Neuromyelitis optica spectrum disorder

Alternate names

Devic syndrome; NMO; Devic's neuromyelitis optica; Devic disease; NMO spectrum disorder; Neuromyelitis optica; Neuromyelitis optica spectrum disorders

Definition

Neuromyelitis optica spectrum disorders (NMOSD) affect the spinal cord and optic nerves (nerves that carry visual messages to and from the brain).

Epidemiology

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

• 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

Symptoms typically begin in adulthood but can start at any age.

Signs and symptoms

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

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

• 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

• 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

• NIH info on Neuromyelitis optica spectrum disorder

Neuromyelitis optica spectrum disorder is a rare disease.

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