Devic's Disease

Signs and Symptoms

The hallmark symptom of Devic's disease is a sudden loss of vision in both eyes. Other symptoms may include:

• Numbness in the arms and/or legs
• Muscle weakness in the arms and/or legs
• Sudden, brief and/or repetitive spasms in the arms and legs
• Balance difficulties
• Difficulties with bladder or bowel function

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Diagnosis

An accurate and early diagnosis of Devic's disease is critical in the management of your child's condition and quality of life.

In making a diagnosis of Devic's disease, your child's doctor will first start by conducting a thorough physical examination, asking about any symptoms your child is experiencing, including when they started and how they've eased or progressed over time. Your child's doctor will also record their full medical history, including information about your immediate and extended family's medical history.

In many ways, Devic's disease closely resembles multiple sclerosis (MS) and therefore may be misdiagnosed in the early stages. However, there are some key differences between the two conditions that a neurologist can recognize. Generally, attacks of Devic's disease tend to be more frequent and severe than in MS. Also, Devic's disease affects only the optic nerves and spinal cord, while MS affects the brain as well.

Magnetic Resonance Imagining (MRI):

Your child will have a magnetic resonance imaging (MRI) of the brain to look for lesions or areas of damage that may indicate MS or other disorders. In most cases, children with Devic's disease will have a normal brain MRI, while those with MS will show evidence of damage or lesions. Your child will also have an MRI of the spinal cord. Children with Devic's disease will show significant inflammation of the spinal cord, whereas those with MS will have less severe inflammation. An MRI scan is a non-invasive procedure that uses powerful magnets and radio waves to construct clear, detailed pictures of brain and spinal cord tissues.

Other Tests:

Your child may have a recently developed blood test that checks for NMO antibodies, which helps establish a definite diagnosis of Devic's disease.

Your child also will have a lumbar puncture or spinal tap to determine if there are abnormalities in their cerebrospinal fluid (CSF). The CSF is the fluid that bathes, cushions and protects the brain and spinal cord. It flows through the skull and spine in the subarachnoid space, which is the area inside the arachnoid membrane. Typically, in Devic's disease, CSF lacks the elevation of antibodies detected in children with MS.

An eye exam by an ophtalmologist who will look for optic nerve damage may also be recommended. In addition, an evoked potentials test, which records electrical activity in the brain when nerves are stimulated, may also be conducted.

A diagnosis of Devic's disease is based upon an evaluation of your child's symptoms, along with the results of their physical exam and tests.

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Treatment

Children and adolescents with Devic's disease receive treatment at our Pediatric Multiple Sclerosis Center. Our team of experts specializes in Devic's disease and related conditions. We work with each patient to develop a unique treatment plan, including long-term follow-up care specifically tailored to his or her needs. When necessary, we also collaborate with other specialists at UCSF or elsewhere to ensure that your child receives the most comprehensive care possible. Additionally, because we are part of an international network of six pediatric MS centers sponsored by the National Multiple Sclerosis Society, we have access to the latest information, research and treatments.

Treatment will depend on the severity of your child's condition and their symptoms. Intravenous (IV) steroids and/or oral steroid therapy may be given to your child to manage attacks and control symptoms. Immune suppressant medications, either taken intravenously or orally, may also be recommended to prevent future attacks. Children who do not respond to steroids or immune suppressant drugs and continue to experience attacks may benefit from plasma exchange. In this procedure, blood is extracted and a machine separates the blood cells from the plasma. Blood cells are then mixed with a replacement solution that resembles plasma, which is then returned to the body.

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