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Mult Scler. 2011 Jun;17(6):720-4. doi: 10.1177/1352458510396923. Epub 2011 Jan 14.

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

Author information

1
Department of Neurology, University Hospital of Strasbourg, 1 Place de l’Hôpital, Strasbourg, France. nicolas.collongues@chru-Strasbourg.fr

Abstract

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.

PMID:
21239412
DOI:
10.1177/1352458510396923
[Indexed for MEDLINE]
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