Myelin Oligodendrocyte Glycoprotein Antibody Disease

Myelin oligodendrocyte glycoprotein antibody disease (MOGAD) is a neurological, immune-mediated demyelinating disorder of the central nervous system that causes inflammation and potential damage in the optic nerve, spinal cord, brain and/or brainstem. Myelin oligodendrocyte glycoprotein (MOG) is a protein found on the covering of nerves in the central nervous system. While the precise function of MOG is not fully understood, it likely plays a role in myelin maturation, myelin integrity, and cell surface interactions. In MOG antibody disease, the immune system attacks the MOG protein found on myelin covering of nerves.

MOGAD causes inflammation on the brain, spinal cord and/or optic nerves and can result in the following symptoms:

• Lost or blurred vision in one or both eyes
• Loss of color vision
• Pain in the eyes, back, legs, arms or chest area
• Paralysis or weakness in arms and legs
• Loss of sensation, numbness or tingling
• Bladder or bowel problems
• Seizures
• Confusion, drowsiness or coma

MOGAD symptoms are often grouped in the following categories:

• Optic neuritis – visual loss, pain with eye movement, reduced color vision
• Transverse myelitis – numbness or weakness of the limbs, bladder dysfunction
• Acute disseminated encephalomyelitis (ADEM) – confusion, drowsiness, encephalopathy with other neurological deficits
• Cerebral encephalitis – seizures, encephalopathy

The exact cause of MOGAD is not known. MOG antibody disease affects males and females almost equally and are more prevalent in children than adults.

Diagnosing MOGAD quickly and correctly is critical. At Children's Hospital of Philadelphia's Neuroscience Center, a child’s care begins with a comprehensive, personalized assessment.

Patients who show the symptoms listed above will undergo a serum blood test. The diagnosis of MOGAD is confirmed if the test comes back clearly positive for MOG antibodies. MRIs of the brain, orbits and spine may also reveal lesions or white matter changes suggestive of an inflammatory process. As appropriate, our Neuroimmune specialists will also rule out other conditions that may look similar to MOG antibody disease.

Diagnostic tests include:

• Lumbar puncture (spinal tap)
• Laboratory blood test
• Magnetic resonance imaging (MRI)

At the Neuroscience Center, our goal is to stop symptoms and restore a child's normal growth and development with as few treatment-related side effects as possible.

Treatments of MOGAD may include:

• Intravenous corticosteroids
• Plasma exchange (PLEX)
• Intravenous immunoglobulin (IVIG)
• Disease modifying therapies (DMTs) including therapies for children with relapsing forms of MOGAD

Most patients respond well to intravenous steroids, with some also requiring oral prednisone for one to four weeks. Complete recovery from the onset attack is common in children, and in general the outcome is favorable. However, patients who experience more severe attacks and relapsing MOGAD are at risk for more severe deficits.

Relapses can occur. In children with relapsing disease, studies suggest use of intravenous immunoglobulin (IVIG) and/or disease modifying therapies (DMTs). Relapses can have long-term effects on the central nervous system, including gait and vision challenges.

The CHOP Neuroscience Center is actively engaged in MOG antibody disease research. Dr. Brenda Banwell, Director of the Neuroimmune Program at CHOP, has studied MOGAD and related neuroimmune conditions for more than 20 years, and has studied immune responses, therapeutic strategies, and imaging features. Dr. Banwell leads an international team working to establish new diagnostic criteria. In partnership with Amit Bar-Or, MD, Director of the NeuroImmune Program at the Hospital of the University of Pennsylvania, we provide an age-span program that offers novel precision neuroimmunology discovery research. Our Neuroimmune Program offers the highest quality of patient care, innovative investigation that drives new therapies, and years of experienced guidance on patient care.