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Neuromyelitis optica spectrum disorder

Key sources
The following summarized guidelines for the evaluation and management of neuromyelitis optica spectrum disorder are prepared by our editorial team based on guidelines from the American College of Rheumatology (ACR 2023), the European League Against Rheumatism (EULAR 2022), the American College of Radiology (ACR 2021), the Latin American Consensus Group on Neuromyelitis Optica Spectrum Disorder (LACG-NMOSD 2020), the French Society of Multiple Sclerosis (SFSEP/OFSEP/NOMADMUS 2018), the Neuromyelitis Optica Study Group (NEMOS 2013), and the European Federation of Neurological Societies (EFNS 2010).
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Guidelines

1.Screening and diagnosis

Diagnosis: as per LACG-NMOSD 2020 guidelines, diagnose NMOSD in the presence of clinical involvement in at least 1 of the 6 CNS regions: optic nerve, spinal cord, area postrema of the dorsal medulla, brainstem, diencephalon, or cerebrum.
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2.Classification and risk stratification

Prognosis
Recognize that the presence of anti-AQP-4 antibodies at disease onset in patients with NMOSD predicts worse medium/long-term disability.
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Recognize that the number of relapses and their severity in patients with NMOSD during the first 2 years predicts medium/long-term disability (5-10 years).
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3.Diagnostic investigations

Aquaporin-4 antibodies: as per LACG-NMOSD 2020 guidelines, obtain testing for serum anti-AQP-4 antibodies in patients with suspected NMOSD.
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  • Myelin oligodendrocyte glycoprotein antibodies

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4.Diagnostic procedures

Cerebrospinal fluid analysis
As per LACG-NMOSD 2020 guidelines:
Perform lumbar puncture with an evaluation of WBC count, protein levels, and oligoclonal bands in CSF to evaluate differential diagnoses in patients with clinically suspected NMOSD with non-typical brain or spinal cord MRI lesions suggestive of multiple sclerosis.
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Do not rule out the diagnosis of NMOSD in the presence of oligoclonal bands in CSF.
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5.Medical management

Setting of care: evaluate patients with suspected NMOSD in a center with experience in diagnosing demyelinating diseases, to ensure an earlier and more precise diagnosis and adequate treatment.
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  • First-line therapy

  • Second-line therapy

  • Considerations for rituximab therapy

6.Specific circumstances

Pregnant patients: as per LACG-NMOSD 2020 guidelines, continue immunosuppressive therapy with azathioprine or rituximab during pregnancy in patients with a history of NMOSD attacks within the past 3 years.
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7.Preventative measures

Routine immunizations: as per ACR 2023 guidelines, consider offering high-dose or adjuvanted influenza vaccination, rather than regular-dose influenza vaccination, in 18-65 years old patients on immunosuppressive medications.
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  • Anti-infective prophylaxis

8.Follow-up and surveillance

Assessment of treatment response: classify patients with NMOSD as having suboptimal treatment response when presenting with relapse after 6 months of therapy within 5 years of starting treatment with azathioprine at a target dose of 2.5-3.0 mg/kg/day adjusted to the total lymphocyte count (< 600-1, 000/µL) and an MCV increase of at least 5 points from the baseline.
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  • Management of relapse