High-risk syndrome for neuromyelitis optica: a descriptive and comparative study

• Published in: Multiple sclerosis Vol. 17; no. 6; pp. 720 - 724
• Main Authors: Collongues, N, Marignier, R, Zéphir, H, Blanc, F, Vukusic, S, Outteryck, O, Fleury, M, Ruet, A, Borgel, F, Thouvenot, E, Moreau, T, Defer, G, Derache, N, Pelletier, J, Audoin, B, Debouverie, M, Labauge, P, Gout, O, Camu, W, Brassat, D, Brochet, B, Vermersch, P, Confavreux, C, Seze, J de
• Format: Journal Article
• Language: English
• Published: London, England SAGE Publications 01.06.2011; Sage Publications; Sage Publications Ltd
• Subjects: Adolescent; Adult; Age of Onset; Aged; Biological and medical sciences; Brain - pathology; Brain - physiopathology; Child; Degenerative and inherited degenerative diseases of the nervous system. Leukodystrophies. Prion diseases; Disability Evaluation; Disease Progression; Evoked Potentials, Visual; Female; France; Humans; Kaplan-Meier Estimate; Magnetic Resonance Imaging; Male; Malformations of the nervous system; Medical sciences; Middle Aged; Myelitis - diagnosis; Myelitis - mortality; Myelitis - pathology; Myelitis - physiopathology; Neurology; Neuromyelitis Optica - diagnosis; Neuromyelitis Optica - mortality; Neuromyelitis Optica - pathology; Neuromyelitis Optica - physiopathology; Optic Neuritis - diagnosis; Optic Neuritis - mortality; Optic Neuritis - pathology; Optic Neuritis - physiopathology; Predictive Value of Tests; Recurrence; Retrospective Studies; Risk Assessment; Risk Factors; Sex Factors; Spinal Cord - pathology; Syndrome; Time Factors; Young Adult; France; Devic’s syndrome; high-risk syndrome; transverse myelitis; Cohort study; NMO-IgG; optic neuritis; High risk; Nervous system diseases; Multiple sclerosis; Optic nerve; Optic neuritis; IgG; Neurooptic myelitis; Inflammatory disease; Transverse myelitis; Eye disease; Cranial nerve disease; Visual pathway; Optic nevritis; Devic's syndrome; Central nervous system disease; Degenerative disease; Spinal cord disease; Comparative study
• ISSN: 1352-4585; 1477-0970
• DOI: 10.1177/1352458510396923

Background: Neuromyelitis optica (NMO) frequently begins with a monofocal episode of optic neuritis or myelitis. A concept named high-risk syndrome (HRS) for NMO has been proposed for patients with monofocal episodes and NMO-IgG antibodies. Objective: To describe HRS patients and compare them with NMO patients. Methods: We identified 30 patients with HRS: 18 with extensive myelitis (HRM) and 12 with optic neuritis (HRON), in a database pooling patients from 25 centres in France. Clinical, laboratory/magnetic resonance imaging (MRI) data and outcome were analysed and compared with a national cohort of 125 NMO patients extracted from the same database. Results: Mean follow-up was 4.8 years. Mean age at onset was 42.8 years (range: 12.4–70) with a female:male ratio of 0.9. Asymptomatic lesions were report on visual evoked potentials in 4/8 tested HRM patients and on spinal cord MRI in 2/7 HRON patients. Three patients died, two owing to a cervical lesion. HRS and NMO patients had similar clinical/paraclinical data, except for a predominance of men in the HRS group and a later mean age at onset in the HRM subgroup. Conclusion: The description of HRS patients is compatible with a monofocal form of NMO. Asymptomatic lesions could be included in a new set of NMO diagnostic criteria.