Neuromyelitis Optica

Neuromyelitis optica is a rare autoimmune condition that causes inflammation in the central nervous system, particularly the nerves from the eye and in the spinal cord. It occurs when the body’s immune system mistakenly attacks cells that support nerves and myelin, the protective sheath that covers nerves. Neuromyelitis optica can cause loss of vision, limb weakness, paralysis, and other symptoms.

About 3 to 5 percent of cases occur in children. The condition can be either monophasic or relapsing:

• Monophasic neuromyelitis optica occurs once, with no other attacks.
• Relapsing neuromyelitis optica involves an initial attack, followed by periodic flareups. The majority of neuromyelitis optica cases are relapsing. All cases where a child has aquaporin 4 antibodies are considered relapsing.
   

The symptoms of neuromyelitis optica depend on the location of nerve inflammation. Symptoms are typically divided into two main groups:

Optic neuritis:

• Vision loss in one or both eyes
• Pain that gets worse when moving the eye(s)
• Decreased ability to see color

Transverse myelitis:

• Weakness in the arms or legs
• Numbness or tingling in the arms or legs
• Muscle stiffness
• Loss of bowel or bladder control
• Paralysis

Neuromyelitis optica can also cause sleepiness, nausea, vomiting, and chronic hiccups in some children.

Symptoms can wax and wane, with relapses occurring weeks, months, or years after the initial attack. They may resolve entirely or lead to permanent damage.

In neuromyelitis, the immune system creates aquaporin 4 antibodies, which trigger a cascade of inflammation that permanently injures certain nerve cells. It may be triggered by an infection. Children with neuromyelitis optica may also have another autoimmune condition, such as multiple sclerosis or lupus.

Your child’s doctor will perform a physical and neurological exam and ask about their medical history and symptoms. They will also recommend other tests to help diagnose neuromyelitis optica, such as:

• Blood tests
• Eye exam
• Lumbar puncture (spinal tap)
• MRI scans

Treatment for neuromyelitis optica is aimed at addressing inflammation and preventing future relapses. Depending on your child’s individual case, treatment options may include:

• Corticosteroids. Steroid medications help relieve inflammation and address related symptoms. They are typically given intravenously at high doses for a set number of days, then tapering off to a lower dose over several weeks. Your child’s doctor may also recommend a course of oral steroid medication for a longer period of time.
• Plasmapheresis (plasma exchange). Plasmapheresis is a procedure that removes the part of your child’s blood called plasma and separates it from the red blood cells. Through this process, antibodies related to neuromyelitis optica are removed from the blood plasma. The red blood cells are then mixed with a plasma substitute and returned to your child’s body. It may be an option for children who have severe symptoms or haven’t responded enough to steroids.
• Other treatments. Your child’s doctor may prescribe immunosuppressants or other drugs to prevent relapses. We recommend long-term treatment for all children who have antibody-positive neuromyelitis optica.

At Boston Children’s Hospital, we care for children with neuromyelitis optica in our Neuroimmunology Center. Our team takes a multidisciplinary approach, working with other specialists throughout Boston Children’s who have expertise in caring for children with neuromyelitis optica, including ophthalmologists, rehabilitation doctors, social workers, physical therapists, education specialists, and others. Together, we will create a treatment plan that puts your family in the center.