A benign form of neuromyelitis optica: does it exist?

BACKGROUND: Few data exist on a possible benign form of neuromyelitis optica (NMO). OBJECTIVES: To identify NMO with a good outcome (go-NMO) among a large population of patients and to describe demographic and clinical variables associated with go-NMO vs standard NMO and 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 retrospectively analyzed from 2 cohorts: 125 in MF and 50 patients of nonwhite race/ethnicity in the FWI. Patients in MF fulfilled the 2006 NMO criteria, whereas patients in the FWI fulfilled the 1999 or 2006 NMO criteria. Neuromyelitis optica and multiple sclerosis databases were reviewed, and patients with a score of 3 or lower on the Expanded Disability Status Scale after a 10-year follow-up period were considered to have go-NMO. MAIN OUTCOME MEASURES: Clinical, laboratory, and magnetic resonance imaging data and course of disability. RESULTS: In MF, go-NMO was observed in 11 patients, including 3 untreated patients. In the FWI, NMO was severe because of disability related to optic neuritis. Compared with standard NMO, 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 resonance imaging during the disease course. Three patients experienced a disabling attack of NMO after 15 years of follow-up. A good outcome occurred less frequently among patients with NMO than among 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-up period, a benign form of NMO cannot be defined.

First episode of acute CNS inflammatory demyelination in childhood: prognostic factors for multiple sclerosis and disability.

OBJECTIVES: To evaluate prognostic factors for second attack and for disability in children presenting with an initial episode of central nervous system (CNS) demyelination. STUDY DESIGN: A cohort of 296 children having a first episode of acute CNS inflammatory demyelination was studied by survival analysis. RESULTS: The average follow-up was 2.9+/-3 years. At the end of the follow-up, 57% of patients had a diagnosis of multiple sclerosis (MS), 29% had a monophasic acute disseminated encephalomyelitis, and 14% had a single focal episode. The rate of a second attack was (1). higher in patients with age at onset >or=10 years (hazard ratio, 1.67; 95% CI, 1.04-2.67), MS-suggestive initial MRI (1.54; 1.02-2.33), or optic nerve lesion (2.59; 1.27-5.29); and (2). lower in patients with myelitis (0.23; 0.10-0.56) or mental status change (0.59; 0.33-1.07). Of patients with a second attack, 29% had an initial diagnosis of acute disseminated encephalomyelitis. At the end of the follow-up period, 90% of patients had no or minor disability. Occurrence of severe disability was associated with a polysymptomatic onset (3.25; 1.16-11.01), sequelae after the first attack (26.65; 9.42-75.35), further relapses (1.49; 1.16-1.92), and progressive MS (3.57; 1.21-8.72). CONCLUSIONS: Risk of second attack of CNS demyelination is higher in older patients and lower in patients with mental status change. Risk of disability is higher in polysymptomatic and relapsing patients.