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Medication Summary

Eculizumab, a monoclonal antibody that targets C5, is the first drug specifically approved by the US Food and Drug Administration (FDA) for adults with neuromyelitis optica spectrum disorder (NMOSD) who are seropositive for anti-aquaporin-4 (AQP4) antibody.^[15] Inebilizumab, a monoclonal antibody that binds to CD19, and satralizumab, a monoclonal antibody that targets the interleukin-6 (IL-6) receptor, are also approved for treating NMOSD.^{[16, 52, 53]}

Other pharmacologic therapy for optic neuritis (ON) is directed at ameliorating the acute symptoms of pain and decreased vision caused by demyelinating inflammation of the nerve. Varying regimens of corticosteroids have been used for this purpose. A 3-day course of high-dose intravenous (IV) methylprednisolone followed by a rapid oral taper of prednisone has been shown to provide a rapid relief of symptoms in the acute phase. This treatment may also delay the short-term development of multiple sclerosis (MS) after ON; however, IV steroids do little to affect the ultimate visual acuity in patients with ON.

For patients with ON whose brain lesions on magnetic resonance imaging (MRI) indicate a high risk of developing clinically definite MS, treatment with immunomodulators (eg, interferon beta-1a, interferon beta-1b, glatiramer acetate) may be considered.^[17] Intravenous immunoglobulin treatment of acute ON has been shown to have no beneficial effect.

Class Summary

Monoclonal antibodies (eg, eculizumab, inebilizumab, satralizumab) that target aquaporin-4-antibody production or binding have been approved by the FDA for adults with neuromyelitis optica spectrum disorder (NMOSD).

Eculizumab (Soliris)

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Monoclonal antibody that specifically binds to the complement protein C5 with high affinity, thereby inhibiting its cleavage to C5a and C5b and preventing the generation of the terminal complement complex C5b-9. The precise mechanism by which eculizumab exerts its therapeutic effect in NMOSD is unknown, but is presumed to involve inhibition of aquaporin-4-antibody–induced terminal complement C5b-9 deposition. It is indicated for adults with neuromyelitis optica spectrum disorder (NMOSD) who are anti-aquaporin-4 (AQP4) antibody–positive.

Inebilizumab (Uplizna)

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Monoclonal antibody that binds with high affinity to CD19, a protein expressed on a broad range of B cells, including antibody-secreting plasmablasts and plasma cells. After binding to CD19, these cells are rapidly depleted from the circulation. Approximately 80% of patients with NMOSD have autoantibodies to a water channel protein called aquaporin-4 (AQP4). These AQP4-IgG autoantibodies are produced by plasmablasts and plasma cells and bind primarily to astrocytes in the central nervous system. Binding of AQP4-IgG antibodies to CNS cells is believed to trigger attacks, which can damage the optic nerve, spinal cord, and brain.

Satralizumab (Enspryng)

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Monoclonal antibody that targets the interleukin 6 (IL-6) receptor. Cytokine IL-6 is thought to be a key cause of NMOSD, triggering the inflammation cascade and leading to damage and disability. It is indicated for NMOSD in adults who are antiaquaporin-4 (AQP4) antibody positive.

Class Summary

These have anti-inflammatory properties and cause profound and varied metabolic effects. In addition, these agents modify the body's immune response to diverse stimuli.

Methylprednisolone (Solu-Medrol, Depo-Medrol, Medrol)

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Methylprednisolone is a synthetic corticosteroid used intravenously as an anti-inflammatory and immunosuppressant agent. It has been shown to facilitate the recovery of vision in the acute phase of optic neuritis even though it may not change the long-term visual outcome. In addition, treatment with methylprednisolone may delay the development of MS.

Prednisone

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Prednisone may decrease inflammation by reversing increased capillary permeability and suppressing polymorphonuclear (PMN) leukocyte activity. It is a commonly used oral agent. Prednisone is used for an oral taper of steroids, which may reduce the emotional effects of steroid withdrawal and the risk of the development of adrenocortical insufficiency. However, these risks are not very high after only 3 days of treatment with high-dose steroids, and most neurologists do not use a prednisone taper.

Prednisolone (Pediapred, Prelone, Orapred)

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Prednisolone may decrease inflammation by reversing increased capillary permeability and suppressing polymorphonuclear (PMN) leukocyte activity. It is a commonly used oral agent. Prednisolone is used for an oral taper of steroids, which may reduce the emotional effects of steroid withdrawal and the risk of the development of adrenocortical insufficiency. However, these risks are not very high after only 3 days of treatment with high-dose steroids, and most neurologists do not use a prednisone taper.

Questions

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Media Gallery

A case of acute optic neuritis. A. 1.5 Tesla, contrast-enhanced spin echo T1-weighted, fat-suppressed coronal MRI through the orbits shows enlargement and contrast enhancement of the left optic nerve in the retrobulbar portion (arrow). B. Coronal spin echo T1-weighted, fat-suppressed MRI of the same patient shows enlargement and contrast enhancement of the nerve in a parasagittal oblique section (arrow).

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Author

Andrew A Dahl, MD, FACS Assistant Professor of Surgery (Ophthalmology), New York College of Medicine (NYCOM); Director of Residency Ophthalmology Training, The Institute for Family Health and Mid-Hudson Family Practice Residency Program; Staff Ophthalmologist, Telluride Medical Center

Andrew A Dahl, MD, FACS is a member of the following medical societies: American Academy of Ophthalmology, American College of Surgeons, American Intraocular Lens Society, American Medical Association, American Society of Cataract and Refractive Surgery, Contact Lens Association of Ophthalmologists, Medical Society of the State of New York, New York State Ophthalmological Society, Outpatient Ophthalmic Surgery Society

Disclosure: Nothing to disclose.

Chief Editor

Edsel B Ing, MD, MPH, FRCSC, PhD, MA Professor, Department of Ophthalmology and Vision Sciences, University of Toronto Faculty of Medicine; Active Staff, Michael Garron Hospital (Toronto East Health Network)

Edsel B Ing, MD, MPH, FRCSC, PhD, MA is a member of the following medical societies: American Academy of Ophthalmology, American Association for Pediatric Ophthalmology and Strabismus, American Society of Ophthalmic Plastic and Reconstructive Surgery, Canadian Medical Association, Canadian Ophthalmological Society, Canadian Society of Oculoplastic Surgery, Chinese Canadian Medical Society, European Society of Ophthalmic Plastic and Reconstructive Surgery, North American Neuro-Ophthalmology Society, Ontario Medical Association, Royal College of Physicians and Surgeons of Canada, Statistical Society of Canada

Disclosure: Nothing to disclose.

Additional Contributors

Erhan Ergene, MD Clinical Assistant Professor, Department of Neurology, University of Illinois College of Medicine at Peoria; Medical Director, Comprehensive Epilepsy Program and Clinical Neurophysiology, Illinois Neurological Institute at OSF Saint Francis Medical Center

Erhan Ergene, MD is a member of the following medical societies: American Academy of Neurology

Disclosure: Nothing to disclose.

Nancy A Machens, APN, CNP Professor of Nursing, Bradley University; Advanced Practice Nurse, Nurse Practitioner, Department of Neurology, Illinois Neurological Institute at OSF Saint Francis Medical Center

Disclosure: Nothing to disclose.

Reuben M Valenzuela, MD Clinical Assistant Professor of Neurology, Section of Neuro-ophthalmology, Section of Multiple Sclerosis, Illinois Neurological Institute, University of Illinois College of Medicine Peoria; Neurophthalmologist, OSF HealthCare Illinois Neurological Institute

Disclosure: Nothing to disclose.

Acknowledgements

Edsel Ing, MD, FRCSC Associate Professor, Department of Ophthalmology and Vision Sciences, University of Toronto Faculty of Medicine; Consulting Staff, Toronto East General Hospital, Canada

Edsel Ing, MD, FRCSC is a member of the following medical societies: American Academy of Ophthalmology, American Association for Pediatric Ophthalmology and Strabismus, American Society of Ophthalmic Plastic and Reconstructive Surgery, Canadian Ophthalmological Society, North American Neuro-Ophthalmology Society, and Royal College of Physicians and Surgeons of Canada

Disclosure: Nothing to disclose.

Simon K Law, MD, PharmD Associate Professor of Ophthalmology, Jules Stein Eye Institute, University of California, Los Angeles, David Geffen School of Medicine

Simon K Law, MD, PharmD is a member of the following medical societies: American Academy of Ophthalmology, American Glaucoma Society, and Association for Research in Vision and Ophthalmology

Disclosure: Nothing to disclose.

Brian R Younge, MD Professor of Ophthalmology, Mayo Clinic School of Medicine

Brian R Younge, MD is a member of the following medical societies: American Medical Association, American Ophthalmological Society, and North American Neuro-Ophthalmology Society

Disclosure: Nothing to disclose.

Acknowledgments

The authors and editors of Medscape Reference gratefully acknowledge the assistance of Ryan I Huffman, MD, with the literature review and referencing for this article.

Adult Optic Neuritis Medication

Medication Summary

Monoclonal Antibodies