What is NMO-SD?: Neuromyelitis optica, also known as Devic’s disease, is an autoimmune disorder in which immune system cells and antibodies mistakenly attack and destroy myelin cells in the optic nerves and the spinal cord. The damage to the optic nerves produces swelling and inflammation causes pain and loss of vision; the damage to the spinal cord causes weakness or paralysis in the legs or arms, loss of sensation, and problems with bladder and bowel function. NMO occurs in two different forms: a single attack extending over a month or two, or a more common relapsing form in which the person experiences multiple attacks.
Who gets pediatric NMO?: Patients usually experience their first symptoms in childhood or adolescence. Patients as young as 16 months have been reported with NMO. The average age for children is 10 years old.
Symptoms of pediatric NMO: Children with NMO usually present with “attacks” of new neurological symptoms appearing within a few hours or days. Children with NMO can have attacks with symptoms of blurred vision, or loss of vision in one eye or both eyes. Other symptoms are weakness or numbness of the arms or legs. Some children experience confusion or extreme lethargy at their attacks. Other possible symptoms including vomiting, fever or seizures.
Laboratory testing: Approximately 65% of children with NMO have antibodies to aquaporin-4 (AQP4), also known as NMO-IgG. AQP4 antibodies are sometimes present at the first symptom, or can appear with time. About 15% of children with NMO have MOG antibodies (see section below). About 15% of children are negative for both antibodies.
Treatment of pediatric NMO: Although there are no FDA approved treatments for adults or children with NMO, children are usually offered immunomodulatory treatments. Attacks are treated with a short course (usually up to a week) of intravenous steroids, or IVIG or plasmapheresis. It is important to prevent new attacks in children with established NMO, and treatment with mycophenolate mofetil or rituximab is usually offered.