Eculizumab may be an effective treatment for acute episodes of neuromyelitis optica spectrum disorder (NMOSD), according to a study recently published in Frontiers in Neurology.

“Given that disease burden and mortality in NMOSD is almost entirely related to relapses, increased use of eculizumab acutely could potentially aid recovery from an attack in very severe attacks, and therefore minimize immediate stepwise accrual of disability,” the authors wrote.

This case report describes a 46-year-old woman who presented with monocular vision loss of the left eye, painful ocular movements, and headache over 24 hours. Her medical history was consistent with NMOSD, which presented 15 years prior.

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The most recent optic neuritis episode had occurred 1 and a half years prior. The patient had previously tested positive for aquaporin-4 immunoglobulin G and had a previous diagnosis of area postrema syndrome. She had a history of traveling overseas, which impeded the proper management of some acute episodes, and at the time of evaluation, she was not taking any medication.

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Physical exam revealed impaired bilateral visual acuity. The left eye was the most affected, only able to perceive light, and it exhibited a relative afferent pupillary defect. The right eye had an acuity of 6/60 and showcased optic nerve head pallor on fundoscopy. Brain magnetic resonance imaging showed signs suggestive of demyelination, right optic nerve atrophy, and high signal with enhancement in the left optic nerve and chiasmal extension.

A diagnosis of an acute relapse of NMOSD was made, and methylprednisolone was administered via intravenous pulses of 1 g daily for 5 days. Nonetheless, the patient did not show improvement in visual acuity. She then underwent 2 cycles of plasmapheresis with no progress.

After12 days, she received 900 mg of eculizumab weekly for 4 weeks, followed by 1200 mg in the fifth week and then every 2 weeks thereafter. The patient demonstrated visual acuity improvement on the second day of eculizumab administration and was able to count fingers with the left eye. At 2 and 5 months later, she continued to improve without any treatment-derived complications.

Eculizumab, a long-acting humanized monoclonal antibody, is a therapeutic option for maintaining remission in patients with NMOSD. The role of this drug in acute episodes is not clear. Hence, these results translate into a potential novel use for these patients.

“The use of eculizumab acutely theoretically accords with the immediate mode of action in inhibiting conversion of C5–C5a/b, perhaps arresting the acute inflammatory process in this disease,” Chatterton and colleagues explained.


Chatterton S, Parratt JDE, Ng K. Eculizumab for acute relapse of neuromyelitis optica spectrum disorder: case report. Front Neurol. 2022;13:951423. doi:10.3389/fneur.2022.951423