Neuromyelitis Optica (NMO)

Categories: Eye diseases, Immune diseases, Neuronal diseases, Rare diseases

Aliases & Classifications for Neuromyelitis Optica

MalaCards integrated aliases for Neuromyelitis Optica:

Name: Neuromyelitis Optica 12 52 25 53 58 36 54 43 15 37 17 71
Devic Syndrome 74 52 25
Devic Disease 52 25 58
Devic's Syndrome 12 53
Devic's Disease 12 25
Neuromyelitis Optica Spectrum Disorders 52
Neuromyelitis Optica Spectrum Disorder 52
Opticospinal Multiple Sclerosis 71
Devic's Neuromyelitis Optica 52
Devic Neuromyelitis Optica 25
Nmo Spectrum Disorder 52
Optic-Spinal Ms 25
Opticospinal Ms 25
Nmo 52


Orphanet epidemiological data:

neuromyelitis optica
Inheritance: Multigenic/multifactorial; Prevalence: 1-9/100000 (Europe); Age of onset: All ages; Age of death: any age;


Orphanet: 58  
Rare neurological diseases
Rare eye diseases

External Ids:

Disease Ontology 12 DOID:8869
KEGG 36 H01491
ICD9CM 34 341.0
MeSH 43 D009471
NCIt 49 C84934
SNOMED-CT 67 25044007
ICD10 32 G36.0
MESH via Orphanet 44 D009471
ICD10 via Orphanet 33 G36.0
UMLS via Orphanet 72 C0027873
Orphanet 58 ORPHA71211
UMLS 71 C0027873 C3711850

Summaries for Neuromyelitis Optica

Genetics Home Reference : 25 Neuromyelitis optica is an autoimmune disorder that affects the nerves of the eyes and the central nervous system, which includes the brain and spinal cord. Autoimmune disorders occur when the immune system malfunctions and attacks the body's own tissues and organs. In neuromyelitis optica, the autoimmune attack causes inflammation of the nerves, and the resulting damage leads to the signs and symptoms of the condition. Neuromyelitis optica is characterized by optic neuritis, which is inflammation of the nerve that carries information from the eye to the brain (optic nerve). Optic neuritis causes eye pain and vision loss, which can occur in one or both eyes. Neuromyelitis optica is also characterized by transverse myelitis, which is inflammation of the spinal cord. The inflammation associated with transverse myelitis damages the spinal cord, causing a lesion that often extends the length of three or more bones of the spine (vertebrae). In addition, myelin, which is the covering that protects nerves and promotes the efficient transmission of nerve impulses, can be damaged. Transverse myelitis causes weakness, numbness, and paralysis of the arms and legs. Other effects of spinal cord damage can include disturbances in sensations, loss of bladder and bowel control, uncontrollable hiccupping, and nausea. In addition, muscle weakness may make breathing difficult and can cause life-threatening respiratory failure in people with neuromyelitis optica. There are two forms of neuromyelitis optica, the relapsing form and the monophasic form. The relapsing form is most common. This form is characterized by recurrent episodes of optic neuritis and transverse myelitis. These episodes can be months or years apart, and there is usually partial recovery between episodes. However, most affected individuals eventually develop permanent muscle weakness and vision impairment that persist even between episodes. For unknown reasons, approximately nine times more women than men have the relapsing form. The monophasic form, which is less common, causes a single episode of neuromyelitis optica that can last several months. People with this form of the condition can also have lasting muscle weakness or paralysis and vision loss. This form affects men and women equally. The onset of either form of neuromyelitis optica can occur anytime from childhood to adulthood, although the condition most frequently begins in a person's forties. Approximately one-quarter of individuals with neuromyelitis optica have signs or symptoms of another autoimmune disorder such as myasthenia gravis, systemic lupus erythematosus, or Sjögren syndrome. Some scientists believe that a condition described in Japanese patients as optic-spinal multiple sclerosis (or opticospinal multiple sclerosis) that affects the nerves of the eyes and central nervous system is the same as neuromyelitis optica.

MalaCards based summary : Neuromyelitis Optica, also known as devic syndrome, is related to optic neuritis and transverse myelitis. An important gene associated with Neuromyelitis Optica is AQP4 (Aquaporin 4), and among its related pathways/superpathways are Innate Immune System and ERK Signaling. The drugs Mitoxantrone and Analgesics have been mentioned in the context of this disorder. Affiliated tissues include Umbilical Cord, brain and spinal cord, and related phenotypes are sensory impairment and visual loss

Disease Ontology : 12 A central nervous system disease characterized by inflammation of the optic nerve (optic neuritis) and inflammation of the spinal cord (myelitis).

NIH Rare Diseases : 52 Neuromyelitis optica spectrum disorders (NMOSD) affect the spinal cord and optic nerves (nerves that carry visual messages to and from the brain). Symptoms include pain, weakness, bowel and bladder problems, and temporary vision loss. NMOSD usually occurs in adulthood, but symptoms may start at any age. Some people have a single attack of symptoms lasting months, but in most people the symptoms come and go over time. People with NMOSD may develop permanent muscle weakness and vision loss. The cause of NMOSD is unknown. It occurs when the body's immune system mistakenly attacks healthy cells in the spinal cord and eyes. It can be diagnosed by a clinical exam, MRI looking for specific signs, and blood tests looking for certain antibodies . Treatment is focused on managing the symptoms and preventing relapses.

NINDS : 53 Neuromyelitis optica (NMO) is an autoimmune disease of the central nervous system (CNS) that predominantly affects the optic nerves and spinal cord. It is sometimes also referred to as NMO spectrum disorder. In NMO, the body's immune system mistakenly attacks healthy cells and proteins in the body, most often those in the spinal cord and eyes. Individuals with NMO develop optic neuritis, which causes pain in the eye and vision loss. Individuals also develop transverse myelitis, which causes weakness or paralysis of arms and legs,and numbness, along with loss of bladder and bowel control. Magnetic resonance imaging of the spine often shows an abnormality that extends over long segments of the spinal cord. Individuals may also develop episodes of severe nausea and vomiting, with hiccups from involvement of a part of the brain that controls vomiting. The disease is caused by abnormal autoantibodies that bind to a protein called aquaporin-4. Binding of the aquaporin-4 antibody activates other components of the immune system, causing inflammation and damage to these cells. This also results in the brain and spinal cord the loss of myelin, the fatty substance that acts as insulation around nerve fibers and helps nerve signals move from cell to cell.  NMO is different from multiple sclerosis (MS). Attacks are usually more severe in NMO than in MS, and NMO is treated differently than MS. Most individuals with NMO experience clusters of attacks days to months or years apart, followed by partial recovery during periods of remission. Women are more often affected by NMO than men. African Americans are at greater risk of the disease than are Caucasians. The onset of NMO varies from childhood to adulthood, with two peaks, one in childhood and the other in adults in their 40s.

KEGG : 36 Neuromyelitis optica (NMO), also known as Devic's disease is an inflammatory demyelinating disease that selectively affects optic nerves and spinal cord. The etiology of NMO is unknown but it is believed to be an autoimmune disorder triggered by an environmental factor, possibly an infection, in genetically susceptible individuals. The principal effector in NMO is the self-reactive, complement-activating anti-AQP4 antibody. AQP4 water channel is a component of the dystroglycan protein complex located in astrocytic foot processes at the blood-brain barrier. Anti-AQP4 antibody is sensitive and highly specific serum markers of autoimmune NMO. For many decades, NMO was considered to be a subtype of multiple sclerosis (MS), but prognosis and optimal treatments differ. Corticosteroids and plasma exchange (PLEX) are the most commonly used therapeutic modalities in acute settings. Corticosteroids exert global immunosuppressive and anti-inflammatory effects, whereas PLEX removes antibodies, complement, and cytokines from the blood. Besides, immunosuppressant agents interfering with the function of T and B cells have been shown to prevent disease relapses and reduce neurological disability in NMO.

Wikipedia : 74 Neuromyelitis optica (NMO) is a heterogeneous condition consisting of the inflammation and demyelination... more...

Related Diseases for Neuromyelitis Optica

Diseases related to Neuromyelitis Optica via text searches within MalaCards or GeneCards Suite gene sharing:

(show top 50) (show all 757)
# Related Disease Score Top Affiliating Genes
1 optic neuritis 32.8 MOG MBP IL6 IL1B HLA-DRB1 GFAP
2 transverse myelitis 32.2 MBP IL6 IL17A CD27 AQP4
3 acute transverse myelitis 32.0 IL6 AQP4
4 allergic encephalomyelitis 31.7 MOG MBP
5 myelitis 31.5 MOG MBP IL6 IL17A HLA-DPB1 CD40LG
6 neuritis 31.5 MOG MBP IL6 IL4 IL1B GFAP
7 spinal cord injury 31.3 IL6 GFAP AQP4
8 systemic autoimmune disease 31.2 IL6 IL17A
9 acute disseminated encephalomyelitis 31.2 MOG MBP IL1B HLA-DRB1 CD40LG AQP4
10 internuclear ophthalmoplegia 31.2 MOG MBP AQP4
11 sjogren syndrome 31.2 TNFSF13B CXCL13 CD40LG AQP5
12 sleep disorder 31.1 MOG IL6 IL1B
13 vasculitis 31.1 TNFSF13B IL6 IL17A CD40LG
14 herpes zoster 31.0 IFNA1 HLA-DRB1 CD40LG
15 myasthenia gravis 31.0 TNFSF13B MBP IL4 HLA-DPB1 CD40LG
16 neutropenia 31.0 IL6 IL1B IFNA1 CD40LG
17 acute cystitis 31.0 IL6 IL1B CD40LG
18 wernicke encephalopathy 31.0 SLC1A2 GFAP AQP4
19 acute retrobulbar neuritis 30.9 MBP CD40LG AQP4
20 optic papillitis 30.9 MOG CD40LG AQP4
21 pulmonary tuberculosis 30.9 IL6 IL4 IL1B HLA-DRB1
22 thyroiditis 30.9 IL6 IL4 IFNA1
23 guillain-barre syndrome 30.9 MOG MBP HLA-DRB1 CD40LG
24 central nervous system disease 30.9 MOG MBP IL6 IL1B IL17A GFAP
25 chickenpox 30.9 IL6 IL4 IFNA1 CD40LG CD27
26 chronic inflammatory demyelinating polyradiculoneuropathy 30.9 MBP IL17A HLA-DRB1
27 exanthem 30.8 IL6 IL1B IFNA1 CD40LG
28 radiculopathy 30.8 IL6 IL17A IFNA1
29 hydrocephalus 30.8 S100B MBP GFAP AQP4 AQP1
30 aseptic meningitis 30.8 IL1B CXCL13 CD40LG
31 aspiration pneumonia 30.8 IL6 IL17A CD40LG
32 balo concentric sclerosis 30.8 MOG MBP AQP4
33 syphilis 30.7 IL6 IL17A HLA-DRB1 CD40LG
34 spinal disease 30.7 IL6 IL1B IL17A GFAP
35 leukoencephalopathy, hereditary diffuse, with spheroids 30.7 SLC1A2 S100B MBP GFAP
36 asymptomatic neurosyphilis 30.7 CXCL13 CD40LG
37 severe acute respiratory syndrome 30.6 IL6 IFNA1 CXCL10 CD40LG
38 central nervous system lymphoma 30.6 TNFSF13B IL6 CXCL13
39 thymoma 30.6 IL6 IL4 IL17A IFNA1
40 opsoclonus-myoclonus syndrome 30.6 TNFSF13B CXCL10
41 bacterial infectious disease 30.6 IL6 IL1B IL17A CD40LG
42 dermatitis herpetiformis 30.5 IL4 IL17A CD40LG
43 xerophthalmia 30.5 TNFSF13B CD40LG AQP5
44 chlamydia pneumonia 30.5 IL6 IL1B CD40LG
45 primary progressive multiple sclerosis 30.5 S100B MOG MBP IL4 HLA-DRB1
46 neurosyphilis 30.5 IL17A CXCL13 CXCL10
47 pemphigus 30.5 IL6 IL4 HLA-DRB1 CD40LG
48 purpura 30.5 TNFSF13B IL6 IL4 CD40LG
49 polyneuropathy 30.5 MBP IL6 IL1B CD40LG
50 immunoglobulin alpha deficiency 30.5 TNFSF13B CD40LG CD27

Graphical network of the top 20 diseases related to Neuromyelitis Optica:

Diseases related to Neuromyelitis Optica

Symptoms & Phenotypes for Neuromyelitis Optica

Human phenotypes related to Neuromyelitis Optica:

58 31 (show all 15)
# Description HPO Frequency Orphanet Frequency HPO Source Accession
1 sensory impairment 58 31 hallmark (90%) Very frequent (99-80%) HP:0003474
2 visual loss 58 31 hallmark (90%) Very frequent (99-80%) HP:0000572
3 paraplegia 58 31 hallmark (90%) Very frequent (99-80%) HP:0010550
4 optic neuritis 58 31 hallmark (90%) Very frequent (99-80%) HP:0100653
5 neuronal loss in central nervous system 58 31 hallmark (90%) Very frequent (99-80%) HP:0002529
6 functional abnormality of the bladder 58 31 hallmark (90%) Very frequent (99-80%) HP:0000009
7 autoimmune antibody positivity 58 31 hallmark (90%) Very frequent (99-80%) HP:0030057
8 ocular pain 58 31 hallmark (90%) Very frequent (99-80%) HP:0200026
9 peripheral demyelination 58 31 hallmark (90%) Very frequent (99-80%) HP:0011096
10 myelitis 58 31 hallmark (90%) Very frequent (99-80%) HP:0012486
11 abnormality of brain morphology 58 31 frequent (33%) Frequent (79-30%) HP:0012443
12 respiratory failure 58 31 occasional (7.5%) Occasional (29-5%) HP:0002878
13 recurrent singultus 58 31 occasional (7.5%) Occasional (29-5%) HP:0100247
14 nausea 58 31 occasional (7.5%) Occasional (29-5%) HP:0002018
15 csf pleocytosis 58 31 occasional (7.5%) Occasional (29-5%) HP:0012229

MGI Mouse Phenotypes related to Neuromyelitis Optica:

# Description MGI Source Accession Score Top Affiliating Genes
1 hematopoietic system MP:0005397 10.07 AQP1 AQP4 CD27 CD40LG CXCL10 CXCL13
2 homeostasis/metabolism MP:0005376 9.97 AQP1 AQP4 AQP5 CD40LG CXCL10 GFAP
3 immune system MP:0005387 9.77 AQP4 CD27 CD40LG CXCL10 CXCL13 GFAP
4 nervous system MP:0003631 9.36 AQP1 AQP4 CD40LG CXCL10 GFAP IL1B

Drugs & Therapeutics for Neuromyelitis Optica

Drugs for Neuromyelitis Optica (from DrugBank, HMDB, Dgidb, PharmGKB, IUPHAR, NovoSeek, BitterDB):

(show top 50) (show all 60)
# Name Status Phase Clinical Trials Cas Number PubChem Id
Mitoxantrone Approved, Investigational Phase 4 65271-80-9 4212
2 Analgesics Phase 4
Ravulizumab Approved, Investigational Phase 3 1803171-55-2
Azathioprine Approved Phase 3 446-86-6 2265
Mycophenolic acid Approved Phase 3 24280-93-1 446541
6 Complement System Proteins Phase 3
7 Vaccines Phase 3
Bortezomib Approved, Investigational Phase 2 179324-69-7 387447 93860
Hydrocortisone Approved, Vet_approved Phase 2 50-23-7 5754
Hydrocortisone acetate Approved, Vet_approved Phase 2 50-03-3
Metoclopramide Approved, Investigational Phase 2 364-62-5 4168
Chlorpheniramine Approved Phase 2 132-22-9, 113-92-8 2725
Histamine Approved, Investigational Phase 1, Phase 2 51-45-6 774
Cetirizine Approved Phase 1, Phase 2 83881-51-0 2678
4-Aminopyridine Approved Phase 2 504-24-5 1727
Prednisone Approved, Vet_approved Phase 2 53-03-2 5865
Carmustine Approved, Investigational Phase 2 154-93-8 2578
Etoposide Approved Phase 2 33419-42-0 36462
Mechlorethamine Approved, Investigational Phase 2 51-75-2 4033
Cytarabine Approved, Experimental, Investigational Phase 2 147-94-4, 65-46-3 6253
Melphalan Approved Phase 2 148-82-3 460612 4053
Dexamethasone acetate Approved, Investigational, Vet_approved Phase 1, Phase 2 1177-87-3
Dexamethasone Approved, Investigational, Vet_approved Phase 1, Phase 2 50-02-2 5743
Phenylalanine Approved, Investigational, Nutraceutical Phase 2 63-91-2 6140
Cortisone Experimental Phase 2 53-06-5 222786
26 Proteasome Inhibitors Phase 2
27 Hydrocortisone 17-butyrate 21-propionate Phase 2
28 Hydrocortisone hemisuccinate Phase 2
29 Histamine Antagonists Phase 1, Phase 2
30 Histamine H1 Antagonists, Non-Sedating Phase 1, Phase 2
31 Anti-Allergic Agents Phase 1, Phase 2
32 Histamine H1 Antagonists Phase 1, Phase 2
Histamine Phosphate Phase 1, Phase 2 51-74-1 65513
34 Neurotransmitter Agents Phase 1, Phase 2
35 Potassium Channel Blockers Phase 2
36 Antimetabolites Phase 2
37 Anti-Infective Agents Phase 2
38 Etoposide phosphate Phase 2
39 Nitrogen Mustard Compounds Phase 2
40 Dermatologic Agents Phase 2
41 Antiviral Agents Phase 2
42 Podophyllotoxin Phase 2 518-28-5
43 Tubulin Modulators Phase 2
44 Keratolytic Agents Phase 2
45 Antimitotic Agents Phase 2
46 Pharmaceutical Solutions Phase 2
L-Alanine Nutraceutical Phase 2 56-41-7 5950
Bevacizumab Approved, Investigational Phase 1 216974-75-3
49 Angiogenesis Inhibitors Phase 1
50 Complement C1 Inactivator Proteins Phase 1

Interventional clinical trials:

(show top 50) (show all 78)
# Name Status NCT ID Phase Drugs
1 Mycophenolate Mofetil Treatment With Neuromyelitis Optica Spectrum Disorders in Chinese Patients Unknown status NCT02809079 Phase 4 Mycophenolate mofetil;Prednisone
2 Efficacy and Safety of Mitoxantrone in Patients With Refractory Neuromyelitis Optica and Spectrum Disorders Unknown status NCT02021825 Phase 4 Mitoxantrone
3 A Pilot Study of Mitoxantrone for the Treatment of Recurrent Neuromyelitis Optica (Devic’s Disease) Completed NCT00304291 Phase 4 Mitoxantrone
4 Rituximab at Low dosE for neuromyelitiS optiCa spectrUm disordEr (RESCUE): a Prospective, Multicenter, Open-label, Follow-up Clinical Trial Active, not recruiting NCT04256252 Phase 4 Rituximab
5 Phase III Randomized, Double-Blind, Sham-Controlled Study of Plasma Exchange for Acute Severe Attacks of Inflammatory Demyelinating Disease Refractory to Intravenous Methylprednisolone Unknown status NCT00004645 Phase 3
6 Safety and Efficacy of Tocilizumab Versus Azathioprine in Neuromyelitis Optica Spectrum Disorders: a Randomized, Controlled, Open-label, Phase 2 Trial Completed NCT03350633 Phase 2, Phase 3 Tocilizumab Injection;Azathioprine
7 Comparison of Annual Relapse Rate, Expanded Disability Status Scale, and Side Effects Between Azathioprine and Rituximab in Patients With Neuromyelitis Optica Spectrum Disorders Completed NCT03002038 Phase 2, Phase 3 Azathioprine;Rituximab
8 Randomized Clinical Trial of Plasma Exchanges Versus Sham Plasma Exchanges in Disabling Multiple Sclerosis Acute Relapses Refractory to Steroid Treatment Completed NCT01442233 Phase 3
9 A Phase 2/3 Open-Label, Single-Arm Trial to Evaluate the Safety and Activity of Eculizumab in Pediatric Patients With Relapsing Neuromyelitis Optica Spectrum Disorder Recruiting NCT04155424 Phase 2, Phase 3 Eculizumab
10 A Phase 3, External Placebo-Controlled, Open-Label, Multicenter Study to Evaluate the Efficacy and Safety of Ravulizumab in Adult Patients With Neuromyelitis Optica Spectrum Disorder (NMOSD) Recruiting NCT04201262 Phase 3
11 A Phase III Study of RC18, a Recombinant Human B Lymphocyte Stimulator Receptor:Immunoglobulin G( IgG ) Fc Fusion Protein for Injection for the Treatment of Subjects With Neuromyelitis Optica Spectrum Disorders. Recruiting NCT03330418 Phase 3
12 A Double-masked, Placebo-controlled Study With Open-label Period to Evaluate the Efficacy and Safety of MEDI-551 in Adult Subjects With Neuromyelitis Optica and Neuromyelitis Optica Spectrum Disorders Active, not recruiting NCT02200770 Phase 2, Phase 3 Inebilizumab
13 A Phase III, Open-label, Extension Trial of ECU-NMO-301 to Evaluate the Safety and Efficacy of Eculizumab in Patients With Relapsing Neuromyelitis Optica (NMO) Active, not recruiting NCT02003144 Phase 3
14 A Multicenter, Randomized, Double-Blind, Placebo-Controlled, Phase 3 Study to Evaluate the Efficacy and Safety of Satralizumab (SA237) as Monotherapy in Patients With Neuromyelitis Optica (NMO) and Neuromyelitis Optica Spectrum Disorder (NMOSD) Active, not recruiting NCT02073279 Phase 3 Satralizumab;Placebo
15 A Multicenter, Randomized, Addition to Baseline Treatment, Double-Blind, Placebo-Controlled, Phase 3 Study to Evaluate the Efficacy and Safety of Satralizumab (SA237) in Patients With Neuromyelitis Optica (NMO) and NMO Spectrum Disorder (NMOSD) Active, not recruiting NCT02028884 Phase 3 Satralizumab;Placebo;Baseline Treatment
16 A Randomized, Double-Blind, Placebo-Controlled, Multi-Center Trial to Evaluate the Safety and Efficacy of Eculizumab in Patients With Relapsing Neuromyelitis Optica (NMO) Terminated NCT01892345 Phase 3 Eculizumab;Placebo
17 A Multicentre randomiSed Controlled TRial of IntraVEnous Immunoglobulin (IVIg) Versus Standard Therapy for the Treatment of Transverse Myelitis in Adults and Children Terminated NCT02398994 Phase 3 Intravenous Methylprednisolone;Intravenous Immunoglobulin
18 Autologous Hematopoietic Stem Cell Transplant for Neuromyelitis Optica Spectrum Disorder (NMOSD) Withdrawn NCT03829566 Phase 2, Phase 3 Rituximab;Cyclophosphamide;Mesna;rATG;Methylprednisolone;G-CSF
19 Phase I/II Study of Umbilical Cord Mesenchymal Stem Cell Therapy for Patients With Progressive Multiple Sclerosis and Neuromyelitis Optica Unknown status NCT01364246 Phase 1, Phase 2
20 Single-center, Open Label Trial of Bortezomib as add-on Treatment in Relapsing Neuromyelitis Optica Spectrum Disorder Completed NCT02893111 Phase 2 Bortezomib
21 Single-center, Open Label Trial of ACTEMRA® (Tocilizumab) as Monotherapy in Highly Active Neuromyelitis Optica Spectrum Disorders (NMOSD) Completed NCT03062579 Phase 1, Phase 2 Tocilizumab
22 Autologous Mesenchymal Stem Cells for the Treatment of Progressive and Refractory Neuromyelitis Optica Spectrum Disorders: an Open-label Phase 2a Proof-of-concept Study Completed NCT02249676 Phase 2
23 NPB-01(Intravenous Immunoglobulin) Therapy for Patients With Anti-aquaporin 4 Antibody Positive Neuromyelitis Optica Spectrum Disorder Not Provided Adequate Effect of Therapy to Steroids Plus Therapy. Completed NCT01845584 Phase 2 NPB-01
24 An Open Label Study of the Effects of Eculizumab in Neuromyelitis Optica Completed NCT00904826 Phase 1, Phase 2 Eculizumab
25 Trial of High Dose Immunosuppressive Therapy With Hematopoietic Stem Cell Support in Devic's Disease Completed NCT00787722 Phase 1, Phase 2 Cyclophosphamide;G-CSF;rATG;Mesna;Rituximab;Methylprednisolone
26 An Open Label, add-on Trial of Cetirizine for Patients With Neuromyelitis Optica Completed NCT02865018 Phase 1, Phase 2 cetirizine
27 Double-Blind, Placebo-Controlled Crossover Trial on the Safety and Efficacy of Sustained-Release Dalfampridine in Transverse Myelitis (Re-Launch) Completed NCT02166346 Phase 2 Dalfampridine;Placebo
28 A Phase II, Double-blind, Randomized, Multi-center, Adaptive Dose-ranging, Placebo-controlled, Parallel-group Study Evaluating Safety, Tolerability and Efficacy on MRI Lesion Parameters and Determining the Dose Response Curve of BAF312 Given Orally Once Daily in Patients With Relapsing-remitting Multiple Sclerosis. Completed NCT00879658 Phase 2 BAF312;Placebo
29 High-Dose Immunosuppressive Therapy Using Carmustine, Etoposide, Cytarabine, and Melphalan (BEAM) + Thymoglobulin Followed by Syngeneic or Autologous Hematopoietic Cell Transplantation for Patients With Autoimmune Neurologic Diseases Recruiting NCT00716066 Phase 2 Carmustine;Cytarabine;Etoposide;Melphalan;Prednisone
30 Interleukin-6 Receptor Antibodies for Modulating the Systemic Inflammatory Response After Out-of-Hospital Cardiac Arrest - a Randomized Clinical Trial Active, not recruiting NCT03863015 Phase 2 Tocilizumab 20 Mg/mL Intravenous Solution;isotonic saline
31 Non-myeloablative Autologous Hematopoietic Stem Cell Transplantation in Patients With Chronic Inflammatory Demyelinating Polyneuropathy: A Phase II Trial Active, not recruiting NCT00278629 Phase 2
32 THOR - Tübingen Choroideremia Gene Therapy Trial Open Label Phase 2 Clinical Trial Using an Adeno-associated Viral Vector (AAV2) Encoding Rab-escort Protein 1 (REP1) Active, not recruiting NCT02671539 Phase 2
33 Comparison of the Efficacy and Safety of Immunoadsorption and Plasma Exchange for Acute Attack of Refractory Neuromyelitis Optica Spectrum Disorders: a Prospective, Multicenter, Single-blind, Randomized Controlled Clinical Trial Not yet recruiting NCT04064944 Phase 2
34 A Phase-2b, Double-Blind, Randomized Controlled Trial to Evaluate the Activity and Safety of Inebilizumab in Anti-Nmda Receptor Encephalitis and Assess Markers of Disease Not yet recruiting NCT04372615 Phase 2 Inebilizumab;Placebo
35 Autologous Hematopoietic Stem Cell Transplant in Patients With Neuromyelitis Optica Terminated NCT01339455 Phase 1, Phase 2
36 An Open-label Phase 1b Study of Avastin® (Bevacizumab) for the Treatment of Acute Optic Neuritis and/or Transverse Myelitis in Neuromyelitis Optica (NMO) and Neuromyelitis Optica Spectrum Disorder (NMOSD). Completed NCT01777412 Phase 1 Bevacizumab
37 A Double-Blind, Placebo-Controlled, Crossover Study to Evaluate the Safety and Efficacy of ABX-1431 in Patients With Central Pain Completed NCT03138421 Phase 1 ABX-1431 HCl;Placebo
38 Phase I, Single-Center, Open Label Trial of Ublituximab + Glucocorticoids for the Treatment of Acute Optic Neuritis and/or Transverse Myelitis in Neuromyelitis Optica (NMO) and Neuromyelitis Optica Spectrum Disorder (NMOSD) Completed NCT02276963 Phase 1 Ublituximab
39 Phase 1b Study of C1-esterase Inhibitor (Cinryze) With Standard of Care for Acute Treatment of Neuromyelitis Optica Exacerbations Completed NCT01759602 Phase 1 C1-esterase inhibitor (Cinryze)
40 Treatment of Multiple Sclerosis and Neuromyelitis Optica With Regulatory Dendritic Cell: Clinical Trial Phase 1 B Completed NCT02283671 Phase 1
41 Open Label Study of Safety and Tolerability of Rituximab in Neuromyelitis Optica, Recurrent Transverse Myelitis and Recurrent Bilateral Simultaneous Optic Neuritis Completed NCT00501748 Phase 1 Rituximab
42 Safety, Tolerability, Pharmacodynamics and Efficacy of HBM9161 Weekly Subcutaneous Administration in Patients With Neuromyelitis Optica Spectrum Disorders (NMOSD) in China Recruiting NCT04227470 Phase 1 HBM9161 Injection
43 A Phase I Clinical Trial of BAT4406F Injection on the Safety, Tolerability, and Pharmacokinetics in Patients With Neuromyelitis Optica Spectrum Disorders Not yet recruiting NCT04146285 Phase 1 BAT4406F
44 Clinical Study of CD19/CD20 tanCAR T Cells in Relapsed and/or Refractory AQP4-IgG Seropositive Neuromyelitis Optica Spectrum Disorders (NMOSD) Withdrawn NCT03605238 Phase 1
45 A Single Center Open Label Pilot Study of Alpha1-Antitrypsin: A Novel Treatment to Mitigate Neuromyelitis Optica Attacks Withdrawn NCT02087813 Phase 1 Alpha1-antitrypsin;methylprednisolone
46 The French Multiple Sclerosis Registry Unknown status NCT02889965
47 Evaluation of Macular and Retinal Nerve Fiber Layer Thickness Measurements for Detection of Neural Loss in Multiple Sclerosis and Neuromyelitis Optica Using High Resolution Optical Coherence Tomography. Unknown status NCT01024985
48 The Correlation Between AQP-4 Ab and the Visual Function of Patients With Demyelinating ON at Onset Unknown status NCT02886377
49 Phase II, Randomized, Single Blind Sham Controlled Trial Investigating Scrambler Therapy for Neuropathic Pain Caused by Neuromyelitis Optica Spectrum Disorder Completed NCT03452176
50 Optical Coherence Tomography for the Etiological Diagnosis of Inflammatory Optic Neuritis Completed NCT02573792

Search NIH Clinical Center for Neuromyelitis Optica

Cell-based therapeutics:

LifeMap Discovery
Data from LifeMap, the Embryonic Development and Stem Cells Database
Read about Neuromyelitis Optica cell therapies at LifeMap Discovery.

Cochrane evidence based reviews: neuromyelitis optica

Genetic Tests for Neuromyelitis Optica

Anatomical Context for Neuromyelitis Optica

MalaCards organs/tissues related to Neuromyelitis Optica:

Brain, Spinal Cord, Eye, B Cells, T Cells, Testes, Bone
LifeMap Discovery
Data from LifeMap, the Embryonic Development and Stem Cells Database

Cells/anatomical compartments in embryo or adult related to Neuromyelitis Optica:
# Tissue Anatomical CompartmentCell Relevance
1 Umbilical Cord Wharton's Jelly Mesenchymal Stem Cells Potential therapeutic candidate

Publications for Neuromyelitis Optica

Articles related to Neuromyelitis Optica:

(show top 50) (show all 3798)
# Title Authors PMID Year
Marked hypotonia in an infant of a mother with Devic disease. 54 61
20028941 2010
Patterns of antibody binding to aquaporin-4 isoforms in neuromyelitis optica. 61 54
20463974 2010
Immunoglobulin M antibodies to aquaporin-4 in neuromyelitis optica and related disorders. 61 54
20184532 2010
Differential expression of aquaporin-4 isoforms localizes with neuromyelitis optica disease activity. 54 61
20227773 2010
Reappraisal of brain MRI features in patients with multiple sclerosis and neuromyelitis optica according to anti-aquaporin-4 antibody status. 61 54
20122699 2010
Standardized method for the detection of antibodies to aquaporin-4 based on a highly sensitive immunofluorescence assay employing recombinant target antigen. 61 54
20117794 2010
Evidences for a leaky scanning mechanism for the synthesis of the shorter M23 protein isoform of aquaporin-4: implication in orthogonal array formation and neuromyelitis optica antibody interaction. 54 61
20007705 2010
Intra-cerebral injection of neuromyelitis optica immunoglobulin G and human complement produces neuromyelitis optica lesions in mice. 54 61
20047900 2010
Syringomyelia in neuromyelitis optica seropositive for aquaporin-4 antibody. 61 54
20154446 2010
Enhanced IL-6 production in aquaporin-4 antibody positive neuromyelitis optica patients. 61 54
20128675 2010
[Construction and clinical application of lentivirus-AQP4 expressing vector]. 54 61
20356560 2010
Association of anti-Helicobacter pylori neutrophil-activating protein antibody response with anti-aquaporin-4 autoimmunity in Japanese patients with multiple sclerosis and neuromyelitis optica. 61 54
19965522 2009
Live cell analysis of aquaporin-4 m1/m23 interactions and regulated orthogonal array assembly in glial cells. 61 54
19843522 2009
Western blot analysis for the detection of serum antibodies recognizing linear Aquaporin-4 epitopes in patients with Neuromyelitis Optica. 61 54
19850359 2009
Anti-aquaporin-4 auto-antibodies orchestrate the pathogenesis in neuromyelitis optica. 54 61
19389490 2009
Pathologic and immunologic profiles of a limited form of neuromyelitis optica with myelitis. 61 54
19917985 2009
Intrathecal pathogenic anti-aquaporin-4 antibodies in early neuromyelitis optica. 54 61
19938104 2009
[Regulation, structure and function of brain aquaporin]. 54 61
20030210 2009
[Experimental models of neuromyelitis optica]. 61 54
20030243 2009
Antiacquaporin 4 antibodies detection by different techniques in neuromyelitis optica patients. 54 61
19667009 2009
Aquaporin-4 orthogonal arrays of particles are the target for neuromyelitis optica autoantibodies. 54 61
19229993 2009
Cytotoxic effect of neuromyelitis optica antibody (NMO-IgG) to astrocytes: an in vitro study. 54 61
19695715 2009
Extensive vasogenic edema of anti-aquaporin-4 antibody-related brain lesions. 54 61
19625334 2009
"Cloud-like enhancement" is a magnetic resonance imaging abnormality specific to neuromyelitis optica. 61 54
19798642 2009
Aquaporins: translating bench research to human disease. 54 61
19448080 2009
Symptomatic, radiological and pathological involvement of the hypothalamus in neuromyelitis optica. 61 54
19448094 2009
Marked increase in cerebrospinal fluid glial fibrillar acidic protein in neuromyelitis optica: an astrocytic damage marker. 54 61
19372295 2009
Neuromyelitis optica with HTLV-1 infection: different from acute progressive HAM? 61 54
19571450 2009
Autoantibodies in inflammatory demyelinating diseases of the central nervous system. 61 54
18951202 2008
Disseminated encephalomyelitis in adults. 61 54
18703274 2008
Antibody to aquaporin-4 in the long-term course of neuromyelitis optica. 54 61
18945724 2008
Neuromyelitis optica in patients with gluten sensitivity associated with antibodies to aquaporin-4. 61 54
18708571 2008
CNS aquaporin-4 autoimmunity in children. 61 54
18509092 2008
Aquaporin-4 antibodies in neuromyelitis optica and longitudinally extensive transverse myelitis. 61 54
18625857 2008
Neuromyelitis optica (NMO) antibody positivity in patients with transverse myelitis and no visual manifestations. 61 54
18432545 2008
Neuromyelitis optica pathogenesis and aquaporin 4. 54 61
18510734 2008
A prominent elevation of glial fibrillary acidic protein in the cerebrospinal fluid during relapse in neuromyelitis optica. 54 61
18509235 2008
Neuromyelitis optica and anti-aquaporin-4 antibodies measured by an enzyme-linked immunosorbent assay. 61 54
18462810 2008
Mechanisms of disease: aquaporin-4 antibodies in neuromyelitis optica. 61 54
18334978 2008
Water movements in the brain: role of aquaporins. 61 54
18054802 2008
Anti-aquaporin 4 antibody in Japanese multiple sclerosis: the presence of optic spinal multiple sclerosis without long spinal cord lesions and anti-aquaporin 4 antibody. 61 54
17702782 2007
The spectrum of neuromyelitis optica. 54 61
17706564 2007
Neuromyelitis optica. 54 61
17495617 2007
Anti-aquaporin-4 antibody is involved in the pathogenesis of NMO: a study on antibody titre. 54 61
17449477 2007
Loss of aquaporin 4 in lesions of neuromyelitis optica: distinction from multiple sclerosis. 61 54
17405762 2007
Pattern-specific loss of aquaporin-4 immunoreactivity distinguishes neuromyelitis optica from multiple sclerosis. 54 61
17282996 2007
Antibody to aquaporin 4 in the diagnosis of neuromyelitis optica. 54 61
17439296 2007
Absence of aquaporin-4 expression in lesions of neuromyelitis optica but increased expression in multiple sclerosis lesions and normal-appearing white matter. 54 61
17143632 2007
Loss of aquaporin-4 in active perivascular lesions in neuromyelitis optica: a case report. 61 54
16778375 2006
Detection of brain-specific autoantibodies to myelin oligodendrocyte glycoprotein, S100beta and myelin basic protein in patients with Devic's neuromyelitis optica. 54 61
11427318 2001

Variations for Neuromyelitis Optica

Expression for Neuromyelitis Optica

Search GEO for disease gene expression data for Neuromyelitis Optica.

Pathways for Neuromyelitis Optica

Pathways related to Neuromyelitis Optica according to GeneCards Suite gene sharing:

(show all 34)
# Super pathways Score Top Affiliating Genes
Show member pathways
13.98 TNFSF13B S100B IL6 IL4 IL1B IL17A
Show member pathways
13.78 TNFSF13B IL6 IL4 IL1B IL17A CXCL13
Show member pathways
Show member pathways
13.36 TNFSF13B IL6 IL4 IL1B IL17A CXCL13
Show member pathways
13.22 TNFSF13B IL6 IL4 IL1B IL17A CXCL13
Show member pathways
Show member pathways
Show member pathways
12.56 IL6 IL4 IL1B IL17A CD40LG
Show member pathways
Show member pathways
12.51 S100B IL6 IL1B IFNA1 CXCL10
12 12.43 TNFSF13B IL4 IL1B IL17A IFNA1 CXCL10
Show member pathways
Show member pathways
12.3 IL6 IL4 IL1B IL17A CXCL10
Show member pathways
12.22 IL6 IL4 IL1B IL17A IFNA1
Show member pathways
Show member pathways
12.15 IL6 IL4 IL1B CXCL10
19 11.96 IL6 IL4 IL1B IL17A
20 11.85 IL6 IL4 IL1B HLA-DRB1 HLA-DPB1
Show member pathways
11.84 IL6 IL4 IL1B IL17A
Show member pathways
11.75 TNFSF13B CD40LG CD27
23 11.74 IL4 IL1B IL17A
24 11.73 S100B MOG GFAP
Show member pathways
11.62 IL6 IL1B CD40LG
26 11.61 IL6 IL4 IL1B
28 11.51 IL6 IL1B CD40LG
29 11.47 IL6 IL4 IL1B CXCL10
31 11.24 IL6 IL4 IL1B IL17A
32 11.17 IL6 IL4 IL1B HLA-DRB1
33 10.81 TNFSF13B IL6 IL4 IL1B IL17A GFAP
34 10.77 IL6 IL4 IL1B

GO Terms for Neuromyelitis Optica

Cellular components related to Neuromyelitis Optica according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 extracellular region GO:0005576 9.93 TNFSF13B S100B IL6 IL4 IL1B IL17A
2 cell surface GO:0009986 9.73 SLC1A2 MBP HLA-DRB1 HLA-DPB1 CD40LG CD27
3 extracellular space GO:0005615 9.7 TNFSF13B S100B IL6 IL4 IL1B IL17A
4 astrocyte end-foot GO:0097450 9.32 GFAP AQP4
5 external side of plasma membrane GO:0009897 9.17 MOG IL17A HLA-DRB1 CXCL10 CD40LG CD27

Biological processes related to Neuromyelitis Optica according to GeneCards Suite gene sharing:

(show all 21)
# Name GO ID Score Top Affiliating Genes
1 signal transduction GO:0007165 10.15 TNFSF13B IL6 IL4 IL1B IFNA1 HLA-DRB1
2 cell-cell signaling GO:0007267 9.88 IL1B IL17A CXCL13 CXCL10
3 regulation of immune response GO:0050776 9.86 TNFSF13B MOG IL4 CD40LG
4 cellular response to lipopolysaccharide GO:0071222 9.81 IL6 IL1B CXCL13 CXCL10
5 inflammatory response GO:0006954 9.8 IL6 IL1B IL17A CXCL13 CXCL10 CD40LG
6 neutrophil chemotaxis GO:0030593 9.75 IL1B CXCL13 CXCL10
7 B cell differentiation GO:0030183 9.74 IL4 IFNA1 CD40LG
8 positive regulation of interleukin-6 secretion GO:2000778 9.63 MBP IL1B IL17A
9 cytokine-mediated signaling pathway GO:0019221 9.63 IL6 IL4 IL1B IL17A IFNA1 CXCL10
10 positive regulation of neuroinflammatory response GO:0150078 9.6 IL6 IL1B
11 antigen processing and presentation of peptide or polysaccharide antigen via MHC class II GO:0002504 9.59 HLA-DRB1 HLA-DPB1
12 multicellular organismal water homeostasis GO:0050891 9.55 AQP4 AQP1
13 cellular water homeostasis GO:0009992 9.54 AQP4 AQP1
14 positive regulation of glial cell proliferation GO:0060252 9.54 IL6 IL1B GFAP
15 pancreatic juice secretion GO:0030157 9.52 AQP5 AQP1
16 carbon dioxide transport GO:0015670 9.48 AQP5 AQP1
17 immunoglobulin secretion GO:0048305 9.43 TNFSF13B CD40LG
18 water transport GO:0006833 9.43 AQP5 AQP4 AQP1
19 positive regulation of T cell proliferation GO:0042102 9.43 TNFSF13B IL6 IL4 IL1B HLA-DPB1 CD40LG
20 B cell costimulation GO:0031296 9.4 TNFSF13B IL4
21 immune response GO:0006955 9.36 TNFSF13B MBP IL6 IL4 IL1B IL17A

Molecular functions related to Neuromyelitis Optica according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 channel activity GO:0015267 9.33 AQP5 AQP4 AQP1
2 cytokine activity GO:0005125 9.28 TNFSF13B IL6 IL4 IL1B IL17A IFNA1
3 CXCR3 chemokine receptor binding GO:0048248 9.26 CXCL13 CXCL10
4 water channel activity GO:0015250 9.13 AQP5 AQP4 AQP1

Sources for Neuromyelitis Optica

9 Cosmic
10 dbSNP
11 DGIdb
17 EFO
18 ExPASy
19 FMA
28 GO
29 GTR
31 HPO
32 ICD10
33 ICD10 via Orphanet
37 LifeMap
41 MedGen
43 MeSH
44 MESH via Orphanet
45 MGI
48 NCI
49 NCIt
54 Novoseek
57 OMIM via Orphanet
61 PubMed
70 Tocris
72 UMLS via Orphanet
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