Neuromyelitis optica spectrum disorder
Alternate names[edit | edit source]
Devic syndrome; NMO; Devic's neuromyelitis optica; Devic disease; NMO spectrum disorder; Neuromyelitis optica; Neuromyelitis optica spectrum disorders
Definition[edit | edit source]
Neuromyelitis optica spectrum disorders (NMOSD) affect the spinal cord and optic nerves (nerves that carry visual messages to and from the brain).
Epidemiology[edit | edit source]
It has been estimated that between 0.5 - 4.4/100,000 people has a neuromyelitis optica spectrum disorder. It has been reported to be more prevalent in Asians and Africans.
Cause[edit | edit source]
- The cause of neuromyelitis optica spectrum disorders (NMOSD) is unknown.
- It is considered an autoimmune disease in which the immune system mistakenly attacks cells in the spinal cord and optic nerves.
- Many people who develop NMOSD have another autoimmune disease.
- It is also possible that genetic factors may be involved.
Onset[edit | edit source]
Symptoms typically begin in adulthood but can start at any age.
Signs and symptoms[edit | edit source]
Signs and symptoms may include:
- Inflammation of the optic nerve (optic neuritis)
- Temporary vision loss
- Inflammation of the spinal cord (acute transverse myelitis)
- Pain
- Abnormal sensations
- Weakness in the arms and legs
- Bladder and bowel control problems
- Episodes of nausea and vomiting
- Optic neuritis tends to occur suddenly and causes eye pain and varying degrees of vision loss.
- Transverse myelitis develops over hours or days.
- Most people with NMOSD have episodes or "attacks" of symptoms months or years apart (the relapsing form), while others have a single episode lasting several months.
- NMOSD may cause permanent disability.
Clinical presntation[edit | edit source]
For most diseases, symptoms will vary from person to person. People with the same disease may not have all the symptoms listed.
80%-99% of people have these symptoms
- Autoimmune antibody positivity
- Functional abnormality of the bladder
- Myelitis(Inflammation of spinal cord)
- Neuronal loss in central nervous system(Loss of brain cells)
- Ocular pain(Eye pain)
- Optic neuritis
- Paraplegia(Leg paralysis)
- Peripheral demyelination
- Sensory impairment
- Visual loss(Loss of vision)
30%-79% of people have these symptoms
- Abnormality of brain morphology(Abnormal shape of brain)
5%-29% of people have these symptoms
- CSF pleocytosis
- Nausea
- Recurrent singultus(Recurrent hiccup)
- Respiratory failure
Diagnosis[edit | edit source]
- A diagnosis of neuromyelitis optica spectrum disorder (NMOSD) is based upon a clinical examination looking for the presence of characteristic symptoms and imaging studies (MRI) of the brain, spinal cord, and eyes.
- Additional testing may include a blood test looking for a specific type of antibody and a spinal tap to collect a small amount of fluid that surrounds the brain and spinal cord to look for white blood cells.
Treatment[edit | edit source]
- There is no cure for NMO.
- The U.S.Food and Drug Administration (FDA) has approved three drug treatments (eculizumab, inebilizumab-cdon, and satralizumab-mwge) which can be injected to reduce the risk of relapses in adults who are anti-aquaporin-4 antibody positive.
- NMO relapses and attacks are often treated with corticosteroid drugs and plasma exchange (also called plasmapheresis, a process used to remove harmful antibodies from the bloodstream).
- Immunosuppressvie drugs used to prevent attacks include mycophenolate mofetil, rituximab, and azathioprine.
- Pain, stiffness, muscle spasms, and bladder and bowel control problems can be managed with medications and therapies.
- Individuals with major disability will require the combined efforts to physical and occupational therapists, along with social services professionals to address complex rehabilitation needs.
NIH genetic and rare disease info[edit source]
Neuromyelitis optica spectrum disorder is a rare disease.
Neuromyelitis optica spectrum disorder Resources | ||
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