A review of cases of neuromyelitis optica.

ABSTRACT

BACKGROUND: Under the therapeutic point of view, neuromyelitis optica (NMO) poses major challenges. Patients with NMO manifest severe disability from recurrent demyelinating lesions and the therapies are only partially effective. We performed a retrospective analysis of the records of patients followed at our institution and provide suggestions for management of acute relapses and preventive therapy. METHODS: We searched the electronic database for patients who met criteria for NMO spectrum between January 2003 and June 2009. Patient characteristics, clinical relapses, treatments, neurological status, and medical complications were recorded. RESULTS: In the 18 patients who met the criteria for NMO different regimens of chemotherapies seemed to be modestly effective in preventing clinical relapses. After the year 2006, when rituximab began to be used for NMO patients at our institution, a significant reduction of the relapse rate was observed. After the administration of rituximab, we have systematically been monitoring the percentage of the circulating B cells and we suggest that the clinical relapses occurring while on rituximab therapy correlate with the reconstitution of circulating B cells. CONCLUSIONS: The lack of response to therapies approved for multiple sclerosis demands prompt recognition of NMO patients and the NMO-antibody testing can be critically important for that purpose. We have observed remarkable variability of the disease course with long-lasting relapse-free intervals and clusters of severe, disabling attacks. The best effects in preventing and interrupting the high frequency of relapses is achieved with rituximab whose repeated dosing should be guided by monitoring the circulating B-cell counts.