NMO
MCID: NRM001
MIFTS: 62

Neuromyelitis Optica (NMO)

Categories: Eye diseases, Immune diseases, Neuronal diseases, Rare diseases
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Aliases & Classifications for Neuromyelitis Optica

MalaCards integrated aliases for Neuromyelitis Optica:

Name: Neuromyelitis Optica 11 19 42 52 53 43 14 36 16 71 33
Devic Syndrome 19 42 75 33
Devic Disease 19 42 58 33
Neuromyelitis Optica Spectrum Disorder 19 58 75
Devic's Disease 11 42
Neuromyelitis Optica Spectrum Disorders 19
Optic Neuritis with Demyelination 33
Opticospinal Multiple Sclerosis 71
Optic Neuroencephalomyelopathy 33
Devic's Neuromyelitis Optica 19
Nmo - [neuromyelitis Optica] 33
Devic Neuromyelitis Optica 42
Ophthalmoneuromyelitis 33
Nmo Spectrum Disorder 19
Optic Neuromyelitis 33
Devic's Syndrome 11
Optic-Spinal Ms 42
Opticospinal Ms 42
Nmosd 58
Devic 33
Nmo 19

Characteristics:


Inheritance:

Neuromyelitis Optica Spectrum Disorder: Multigenic/multifactorial 58

Prevelance:

Neuromyelitis Optica Spectrum Disorder: 1-9/1000000 (Martinique, Worldwide, Austria, Spain, Hungary, Australia, India, Canada, Malaysia, Cuba, Costa rica) <1/1000000 (Austria, United Kingdom, Netherlands, Spain, Denmark, Sweden, Australia, United States, Cuba) 1-9/100000 (Worldwide, United Kingdom, Denmark, Sweden, Hungary, United States, India, Japan, Malaysia, Iran, Islamic Republic of) 1-5/10000 (Martinique) 58

Age Of Onset:

Neuromyelitis Optica Spectrum Disorder: Adolescent,Adult,Childhood,Elderly 58

Age Of Death:

Neuromyelitis Optica Spectrum Disorder: any age 58

Classifications:

Orphanet: 58  
Rare neurological diseases
Rare eye diseases


External Ids:

Disease Ontology 11 DOID:8869
ICD9CM 34 341.0
MeSH 43 D009471
NCIt 49 C84934
SNOMED-CT 68 25044007
ICD10 31 G36.0
MESH via Orphanet 44 D009471
ICD10 via Orphanet 32 G36.0
UMLS via Orphanet 72 C0027873
Orphanet 58 ORPHA71211
UMLS 71 C0027873 C3711850

Summaries for Neuromyelitis Optica

MedlinePlus Genetics: 42 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 transverse myelitis and neuritis. An important gene associated with Neuromyelitis Optica is AQP4 (Aquaporin 4), and among its related pathways/superpathways are Innate Immune System and Akt Signaling. The drugs Antineoplastic Agents, Immunological and Daratumumab have been mentioned in the context of this disorder. Affiliated tissues include Umbilical Cord, spinal cord and eye, and related phenotypes are optic neuritis and visual loss

NINDS: 52 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.

GARD: 19 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.

Orphanet: 58 A rare inflammatory disease of the central nervous system characterized mainly by attacks of uni- or bilateral optic neuritis (ON) and acute myelitis.

Disease Ontology: 11 A central nervous system disease characterized by inflammation of the optic nerve (optic neuritis) and inflammation of the spinal cord (myelitis).

Wikipedia: 75 Neuromyelitis optica spectrum disorders (NMOSD), including neuromyelitis optica (NMO), are autoimmune... 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 902)
# Related Disease Score Top Affiliating Genes
1 transverse myelitis 32.3 MOG MBP IL6 HLA-DRB1 AQP4
2 neuritis 32.3 MOG MBP IL6 IL1B GFAP AQP4
3 myelitis 32.0 MOG MBP IL6 IL17A HLA-DPB1 CD27
4 optic neuritis 31.9 MOG MBP IL6 IL1B HLA-DRB1 HLA-DQB1
5 spinal cord disease 31.6 MOG MBP IL6 GFAP AQP4
6 relapsing-remitting multiple sclerosis 31.6 MOG MBP IL6 IL1B IL17A
7 sjogren syndrome 31.6 TNFSF13B CXCL13 CD40LG AQP5
8 demyelinating disease 31.5 NEFL MOG MBP IL6 IL1B IL17A
9 acute disseminated encephalomyelitis 31.4 NEFL MOG MBP IL6 AQP4
10 myasthenia gravis 31.3 TNFSF13B MBP IL6 HLA-DRB1 HLA-DQB1 HLA-DPB1
11 pediatric multiple sclerosis 31.2 HLA-DRB1 HLA-DQB1
12 encephalitis 31.2 MOG IL6 IL1B GFAP AQP4
13 central nervous system disease 31.1 MOG MBP IL17A GFAP
14 internuclear ophthalmoplegia 31.1 MOG MBP AQP4
15 autoimmune disease 31.1 TNFSF13B MOG MBP IL6 IL1B IL17A
16 sleep disorder 31.1 MOG IL6 IL1B HLA-DQB1
17 optic nerve disease 31.0 TNFSF13B NEFL MOG MBP IL6 IL1B
18 acute retrobulbar neuritis 31.0 MOG MBP AQP4
19 proteasome-associated autoinflammatory syndrome 1 31.0 IL6 IL1B IL17A
20 systemic scleroderma 30.9 TNFSF13B IL6 IL1B IL17A HLA-DRB1
21 facial paralysis 30.9 MOG MBP IL6 AQP4
22 exanthem 30.9 IL6 IL1B IL17A
23 viral infectious disease 30.9 IL6 IL1B IL17A CD40LG
24 pulmonary tuberculosis 30.9 IL6 IL1B HLA-DRB1 HLA-DQB1 C3
25 thyroiditis 30.9 IL6 IL1B HLA-DRB1 HLA-DQB1
26 spinal cord injury 30.9 IL6 GFAP AQP4
27 central pontine myelinolysis 30.8 MBP AQP4 AQP1
28 chickenpox 30.8 MOG IL6 IL1B IL17A CD27 AQP4
29 japanese encephalitis 30.8 HLA-DRB1 HLA-DQB1 HLA-DPB1
30 syphilis 30.7 IL17A HLA-DRB1 CD40LG
31 balo concentric sclerosis 30.7 MOG MBP AQP4
32 aseptic meningitis 30.7 MOG IL6 IL1B
33 connective tissue disease 30.7 TNFSF13B IL6 IL1B IL17A HLA-DRB1 HLA-DPB1
34 severe acute respiratory syndrome 30.7 IL6 IL1B IL17A
35 stroke, ischemic 30.7 SLC1A2 MBP IL6 IL1B AQP4
36 demyelinating polyneuropathy 30.7 NEFL MOG MBP IL6 AQP4
37 meningoencephalitis 30.7 MOG MBP IL6 IL1B CD40LG
38 optic papillitis 30.7 MOG AQP4
39 myositis 30.6 IL6 IL1B IL17A HLA-DRB1
40 guillain-barre syndrome 30.6 MOG MBP IL17A HLA-DRB1 HLA-DQB1 CD40LG
41 progressive multifocal leukoencephalopathy 30.6 TNFSF13B MOG MBP IL17A AQP4
42 pneumonia 30.6 IL6 IL1B IL17A CD40LG
43 dermatitis herpetiformis 30.6 IL17A HLA-DRB1 HLA-DQB1
44 hydrocephalus 30.6 S100B MBP GFAP AQP4 AQP1
45 tetanus 30.6 IL6 IL1B CD40LG CD27
46 spondylitis 30.6 IL6 IL1B IL17A HLA-DRB1
47 celiac disease 1 30.6 TNFSF13B IL6 IL1B IL17A HLA-DRB1 HLA-DQB1
48 secondary progressive multiple sclerosis 30.6 S100B NEFL MOG MBP IL17A CD40LG
49 sarcoidosis 1 30.5 IL6 IL1B IL17A HLA-DRB1 HLA-DQB1 CD27
50 spondyloarthropathy 1 30.5 IL6 IL1B IL17A CD40LG

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 30 (show all 16)
# Description HPO Frequency Orphanet Frequency HPO Source Accession
1 optic neuritis 58 30 Hallmark (90%) Very frequent (99-80%)
HP:0100653
2 visual loss 58 30 Hallmark (90%) Very frequent (99-80%)
HP:0000572
3 paraplegia 58 30 Hallmark (90%) Very frequent (99-80%)
HP:0010550
4 neuronal loss in central nervous system 58 30 Hallmark (90%) Very frequent (99-80%)
HP:0002529
5 functional abnormality of the bladder 58 30 Hallmark (90%) Very frequent (99-80%)
HP:0000009
6 autoimmune antibody positivity 58 30 Hallmark (90%) Very frequent (99-80%)
HP:0030057
7 ocular pain 58 30 Hallmark (90%) Very frequent (99-80%)
HP:0200026
8 peripheral demyelination 58 30 Hallmark (90%) Very frequent (99-80%)
HP:0011096
9 myelitis 58 30 Hallmark (90%) Very frequent (99-80%)
HP:0012486
10 somatic sensory dysfunction 30 Hallmark (90%) HP:0003474
11 abnormality of brain morphology 58 30 Frequent (33%) Frequent (79-30%)
HP:0012443
12 recurrent singultus 58 30 Occasional (7.5%) Occasional (29-5%)
HP:0100247
13 respiratory failure 58 30 Occasional (7.5%) Occasional (29-5%)
HP:0002878
14 nausea 58 30 Occasional (7.5%) Occasional (29-5%)
HP:0002018
15 csf pleocytosis 58 30 Occasional (7.5%) Occasional (29-5%)
HP:0012229
16 sensory impairment 58 Very frequent (99-80%)

GenomeRNAi Phenotypes related to Neuromyelitis Optica according to GeneCards Suite gene sharing:

25
# Description GenomeRNAi Source Accession Score Top Affiliating Genes
1 no effect GR00402-S-1 10.14 AQP1 AQP4 AQP5 C3 CD27 CD40LG
2 no effect GR00402-S-2 10.14 AQP4 C3 CD27 CD40LG CXCL13 HLA-DPB1

MGI Mouse Phenotypes related to Neuromyelitis Optica:

45
# Description MGI Source Accession Score Top Affiliating Genes
1 nervous system MP:0003631 10.1 AQP1 AQP4 C3 CD40LG GFAP HLA-DQB1
2 growth/size/body region MP:0005378 10.07 AQP1 AQP4 AQP5 C3 GFAP HLA-DQB1
3 behavior/neurological MP:0005386 9.97 AQP1 AQP4 AQP5 C3 GFAP HLA-DQB1
4 immune system MP:0005387 9.8 AQP1 AQP4 C3 CD27 CD40LG CXCL13
5 hematopoietic system MP:0005397 9.44 AQP1 AQP4 C3 CD27 CD40LG CXCL13

Drugs & Therapeutics for Neuromyelitis Optica

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

(show top 50) (show all 86)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1 Antineoplastic Agents, Immunological Phase 4
2
Daratumumab Approved Phase 2, Phase 3 945721-28-8
3
Ravulizumab Approved, Investigational Phase 3 1803171-55-2
4
Azathioprine Approved Phase 3 446-86-6 2265
5
Eculizumab Approved, Investigational Phase 3 219685-50-4
6 Antimetabolites Phase 3
7 glucocorticoids Phase 3
8 Complement System Proteins Phase 3
9 Immunologic Factors Phase 3
10 Vaccines Phase 3
11 Hormones Phase 3
12 Hormone Antagonists Phase 3
13 Anti-Inflammatory Agents Phase 3
14
Hydrochlorothiazide Approved, Vet_approved Phase 2 58-93-5 3639
15
Amiloride Approved Phase 2 17440-83-4, 2016-88-8, 2609-46-3 16231
16
Chlorpheniramine Approved Phase 2 113-92-8, 132-22-9 2725
17
Metoclopramide Approved, Investigational Phase 2 364-62-5 4168
18
Hydrocortisone Approved, Vet_approved Phase 2 50-23-7 3640 5754
19
Hydrocortisone succinate Approved Phase 2 2203-97-6 3643
20
Hydrocortisone acetate Approved, Vet_approved Phase 2 50-03-3
21
Bortezomib Approved, Investigational Phase 2 179324-69-7 387447 93860
22
Cetirizine Approved Phase 1, Phase 2 83881-51-0 2678
23
Histamine Approved, Investigational Phase 1, Phase 2 51-45-6 774
24
Dalfampridine Approved Phase 2 504-24-5 1727
25
Belimumab Approved Phase 1, Phase 2 356547-88-1
26
Prednisone Approved, Vet_approved Phase 2 53-03-2 5865
27
Cytarabine Approved, Experimental, Investigational Phase 2 147-94-4, 65-46-3 596 6253 6175
28
Etoposide Approved Phase 2 33419-42-0 36462
29
Carmustine Approved, Investigational Phase 2 154-93-8 2578
30
Melphalan Approved Phase 2 148-82-3 4053 460612
31
Mechlorethamine Approved, Investigational Phase 2 51-75-2 4033
32
Picropodophyllin Approved, Investigational Phase 2 518-28-5, 477-47-4 10607 72435
33
Zanubrutinib Approved, Investigational Phase 2 1691249-45-2 135565884
34
Ofatumumab Approved Phase 1, Phase 2 679818-59-8
35
Mitoxantrone Approved, Investigational Phase 2 70476-82-3, 65271-80-9 4212
36
Rituximab Approved Phase 1, Phase 2 174722-31-7
37
Dexamethasone acetate Approved, Investigational, Vet_approved Phase 1, Phase 2 1177-87-3 3680
38
Dexamethasone Approved, Investigational, Vet_approved Phase 1, Phase 2 50-02-2 3003 5743
39
D-Phenylalanine Approved, Experimental, Investigational, Nutraceutical Phase 2 63-91-2, 673-06-3 6140 71567
40
D-Tyrosine Approved, Experimental, Investigational, Nutraceutical Phase 2 133585-56-5, 556-02-5, 60-18-4 1153 6057
41
Cortisone Experimental Phase 2 53-06-5 222786
42 Antihypertensive Agents Phase 2
43 Sodium Chloride Symporter Inhibitors Phase 2
44 Sodium Channel Blockers Phase 2
45 Amiloride, hydrochlorothiazide drug combination Phase 2
46 Diuretics, Potassium Sparing Phase 2
47 diuretics Phase 2
48 Hydrocortisone 17-butyrate 21-propionate Phase 2
49 Proteasome Inhibitors Phase 2
50
Histamine phosphate Phase 1, Phase 2 51-74-1 134614

Interventional clinical trials:

(show top 50) (show all 97)
# Name Status NCT ID Phase Drugs
1 Efficacy and Safety of Mitoxantrone in Patients With Refractory Neuromyelitis Optica and Spectrum Disorders Unknown status NCT02021825 Phase 4 Mitoxantrone
2 Mycophenolate Mofetil Treatment With Neuromyelitis Optica Spectrum Disorders in Chinese Patients Unknown status NCT02809079 Phase 4 Mycophenolate mofetil;Prednisone
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 Completed NCT04256252 Phase 4 Rituximab
5 SAkuraBonsai: Clinical, Imaging And Biomarker Open-Label Study In Neuromyelitis Optica Spectrum Disorder (NMOSD) With Satralizumab As An Intervention Recruiting NCT05269667 Phase 4 Satralizumab 120 mg
6 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
7 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) Completed NCT02073279 Phase 3 Satralizumab;Placebo
8 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. Completed NCT02200770 Phase 2, Phase 3 Inebilizumab
9 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) Completed NCT02003144 Phase 3
10 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
11 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) Completed NCT02028884 Phase 3 Satralizumab;Placebo;Baseline Treatment
12 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
13 A Multi-center, Randomized, Double-Blind, Placebo-Controlled Trial to Evaluate the Safety and Efficacy of Daratumumab in Patients With Anti-Aquaporin 4 Antibody Positive Neuromyelitis Optica Spectrum Disorders (NMOSD) Recruiting NCT05403138 Phase 2, Phase 3 Daratumumab;Placebo
14 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
15 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
16 A Phase 2/3, Open-label, Historical-controlled, Single-arm, Multicenter Study to Evaluate the Efficacy, Pharmacokinetics, Pharmacodynamics, and Safety of Ravulizumab in Children and Adolescents With Aquaporin-4 Antibody Positive (AQP4-Ab [+]) Neuromyelitis Optica Spectrum Disorder (NMOSD) Recruiting NCT05346354 Phase 2, Phase 3 Ravulizumab
17 A Multicenter, Phase Ib/III Study to Evaluate the Safety and Efficacy of MIL62 in Patients With Neuromyelitis Optica Spectrum Disorder (NMOSD) Recruiting NCT05314010 Phase 3 MIL62
18 A Phase III, Multicenter, Open-Label, Uncontrolled Study To Evaluate Pharmacokinetics, Efficacy, Safety, Tolerability, And Pharmacodynamics Of Satralizumab In Pediatric Patients With AQP4 Antibody Positive Neuromyelitis Optica Spectrum Disorder (NMOSD) Recruiting NCT05199688 Phase 3 Satralizumab
19 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) Active, not recruiting NCT04201262 Phase 3
20 A Multicenter, Single Arm, Open-Label Study to Evaluate the Long-Term Safety and Efficacy of Satralizumab in Patients With Neuromyelitis Optica Spectrum Disorder (NMOSD) Active, not recruiting NCT04660539 Phase 3 satralizumab;azathioprine (AZA);mycophenolate mofetil (MMF);oral corticosteroids
21 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
22 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
23 Autologous Hematopoietic Stem Cell Transplant for Neuromyelitis Optica Spectrum Disorder (NMOSD) Withdrawn NCT03829566 Phase 2, Phase 3 Rituximab;Cyclophosphamide;Mesna;rATG;Methylprednisolone;G-CSF
24 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
25 A Randomized, Placebo-Controlled, Double-Blind, Phase IIa Study of Amiloride in the Treatment of Acute Autoimmune Optic Neuritis Unknown status NCT01879527 Phase 2 Amiloride hydrochlorothiazide;Sugar pill
26 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
27 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
28 Single-center, Open Label Trial of Bortezomib as add-on Treatment in Relapsing Neuromyelitis Optica Spectrum Disorder Completed NCT02893111 Phase 2 Bortezomib
29 An Open Label, add-on Trial of Cetirizine for Patients With Neuromyelitis Optica Completed NCT02865018 Phase 1, Phase 2 cetirizine
30 An Open Label Study of the Effects of Eculizumab in Neuromyelitis Optica Completed NCT00904826 Phase 1, Phase 2 Eculizumab
31 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
32 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
33 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
34 Efficacy and Safety of Belimumab in Neuromyelitis Optica Spectrum Disorders Recruiting NCT05154734 Phase 1, Phase 2 Belimumab
35 An Open-Label Multicenter Study to Evaluate the Pharmacokinetics, Pharmacodynamics, and Safety of Inebilizumab in Pediatric Subjects With Neuromyelitis Optica Spectrum Disorder Recruiting NCT05549258 Phase 2 Inebilizumab
36 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
37 An Open Label Trial Evaluating the Efficacy and Safety of Bruton's Tyrosine Kinase (BTK) Inhibitor Zanubrutinib in Adult Patients With Neuromyelitis Optical Spectrum Disorders (NMOSDs) Recruiting NCT05356858 Phase 2 zanubrutinib
38 Efficacy and Safety of Ofatumumab in AQP4-IgG Seropositive NMOSD: an Open-label, Single-arm, Multicentre Prospective Pilot Study Recruiting NCT05504694 Phase 1, Phase 2 Ofatumumab
39 A Two-arm, Randomized, Double-blind, Placebocontrolled, Monocentric Study to Evaluate the Safety and Tolerability of ACT-01 Compared to Placebo in Patients With Acute Optic Neuritis Recruiting NCT04762017 Phase 2 ACT-01 IV administration
40 An Open Label Phase II Clinical Trial Evaluating the Efficacy and Safety of SHR1459 in Adult Patients With Neuromyelitis Optical Spectrum Disorders (NMOSDs) Active, not recruiting NCT04670770 Phase 2 Drug - SHR1459
41 High Frequency Impulse Therapy for Neuropathic Pain in NMOSD Active, not recruiting NCT04614454 Phase 2
42 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
43 A Randomized, Double-blind, Placebo-controlled Phase Ⅱ Clinical Study to Evaluate the Efficacy and Safety of Mitoxantrone Hydrochloride Liposome Injection in the Treatment of Neuromyelitis Optica Spectrum Disorder (NMOSD) Not yet recruiting NCT05551598 Phase 2 Mitoxantrone Hydrochloride Liposome Injection
44 Autologous Hematopoietic Stem Cell Transplant in Patients With Neuromyelitis Optica Terminated NCT01339455 Phase 1, Phase 2
45 A Phase 2, Randomized, DB-PC, Parallel Group Study for the Treatment of Visual Pathway Deficits In Chronic Optic Neuropathy to Assess the Efficacy, Safety, Tolerability and Pharmacokinetics of CNM-Au8 For Remyelination In Multiple Sclerosis Terminated NCT03536559 Phase 2 CNM-Au8;Placebo
46 A Phase I Clinical Trial of BAT4406F Injection on the Safety, Tolerability, and Pharmacokinetics in Patients With Neuromyelitis Optica Spectrum Disorders Unknown status NCT04146285 Phase 1 BAT4406F
47 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
48 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
49 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
50 Safety, Tolerability, Pharmacodynamics and Efficacy of HBM9161 Weekly Subcutaneous Administration in Patients With Neuromyelitis Optica Spectrum Disorders (NMOSD) in China Completed NCT04227470 Phase 1 HBM9161 Injection

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

Organs/tissues related to Neuromyelitis Optica:

MalaCards : Spinal Cord, Eye, Brain, T Cells, B Cells, Medulla Oblongata, Neutrophil
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 5439)
# Title Authors PMID Year
1
Marked hypotonia in an infant of a mother with Devic disease. 53 62
20028941 2010
2
Patterns of antibody binding to aquaporin-4 isoforms in neuromyelitis optica. 53 62
20463974 2010
3
Immunoglobulin M antibodies to aquaporin-4 in neuromyelitis optica and related disorders. 53 62
20184532 2010
4
Differential expression of aquaporin-4 isoforms localizes with neuromyelitis optica disease activity. 53 62
20227773 2010
5
Reappraisal of brain MRI features in patients with multiple sclerosis and neuromyelitis optica according to anti-aquaporin-4 antibody status. 53 62
20122699 2010
6
Standardized method for the detection of antibodies to aquaporin-4 based on a highly sensitive immunofluorescence assay employing recombinant target antigen. 53 62
20117794 2010
7
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. 53 62
20007705 2010
8
Intra-cerebral injection of neuromyelitis optica immunoglobulin G and human complement produces neuromyelitis optica lesions in mice. 53 62
20047900 2010
9
Syringomyelia in neuromyelitis optica seropositive for aquaporin-4 antibody. 53 62
20154446 2010
10
Enhanced IL-6 production in aquaporin-4 antibody positive neuromyelitis optica patients. 53 62
20128675 2010
11
[Construction and clinical application of lentivirus-AQP4 expressing vector]. 53 62
20356560 2010
12
Live cell analysis of aquaporin-4 m1/m23 interactions and regulated orthogonal array assembly in glial cells. 53 62
19843522 2009
13
Western blot analysis for the detection of serum antibodies recognizing linear Aquaporin-4 epitopes in patients with Neuromyelitis Optica. 53 62
19850359 2009
14
Anti-aquaporin-4 auto-antibodies orchestrate the pathogenesis in neuromyelitis optica. 53 62
19389490 2009
15
Association of anti-Helicobacter pylori neutrophil-activating protein antibody response with anti-aquaporin-4 autoimmunity in Japanese patients with multiple sclerosis and neuromyelitis optica. 53 62
19965522 2009
16
[Regulation, structure and function of brain aquaporin]. 53 62
20030210 2009
17
Intrathecal pathogenic anti-aquaporin-4 antibodies in early neuromyelitis optica. 53 62
19938104 2009
18
Pathologic and immunologic profiles of a limited form of neuromyelitis optica with myelitis. 53 62
19917985 2009
19
[Experimental models of neuromyelitis optica]. 53 62
20030243 2009
20
Cytotoxic effect of neuromyelitis optica antibody (NMO-IgG) to astrocytes: an in vitro study. 53 62
19695715 2009
21
Antiacquaporin 4 antibodies detection by different techniques in neuromyelitis optica patients. 53 62
19667009 2009
22
Aquaporin-4 orthogonal arrays of particles are the target for neuromyelitis optica autoantibodies. 53 62
19229993 2009
23
"Cloud-like enhancement" is a magnetic resonance imaging abnormality specific to neuromyelitis optica. 53 62
19798642 2009
24
Extensive vasogenic edema of anti-aquaporin-4 antibody-related brain lesions. 53 62
19625334 2009
25
Symptomatic, radiological and pathological involvement of the hypothalamus in neuromyelitis optica. 53 62
19448094 2009
26
Aquaporins: translating bench research to human disease. 53 62
19448080 2009
27
Marked increase in cerebrospinal fluid glial fibrillar acidic protein in neuromyelitis optica: an astrocytic damage marker. 53 62
19372295 2009
28
Neuromyelitis optica with HTLV-1 infection: different from acute progressive HAM? 53 62
19571450 2009
29
Disseminated encephalomyelitis in adults. 53 62
18703274 2008
30
Antibody to aquaporin-4 in the long-term course of neuromyelitis optica. 53 62
18945724 2008
31
Autoantibodies in inflammatory demyelinating diseases of the central nervous system. 53 62
18951202 2008
32
Neuromyelitis optica in patients with gluten sensitivity associated with antibodies to aquaporin-4. 53 62
18708571 2008
33
Aquaporin-4 antibodies in neuromyelitis optica and longitudinally extensive transverse myelitis. 53 62
18625857 2008
34
CNS aquaporin-4 autoimmunity in children. 53 62
18509092 2008
35
Neuromyelitis optica pathogenesis and aquaporin 4. 53 62
18510734 2008
36
Neuromyelitis optica (NMO) antibody positivity in patients with transverse myelitis and no visual manifestations. 53 62
18432545 2008
37
Neuromyelitis optica and anti-aquaporin-4 antibodies measured by an enzyme-linked immunosorbent assay. 53 62
18462810 2008
38
A prominent elevation of glial fibrillary acidic protein in the cerebrospinal fluid during relapse in neuromyelitis optica. 53 62
18509235 2008
39
Mechanisms of disease: aquaporin-4 antibodies in neuromyelitis optica. 53 62
18334978 2008
40
Water movements in the brain: role of aquaporins. 53 62
18054802 2008
41
The spectrum of neuromyelitis optica. 53 62
17706564 2007
42
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. 53 62
17702782 2007
43
Neuromyelitis optica. 53 62
17495617 2007
44
Loss of aquaporin 4 in lesions of neuromyelitis optica: distinction from multiple sclerosis. 53 62
17405762 2007
45
Pattern-specific loss of aquaporin-4 immunoreactivity distinguishes neuromyelitis optica from multiple sclerosis. 53 62
17282996 2007
46
Anti-aquaporin-4 antibody is involved in the pathogenesis of NMO: a study on antibody titre. 53 62
17449477 2007
47
Antibody to aquaporin 4 in the diagnosis of neuromyelitis optica. 53 62
17439296 2007
48
Absence of aquaporin-4 expression in lesions of neuromyelitis optica but increased expression in multiple sclerosis lesions and normal-appearing white matter. 53 62
17143632 2007
49
Loss of aquaporin-4 in active perivascular lesions in neuromyelitis optica: a case report. 53 62
16778375 2006
50
Detection of brain-specific autoantibodies to myelin oligodendrocyte glycoprotein, S100beta and myelin basic protein in patients with Devic's neuromyelitis optica. 53 62
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 22)
# Super pathways Score Top Affiliating Genes
1
Show member pathways
13.82 TNFSF13B S100B IL6 IL1B IL17A HLA-DRB1
2
Show member pathways
13.28 TNFSF13B IL6 IL1B IL17A CXCL13 CD40LG
3
Show member pathways
13.15 TNFSF13B IL6 IL1B IL17A CXCL13 CD40LG
4
Show member pathways
12.93 TNFSF13B S100B IL6 IL1B IL17A HLA-DRB1
5 12.44 SLC1A2 S100B NEFL MBP GFAP
6 11.94 GFAP IL1B IL6 SLC1A2
7
Show member pathways
11.78 IL6 IL1B IL17A
8
Show member pathways
11.73 IL6 IL1B IL17A
9
Show member pathways
11.65 HLA-DRB1 HLA-DQB1 HLA-DPB1
10 11.59 IL6 CD40LG C3
11 11.57 IL6 IL1B IL17A
12 11.57 S100B NEFL MOG GFAP
13 11.56 TNFSF13B MBP IL6 IL1B GFAP AQP4
14
Show member pathways
11.52 CD40LG IL1B IL6
15 11.42 TNFSF13B IL6 IL1B CD40LG
16 11.38 HLA-DRB1 HLA-DQB1 HLA-DPB1 CD40LG
17 11.19 IL1B IL17A HLA-DRB1 HLA-DQB1 HLA-DPB1 CXCL13
18 11.14 IL6 IL1B IL17A
19 11.07 IL6 IL1B HLA-DRB1
20 11.03 IL6 IL1B IL17A
21 10.7 CD40LG CXCL13 GFAP IL17A IL1B IL6
22 10.67 IL6 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 plasma membrane GO:0005886 10.39 SLC1A2 HLA-DRB1 CD40LG CD27 AQP5 AQP4
2 plasma membrane GO:0005887 10.39 SLC1A2 HLA-DRB1 CD40LG CD27 AQP5 AQP4
3 external side of plasma membrane GO:0009897 9.93 AQP4 CD27 CD40LG HLA-DRB1 IL17A MOG
4 cell surface GO:0009986 9.77 SLC1A2 MBP HLA-DRB1 HLA-DPB1 CD40LG CD27
5 transport vesicle membrane GO:0030658 9.72 HLA-DRB1 HLA-DQB1 HLA-DPB1
6 astrocyte end-foot GO:0097450 9.56 GFAP AQP4
7 MHC class II protein complex GO:0042613 9.1 HLA-DRB1 HLA-DQB1 HLA-DPB1

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

(show all 21)
# Name GO ID Score Top Affiliating Genes
1 inflammatory response GO:0006954 10.3 IL6 IL1B IL17A CXCL13 CD40LG C3
2 positive regulation of interleukin-6 production GO:0032755 10.08 IL17A IL1B IL6 MBP
3 T cell receptor signaling pathway GO:0050852 10.05 MOG HLA-DRB1 HLA-DQB1 HLA-DPB1
4 positive regulation of T cell activation GO:0050870 9.96 HLA-DRB1 HLA-DQB1 HLA-DPB1
5 positive regulation of vascular endothelial growth factor production GO:0010575 9.93 IL6 IL1B C3
6 water transport GO:0006833 9.91 AQP5 AQP4 AQP1
7 pancreatic juice secretion GO:0030157 9.9 AQP5 AQP1
8 vascular endothelial growth factor production GO:0010573 9.89 IL6 IL1B
9 antigen processing and presentation of exogenous peptide antigen via MHC class II GO:0019886 9.89 HLA-DRB1 HLA-DQB1 HLA-DPB1
10 cellular water homeostasis GO:0009992 9.88 AQP4 AQP1
11 D-aspartate import across plasma membrane GO:0070779 9.88 SLC1A2 GFAP
12 regulation of vascular endothelial growth factor production GO:0010574 9.85 AQP4 IL6
13 positive regulation of glial cell proliferation GO:0060252 9.8 IL6 IL1B GFAP
14 carbon dioxide transport GO:0015670 9.8 AQP5 AQP4 AQP1
15 peptide antigen assembly with MHC class II protein complex GO:0002503 9.77 HLA-DRB1 HLA-DQB1 HLA-DPB1
16 antigen processing and presentation GO:0019882 9.74 HLA-DRB1 HLA-DQB1 HLA-DPB1
17 water homeostasis GO:0030104 9.71 AQP1 AQP4
18 positive regulation of T cell proliferation GO:0042102 9.65 TNFSF13B IL6 IL1B HLA-DPB1 CD40LG
19 immune response GO:0006955 9.58 TNFSF13B MBP IL6 IL1B IL17A HLA-DRB1
20 immunoglobulin production involved in immunoglobulin-mediated immune response GO:0002381 9.43 HLA-DPB1 HLA-DQB1 HLA-DRB1
21 antigen processing and presentation of peptide or polysaccharide antigen via MHC class II GO:0002504 9.33 HLA-DRB1 HLA-DQB1 HLA-DPB1

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

# Name GO ID Score Top Affiliating Genes
1 peptide antigen binding GO:0042605 9.72 HLA-DRB1 HLA-DQB1 HLA-DPB1
2 MHC class II protein complex binding GO:0023026 9.65 HLA-DRB1 HLA-DQB1 HLA-DPB1
3 water channel activity GO:0015250 9.43 AQP5 AQP4 AQP1
4 cytokine activity GO:0005125 9.36 TNFSF13B IL6 IL1B IL17A CXCL13 CD40LG
5 channel activity GO:0015267 9.33 AQP5 AQP4 AQP1

Sources for Neuromyelitis Optica

2 CDC
6 CNVD
8 Cosmic
9 dbSNP
10 DGIdb
16 EFO
17 ExPASy
18 FMA
19 GARD
27 GO
28 GTR
29 HMDB
30 HPO
31 ICD10
32 ICD10 via Orphanet
33 ICD11
34 ICD9CM
35 IUPHAR
36 LifeMap
38 LOVD
40 MedGen
43 MeSH
44 MESH via Orphanet
45 MGI
48 NCI
49 NCIt
50 NDF-RT
52 NINDS
53 Novoseek
55 ODiseA
56 OMIM via Orphanet
57 OMIM® (Updated 24-Oct-2022)
61 PubChem
62 PubMed
64 QIAGEN
69 SNOMED-CT via HPO
70 Tocris
71 UMLS
72 UMLS via Orphanet
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