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


CONCLUSIONS
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.
Department of Neurology, University Hospital of Strasbourg, 1 Place de l’Hôpital, Strasbourg, France. nicolas.collongues@chru-Strasbourg.fr
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Collongues
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Marignier
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Zéphir
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Blanc
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Vukusic
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Outteryck
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Fleury
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Ruet
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Borgel
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Thouvenot
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Moreau
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Defer
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N
Derache
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Pelletier
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B
Audoin
M
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Debouverie
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P
Labauge
O
O
Gout
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Camu
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Brassat
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Brochet
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Vermersch
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Confavreux
J
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de Seze
D000293:Adolescent; D000328:Adult; D017668:Age of Onset; D000368:Aged; D001921:Brain / Q000473:pathology / Q000503:physiopathology; D002648:Child; D004185:Disability Evaluation; D018450:Disease Progression; D005074:Evoked Potentials, Visual; D005260:Female; D005602:France; D006801:Humans; D053208:Kaplan-Meier Estimate; D008279:Magnetic Resonance Imaging; D008297:Male; D008875:Middle Aged; D009187:Myelitis / Q000175:diagnosis* / Q000401:mortality / Q000473:pathology / Q000503:physiopathology; D009471:Neuromyelitis Optica / Q000175:diagnosis* / Q000401:mortality / Q000473:pathology / Q000503:physiopathology; D009902:Optic Neuritis / Q000175:diagnosis* / Q000401:mortality / Q000473:pathology / Q000503:physiopathology; D011237:Predictive Value of Tests; D012008:Recurrence; D012189:Retrospective Studies; D018570:Risk Assessment; D012307:Risk Factors; D012737:Sex Factors; D013116:Spinal Cord / Q000473:pathology; D013577:Syndrome; D013997:Time Factors; D055815:Young Adult