Longevity with Neuromyelitis Optica

Neuromyelitis optica or NMO or Devic disease is an inflammatory disorder affecting the eyes and spinal cord. Until recently neuromyelitis optica often was misdiagnosed or confused with a type of multiple scelerosis or MS. It is a serious and incurable but treatable disease that may be monophasic or relapsing.

Symptoms

• Generally NMO is associated with symptoms such as vision loss in one or both eyes, paralysis, sometimes loss of bowel or bladder function, and possibly painful spasms.

  Many patients only experience minor vision loss, but up to 40 percent have total blindness in the affected eye.

  After onset of symptoms, conditions may worsen over hours or days, but eventually 78 to 88 percent of patients regain some function in the affected area.

Diagnosis

• Diagnosis of neuromyelitis optica requires MRI or magnetic resonance imaging of the brain and spinal cord. Discovery of an antibody in the blood of 70 percent of those with neuromyelitis optica which is not found in individuals with MS has helped with diagnosing the disorder. This antibody known as NMO-IgG can be detected with a simple blood test.

Monophasic NMO

• The monophasic form, 13 percent of the NMO cases, is when the disease strikes and lasts approximately a month or two with severe symptoms, and affects men and women equally. There is usually moderate recovery. Typically vision regained is in the 20/30 level or better although 22 percent remain functionally blind, at 20/200 or worse in at least one eye.

  Limb weakness is frequently seen, along with moderate sphincter problems and occasional urinary incontinence. Some permanent paralysis occurs in 31 percent of monophasic patients.

  Those with monophasic forms of NMO after the recovery period will remain stable. The five year survival rate for this type of neuromyelitis optica is 90 percent.

Relapsing

• Relapsing neuromyelitis optica is much more common than the monophasic variety, and makes up between 70 and 87 percent of NMO cases. This form affects women at a much greater rate, four to one, than men.

  The relapsing form has a highly erratic course, with clusters of attacks spaced months or years apart, sometimes with periods of partial recovery in the remissions. The effects of the attacks, however, are cumulative and each new bout with the disease means a new area is attacked. Muscle weakness and paralysis are common, and the most dangerous complication is when respiration is affected by that muscle weakness. The use of artificial ventilation is often required.

  Prognosis of relapsing neuromyelitis is a five year survival rate of 68 percent. Those who succumb to the disorder typically die from respiratory failure.

Treatment

• Treatment of neuromyelitis optica consists of managing acute attacks, reducing symptoms, preventing future attacks and rehabilitating patients with permanent disabilities.

  Generally, treatment is with corticosteroids via IV for acute phases, and with oral dosing as follow up. Those who don't respond well to corticosteroid therapy may do better with treatment with plasma exchange, where the patient has their antibodies are separated in the blood.

  Ongoing research into other treatment regimens, specifically other drugs to treat NMO are yielding some hopeful results.