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Retrospective Analysis of 25 Patients

Anu Jacob, MD, MRCP, DM; Brian G. Weinshenker, MD; Ivo Violich, BS; Nancy McLinskey, MD; Lauren Krupp, MD; Robert J. Fox, MD; Dean M. Wingerchuk, MD; Mike Boggild, MD, MRCP; Cris S. Constantinescu, PhD; Aaron Miller, MD; Tracy De Angelis, MD; Marcelo Matiello, MD; Bruce A. C. Cree, MD, PhD, MCR

Arch Neurol. 2008;65(11):(doi:10.1001/archneur.65.11.noc80069).

Background  Neuromyelitis optica (NMO) is an uncommon, life-threatening inflammatory demyelinating disorder. Recently, much has become known about its immunopathogenesis. However, optimal treatments, with expected outcomes, have not been established.

Objective  To evaluate the use and efficacy of rituximab for treating NMO.

Design  Retrospective multicenter case series of NMO patients treated with rituximab.

Setting  Seven tertiary medical centers in the United States and England.

Patients  Twenty-five patients (including 2 children), 23 of whom experienced relapses despite use of other drugs before rituximab. Extended follow-up of 7 previously reported patients is included.

Interventions  Infusions of rituximab at median intervals of 8 months.

Main Outcome Measures  Annualized relapse rate and disability (expressed as Expanded Disability Status Scale score).

Results  At a median follow-up of 19 months, the median annualized posttreatment relapse rate was lower than the pretreatment rate (0 [range 0-3.2] vs 1.7 [range, 0.5-5] relapses, P < .001). Disability improved or stabilized in 20 of 25 patients (80%, P = .02). Two patients died during the follow-up period, 1 owing to a brainstem relapse and 1 owing to suspected septicemia. Infections were reported in 20% of patients.

Conclusions  In NMO, treatment with rituximab appears to reduce the frequency of attacks, with subsequent stabilization or improvement in disability.

Author Affiliations: Mayo Clinic, Rochester, Minnesota (Drs Jacob, Weinshenker, and Matiello); The Walton Centre, Liverpool, England (Drs Jacob and Boggild); University of California–San Francisco, San Francisco (Mr Violich and Dr Cree); State University of New York, Stony Brook (Drs McLinskey and Krupp); Mellen Center, Cleveland Clinic, Cleveland, Ohio (Dr Fox); Mayo Clinic, Scottsdale, Arizona (Dr Wingerchuk); Queens' Medical Centre, Nottingham, England (Dr Constantinescu); and Mount Sinai Hospital, New York, New York (Drs Miller and De Angelis).

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