article

Autoimmune Epilepsy.

Husari KS, Dubey D
Neurotherapeutics 2019 Jul;16(3):685-702. doi: 10.1007/s13311-019-00750-3. PMID: 31240596 Free PMC Article

Autoimmune Glial Fibrillary Acidic Protein Astrocytopathy: A Review of the Literature.

Shan F, Long Y, Qiu W
Front Immunol 2018;9:2802. Epub 2018 Dec 5 doi: 10.3389/fimmu.2018.02802. PMID: 30568655 Free PMC Article

GAD65 neurological autoimmunity.

McKeon A, Tracy JA
Muscle Nerve 2017 Jul;56(1):15-27. Epub 2017 Apr 11 doi: 10.1002/mus.25565. PMID: 28063151

Vaccination and Induction of Autoimmune Diseases.

Toussirot É, Bereau M
Inflamm Allergy Drug Targets 2015;14(2):94-8. doi: 10.2174/1871528114666160105113046. PMID: 26728772

See all (10777)

Recent systematic reviews

Primary Sjögren syndrome-related peripheral neuropathy: A systematic review and meta-analysis.

Liampas A, Parperis K, Erotocritou MF, Nteveros A, Papadopoulou M, Moschovos C, Akil M, Coaccioli S, Hadjigeorgiou GM, Hadjivassiliou M, Zis P
Eur J Neurol 2023 Jan;30(1):255-265. Epub 2022 Sep 23 doi: 10.1111/ene.15555. PMID: 36086910 Free PMC Article

Triggers of Guillain-Barré Syndrome: Campylobacter jejuni Predominates.

Finsterer J
Int J Mol Sci 2022 Nov 17;23(22) doi: 10.3390/ijms232214222. PMID: 36430700 Free PMC Article

Low-Dose Naltrexone for Chronic Pain: Update and Systemic Review.

Kim PS, Fishman MA
Curr Pain Headache Rep 2020 Aug 26;24(10):64. doi: 10.1007/s11916-020-00898-0. PMID: 32845365

Efficacy of aquatic therapy for multiple sclerosis: a systematic review.

Corvillo I, Varela E, Armijo F, Alvarez-Badillo A, Armijo O, Maraver F
Eur J Phys Rehabil Med 2017 Dec;53(6):944-952. Epub 2017 Feb 17 doi: 10.23736/S1973-9087.17.04570-1. PMID: 28215060

Recommendation for a definition of acute symptomatic seizure.

Beghi E, Carpio A, Forsgren L, Hesdorffer DC, Malmgren K, Sander JW, Tomson T, Hauser WA
Epilepsia 2010 Apr;51(4):671-5. Epub 2009 Sep 3 doi: 10.1111/j.1528-1167.2009.02285.x. PMID: 19732133

See all (432)

MedGen

National Center for Biotechnology Information

Send to:

Autoimmune disorder of the nervous system

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

Related information

Recent activity