A Benign Form of Neuromyelitis Optica
Does It Exist?
Nicolas Collongues, MD; Philippe Cabre, MD, PhD; Romain Marignier, MD; H?l?ne Z?phir, MD; Caroline Papeix, MD; Bertrand Audoin, MD, PhD; Christine Lebrun-Frenay, MD; Jean Pelletier, MD, PhD; Bertrand Fontaine, MD, PhD; Patrick Vermersch, MD, PhD; Christian Confavreux, MD, PhD; J?r?me de Seze, MD, PhD; Group Members for NOMADMUS and CF-SEP
Arch Neurol.?2011;68(7):918-924. doi:10.1001/archneurol.2011.127
Background? Few data exist on a possible benign form ofneuromyelitis optica (NMO).
Objectives? To identify NMO with a good outcome (go-NMO)among a large population of patients and to describe demographicand clinical variables associated with go-NMO vs standard NMOand benign multiple sclerosis.
Design? Observational retrospective multicenter study.
Setting? Twenty-five medical centers in metropolitan France(MF) and 3 medical centers in the French West Indies (FWI).
Patients? A total of 175 patients with NMO were retrospectivelyanalyzed from 2 cohorts: 125 in MF and 50 patients of nonwhiterace/ethnicity in the FWI. Patients in MF fulfilled the 2006NMO criteria, whereas patients in the FWI fulfilled the 1999or 2006 NMO criteria. Neuromyelitis optica and multiple sclerosisdatabases were reviewed, and patients with a score of 3 or loweron the Expanded Disability Status Scale after a 10-year follow-upperiod were considered to have go-NMO.
Main Outcome Measures? Clinical, laboratory, and magneticresonance imaging data and course of disability.
Results? In MF, go-NMO was observed in 11 patients, including3 untreated patients. In the FWI, NMO was severe because ofdisability related to optic neuritis. Compared with standardNMO, go-NMO was associated with a lower annualized relapse rate(0.3 vs 1.0, P?< ?.01), and 8 of 11 patients with go-NMO showed complete regression of myelitis on magnetic resonanceimaging during the disease course. Three patients experienceda disabling attack of NMO after 15 years of follow-up. A goodoutcome occurred less frequently among patients with NMO thanamong patients with multiple sclerosis (12.0% vs 22.4%, P?=?.03).
Conclusions? Among patients in MF, go-NMO occurs rarely.However, because a disabling attack may occur after a long follow-upperiod, a benign form of NMO cannot be defined.