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A benign form of neuromyelitis optica: does it exist?
Nicolas Collongues1, Philippe Cabre, Romain Marignier
1Hôpital Universitaire de Strasbourg, Service de Neurologie et Centre d'Investigation Clinique INSERM 1002, Strasbourg, France. nicolas.collongues@chru-Strasbourg.fr
Archives of Neurology
|July 13, 2011
Summary
Neuromyelitis optica with a good outcome (go-NMO) is rare and typically has fewer relapses. However, a disabling attack can occur late, meaning a truly benign form of NMO is not yet defined.
Area of Science:
- Neurology
- Neuroimmunology
- Clinical Neuroscience
Background:
- Limited data exist on benign forms of Neuromyelitis Optica (NMO).
- Identifying NMO with a good outcome (go-NMO) is crucial for understanding disease heterogeneity.
Purpose of the Study:
- To identify patients with go-NMO within a large cohort.
- To characterize demographic and clinical factors distinguishing go-NMO from standard NMO and benign multiple sclerosis.
Main Methods:
- Retrospective, multicenter observational study involving 175 NMO patients across France.
- Patients were classified as go-NMO if they had an Expanded Disability Status Scale score of ≤3 after 10 years.
- Data collected included clinical, laboratory, MRI, and disability progression.
Main Results:
- go-NMO was observed in 11 patients (8.8%) in metropolitan France, with 3 untreated.
- go-NMO cases showed a significantly lower annualized relapse rate (0.3 vs 1.0) compared to standard NMO.
- Despite a good outcome in most, 3 go-NMO patients experienced a disabling attack after 15 years; go-NMO was less frequent than in multiple sclerosis (12.0% vs 22.4%).
Conclusions:
- Neuromyelitis Optica with a good outcome (go-NMO) is rare in the French population.
- The possibility of late-onset disabling attacks precludes defining a truly benign form of NMO.
- Further research is needed to fully understand the long-term prognosis and potential for late relapses in NMO.
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