Long-term follow-up of neuromyelitis optica with a pediatric onset.
BACKGROUND
Neuromyelitis optica (NMO) is a rare inflammatory disease. Average age at onset is 35 years. Few data exist on patients with pediatric-onset NMO (p-NMO), with disease onset before age 18 years. We report the clinical and paraclinical features and long-term outcome of patients with p-NMO and compare them with a large adult-onset NMO (a-NMO) cohort.


METHODS
We performed a retrospective, multicenter study of patients with p-NMO in pediatric and adult medical centers. We identified 125 patients with NMO (12 p-NMO; 113 a-NMO) fulfilling the 2006 criteria. Data were collected using hospital files and standardized assessment forms for NMO.


RESULTS
Patients with p-NMO were followed up during a mean 19.3 years. Median age at onset was 14.5 years (4.1-17.9) with a female:male ratio of 3:1. Three patients (25%) fulfilled Paty criteria for multiple sclerosis on first brain MRI, including one patient with acute disseminated encephalomyelitis. Median interval between onset and residual Expanded Disability Status Scale (EDSS) score 4 was 20.7 years, score 6 was 26 years, and score 7 was 28.7 years. Median interval between onset and residual visual loss ≤1/10 was 1.3 years. Compared with a-NMO, p-NMO showed a longer time to EDSS scores 4 and 6, largely explained by the severity of the first myelitis in the a-NMO group. Time to first treatment was longer in the p-NMO group (13.1 vs 3.4 years).


CONCLUSIONS
Patients with p-NMO can present a diffuse inflammatory process on first brain MRI and have a longer time to disability than patients with a-NMO.
Collongues
N
N
Department of Neurology, University Hospital of Strasbourg, 1, Place de l'Hôpital, 67091 Strasbourg, France. nicolas.collongues@chru-Strasbourg.fr
Marignier
R
R
Zéphir
H
H
Papeix
C
C
Fontaine
B
B
Blanc
F
F
Rodriguez
D
D
Fleury
M
M
Vukusic
S
S
Pelletier
J
J
Audoin
B
B
Thouvenot
E
E
Camu
W
W
Barroso
B
B
Ruet
A
A
Brochet
B
B
Vermersch
P
P
Confavreux
C
C
de Seze
J
J
D000293:Adolescent; D000328:Adult; D017668:Age of Onset; D002648:Child; D002675:Child, Preschool; D004185:Disability Evaluation; D018450:Disease Progression; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D007249:Inflammation / Q000473:pathology; D053208:Kaplan-Meier Estimate; D008279:Magnetic Resonance Imaging; D008297:Male; D009103:Multiple Sclerosis / Q000175:diagnosis*; D009471:Neuromyelitis Optica / Q000473:pathology*; D012189:Retrospective Studies; D018709:Statistics, Nonparametric