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Neuromyelitis optica spectrum disorder
Guidelines
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 2023), the American College of Radiology (ACR 2021), the Latin American Consensus Group on Neuromyelitis Optica Spectrum Disorder (LACG-NMOSD 2020), the French Society ...
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Screening and diagnosis
Classification and risk stratification
Prognosis
As per LACG-NMOSD 2020 guidelines:
Recognize that the presence of anti-AQP-4 antibodies at disease onset in patients with NMOSD predicts worse medium/long-term disability.
E
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).
E
Diagnostic investigations
AQP-4 antibodies: as per LACG-NMOSD 2020 guidelines, obtain testing for serum anti-AQP-4 antibodies in patients with suspected NMOSD.
E
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Myelin oligodendrocyte glycoprotein antibodies
MRI
Pretreatment evaluation
Diagnostic procedures
CSF 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.
E
Do not rule out the diagnosis of NMOSD in the presence of oligoclonal bands in CSF.
D
Medical management
Setting of care: as per LACG-NMOSD 2020 guidelines, 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.
E
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First-line therapy
Second-line therapy
Considerations for rituximab therapy
Specific circumstances
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.
C
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Anti-infective prophylaxis
Follow-up and surveillance
Assessment of treatment response: as per LACG-NMOSD 2020 guidelines, 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/mcL) and an MCV increase of at least 5 points from the baseline.
E
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Management of relapse