MOG antibody-associated disease
MOG antibody-associated disease is an antibody-mediated inflammatory demyelinating disorder of the central nervous system, directed at myelin oligodendrocyte glycoprotein, that presents as optic neuritis, transverse myelitis, or acute disseminated encephalomyelitis and encephalitis, with a monophasic or recurrent, steroid-responsive course. It is confirmed by MOG antibody testing in a compatible syndrome, separated from multiple sclerosis by limited brain involvement, longitudinally extensive cord lesions, and marked CSF pleocytosis; attacks are treated with high-dose IV methylprednisolone and plasma exchange, and recurrent disease with long-term immunosuppression.
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What this article covers
- Overview
Definition: MOG antibody-associated disease (MOGAD) is an antibody-mediated inflammatory demyelinating disorder of the central nervous system in which antibodies against myelin oligodendrocyte…
- Epidemiology
Less common than MS: MS remains the most common inflammatory demyelinating disease of the central nervous system, so MOGAD must be actively sought with antibody testing.
- Risk factors
Preceding infection: single-episode demyelinating events such as ADEM and transverse myelitis are often postinfectious, and most transverse myelitis follows a recent gastroenteritis or upper respiratory infection.
- Pathophysiology
Antibody-directed inflammation: antibodies against myelin oligodendrocyte glycoprotein trigger inflammatory demyelination, concentrated in the optic nerves and spinal cord.
- Clinical features
Optic neuritis: subacute loss of vision in one or both eyes over days, with pain on eye movement, reduced acuity, a scotoma, impaired color vision, and a relative afferent pupillary defect…
- Differential diagnosis
Spinal cord compression and spinal epidural abscess: compressive myelopathy with localized back pain and, in abscess, fever; urgent spine MRI excludes it before an inflammatory diagnosis is made; see spinal cord compression.
- Diagnosis
Basis of diagnosis: MOGAD is established by a positive MOG antibody test in a patient with a core inflammatory demyelinating syndrome (optic neuritis, transverse myelitis, or ADEM or encephalitis)…
- Management
First, start high-dose IV glucocorticoids for the acute attack: methylprednisolone 1 g IV once daily for 3-5 days (up to 7 days for myelitis); screen for infection, uncontrolled diabetes, and peptic ulcer disease before the pulse.
- Complications
Permanent visual loss: optic nerve atrophy, seen as a pale, flat disc weeks to months after optic neuritis, can leave fixed visual deficits.
- Prognosis
Steroid-responsive attacks: acute attacks generally respond to high-dose glucocorticoids, with plasma exchange for those that do not.
- Prevention
No primary prevention: prevention is directed at relapses and at avoiding harmful MS therapy.
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- Multiple sclerosis
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- Optic neuritis
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- Transverse myelitis
Transverse myelitis is inflammation across one or more contiguous spinal cord segments causing subacute weakness, a sensory level, and bladder or bowel dysfunction; diagnosis requires spinal MRI showing cord enhancement or CSF inflammation with exclusion of compressive and infectious causes, and idiopathic disease is treated with high-dose intravenous methylprednisolone.
- Acute disseminated encephalomyelitis
Acute disseminated encephalomyelitis is a monophasic, often postinfectious, immune-mediated demyelinating illness that produces large, diffuse inflammatory lesions of the brain and spinal cord. Fever and encephalopathy with multifocal deficits separate it from multiple sclerosis; treatment is high-dose IV methylprednisolone, with plasma exchange for steroid-refractory disease, after infection is excluded.
- Autoimmune encephalitis
Autoimmune encephalitis is antibody-mediated brain inflammation, most often N-methyl-D-aspartate (NMDA) receptor antibody disease, that classically opens with a psychiatric prodrome and psychosis in a young patient before progressing to seizures, abnormal movements, and autonomic instability. Diagnosis requires antibody testing of blood and cerebrospinal fluid plus a tumor search; treatment is tumor removal plus immunotherapy, intravenous methylprednisolone and immune globulin first, escalating to rituximab or cyclophosphamide.
- Spinal cord compression
Spinal cord compression is mechanical narrowing of the spinal canal by tumor, hematoma, abscess, disc herniation, or trauma that produces myelopathy; it is a neurologic emergency requiring urgent MRI of the affected level and immediate treatment of the cause, since surgical decompression rarely restores function once paraplegia develops.