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The early and accurate identification of neuromyelitis optica spectrum disorder is integral to the prognosis and reduction of relapses. Treatment and management strategies include immunosuppressive agents, steroids, and novel monoclonal antibodies.

Neuromyelitis optica spectrum disorder (NMOSD) is an autoimmune disorder mainly involving the optic nerve (optic neuritis) and the spinal cord (myelitis). Prompt and early diagnosis of NMOSD is integral to effectively treating this disorder and preventing further attacks. 

This study, published in the Journal for Nurse Practitioners, describes the case of a previously healthy female suffering from NMOSD. It concludes that nurse practitioners are integral to identifying and differentiating presenting symptoms of NMOSD patients to avoid complications stemming from delayed care. The study also describes the role of monoclonal antibodies in treating NMOSD.  

Epidemiology and Etiology

Previously, NMOSD was considered a form of multiple sclerosis (MS), and no definitive etiological factors are associated with NMOSD. According to an epidemiological analysis, NMOSD prevalence is estimated to be 0.3–4.4 per 100,000. The disorder is more common among Asian and African populations, with a greater predisposition among females.

Pathogenesis and Clinical Features 

NMOSD is now considered an autoimmune astrocytopathy disorder rather than a demyelinating disorder and is associated with the synthesis of immunoglobulin G (IgG) antibodies directed against the aquaporin 4 (AQP4) water channel found on the cell membrane in optic nerves, the spinal cord, and the brain. The main clinical features of NMOSD include optic neuritis and transverse myelitis. 

In cases presenting exclusively with optic neuritis or transverse myelitis, the diagnosis is relatively difficult. The optic neuritis attacks are initially unilateral but may become bilateral with the progression of the disease. Transverse myelitis is associated with sudden onset visual impairment, bladder dysfunction, quadriparesis, and paraparesis. Clinical decisionmaking concerning diagnosis and treatment remains challenging in the presence of atypical symptoms, such as area postrema syndrome, which involves intractable hiccups, nausea, and vomiting.  

Diagnosis, Treatment, and Management 

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A revised diagnostic criterion is established for NMOSD patients with and without AQP4-IgG. The standard management strategy for NMOSD patients is the treatment of acute episodes and the prevention of relapses. Acute-phase treatment and long-term management are mainstays of NMOSD management, employing immunosuppressive agents. 

Acute treatment involves the administration of high-dose steroids, including methylprednisolone, with subsequent plasmapheresis. Following intravenous steroids, the patient is administered oral prednisolone. Failure to provide treatment contributes to increased morbidity risk. The traditional treatment includes immunosuppressants and low-dose corticosteroids. New emergent strategies include monoclonal antibodies, which effectively mitigate the risk of relapse. Approved monoclonal antibodies include inebilizumab, eculizumab, and satralizumab.

For effective treatment and prevention of future attacks, early diagnosis of NMOSD is crucial.Using monoclonal antibodies, such as inebilizumab, eculizumab, and satralizumab, to treat NMOSD and reduce the risk of relapse is promising.

Source

George, M., & Gantioque, R. (2023). Neuromyelitis Optica Spectrum Disorder: An Early Diagnosis to Prevent Blindness and Paraplegia. The Journal for Nurse Practitioners, 19(7), 104654. https://doi.org/10.1016/j.nurpra.2023.104654