Inebilizuma treatment for acute serum AQP4 - IgG neuromyelitis optica: Case report.

RATIONALE: Neuromyelitis optica spectrum disorder (NMOSD) is a relapsing-remitting, chronic progressive inflammatory demyelinating disease of the central nervous system, predominantly affecting the optic nerves and spinal cord. Currently, there is no effective cure, and the overall prognosis is poor. Notably, the aquaporin-4 immunoglobulin G (AQP4-IgG) seropositive subtype (historically termed neuromyelitis optica) is associated with a significantly worse prognosis. PATIENT CONCERNS: Findings included left optic disc edema, prolonged P100 wave latencies and reduced amplitudes on visual evoked potentials (VEP) bilaterally, and concentric constriction of the left visual field. Fundus fluorescein angiography of the left eye revealed hyperfluorescence of the optic nerve. Contrast-enhanced magnetic resonance imaging demonstrated patchy and linear enhancement in the left optic nerve and linear enhancement in the right optic nerve (serum AQP4-IgG positivity confirmed by a tertiary hospital). DIAGNOSES: The patient has been diagnosed with AQP4-IgG seropositive NMOSD. INTERVENTIONS: The regimen consisted of intravenous methylprednisolone pulse 1000 mg daily for 5 consecutive days, combined with a single intravenous infusion of inebilizumab (brand name: Uplizna; MedImmune Pharma B.V.; specification: 100 mg/vial) at a dose of 300 mg. OUTCOMES: At the 1-month follow-up post-treatment, the patient's left eye visual acuity recovered to 1.0. Fundus examination revealed normal retinal and macular structure bilaterally with resolved optic disc edema. Right eye VEP showed normalized P100 latency and amplitude. Left eye VEP still exhibited prolonged P100 latency and subnormal amplitude. Visual field testing results were normal in both eyes. LESSONS: This case demonstrates that combination therapy with IVMP and inebilizumab during the acute phase significantly promoted visual recovery in this AQP4-IgG seropositive NMOSD patient. This finding enhances our understanding of NMOSD management. The case offers valuable clinical insights for therapeutic strategies in NMOSD. Future studies with larger cohorts are warranted to further evaluate the efficacy of the intravenous methylprednisolone pulse-inebilizumab combination in reducing relapse rates and improving long-term neurological outcomes in NMOSD.