From the Guidelines
Papilledema in neuromyelitis optica (NMO) patients should be treated promptly with high-dose intravenous methylprednisolone, followed by oral prednisone taper, and consideration of immunosuppressive medications like rituximab for long-term management, as evidenced by a case vignette of a 35-year-old woman with bilateral acute optic neuritis and papilledema who stabilized with rituximab 1.
Key Considerations
- Papilledema in NMO can result from inflammatory processes affecting the optic nerve or brain lesions causing increased intracranial pressure.
- Treatment typically involves high-dose intravenous methylprednisolone (1000 mg daily for 3-5 days) followed by oral prednisone taper (starting at 1 mg/kg/day), as seen in the management of acute optic neuritis and other inflammatory conditions.
- For refractory cases, plasma exchange (5-7 exchanges over 10-14 days) may be necessary, considering the patient's response to initial steroid treatment.
- Long-term management includes immunosuppressive medications such as rituximab (375 mg/m² weekly for 4 weeks or 1000 mg given twice, two weeks apart), which has been shown to stabilize symptoms in patients with recurrent optic neuritis and myelitis, as in Example 1 from the study 1.
Monitoring and Follow-Up
- Regular ophthalmologic monitoring with visual field testing, optical coherence tomography, and fundoscopic examinations is crucial to track disease progression and treatment response.
- The goal of treatment is to reduce inflammation, prevent further optic nerve damage, and preserve vision, highlighting the importance of prompt and aggressive management of papilledema in NMO patients, as suggested by the clinical presentation and treatment outcomes in the provided case vignettes 1.
From the Research
Papilledema in NMO
- Papilledema is not directly mentioned in the provided studies as a specific symptom or complication of Neuromyelitis Optica (NMO) or Neuromyelitis Optica Spectrum Disorder (NMOSD) 2, 3, 4, 5, 6.
- However, optic neuritis (ON) is a common symptom of NMO and NMOSD, which can cause visual impairment and potentially lead to papilledema 2, 5.
- The studies focus on the treatment and management of NMO and NMOSD, including the use of intravenous methylprednisolone (IVMP) and plasma exchange (PLEX) to reduce relapse frequency and severity 3, 4, 5, 6.
- The effects of IVMP on inflammatory and resolution factors in NMOSD patients are also discussed, highlighting the potential benefits of early treatment with IVMP to preserve visual function and reduce inflammation 6.
Treatment and Management
- IVMP is a recommended initial treatment option for acute onset NMOSD, and its therapeutic mechanism involves reducing the expression of pro-inflammatory factors and increasing the resolution of inflammation 6.
- PLEX is used as an add-on treatment for patients who do not respond to IVMP alone, and early initiation of PLEX may be beneficial for preserving visual function 4.
- Other treatments, such as satralizumab, inebilizumab, and eculizumab, have shown significant relapse reduction in AQP4-ab-positive patients with NMOSD 2.
Visual Outcomes
- Rapid initiation of high-dose IVMP pulse therapy is essential to preserve eventual visual acuity in patients with serum AQP4-IgG-positive NMOSD 5.
- The duration from optic neuritis onset to the initiation of high-dose IVMP pulse therapy and the lesion length on orbital MRI are correlated with the eventual visual prognosis 5.