Neuromyelitis optica and NMO-IgG in European pediatric patients.
OBJECTIVE
Neuromyelitis optica (NMO) is currently considered a severe relapsing CNS demyelinating disorder that is associated with aquaporin-4 immunoglobulin G (NMO-IgG) while in earlier reports of NMO in childhood it has been described as a benign and monophasic disorder. This study was performed to analyze the prevalence and the clinical course of NMO in a European pediatric cohort of patients with demyelinating CNS disorders.


METHODS
A cohort study was performed evaluating 118 pediatric patients presenting at the Center for Multiple Sclerosis in Childhood and Adolescents, Göttingen, Germany, with demyelinating CNS disorders between 2000 and 2009. In all patients, NMO-IgG status was determined.


RESULTS
The majority of patients (94%) were diagnosed with remitting recurrent multiple sclerosis. Six patients fulfilled the clinical criteria for NMO but only 1 was seropositive for NMO-IgG. This patient had a severe relapsing course in contrast to the seronegative patients who showed a mild and in the majority of cases monophasic course.


CONCLUSIONS
The diagnostic criteria clearly distinguished the patients with NMO from patients with other demyelinating CNS disorders. In the European pediatric population, NMO is very rare and in the majority of patients not associated with NMO-IgG. These seronegative cases have a benign and predominantly monophasic course and therefore do not need the immunosuppressant therapy that is recommended for NMO in the recent literature.
Department of Pediatrics and Pediatric Neurology, Georg August University, Faculty of Medicine, Robert-Koch-Strasse 40, D-37075 Göttingen, Germany. phuppke@med.uni-goettingen.de
P
P
Huppke
M
M
Blüthner
O
O
Bauer
W
W
Stark
K
K
Reinhardt
B
B
Huppke
J
J
Gärtner
D000293:Adolescent; D000367:Age Factors; D051401:Aquaporin 4 / Q000097:blood; D002648:Child; D015331:Cohort Studies; D005260:Female; D005500:Follow-Up Studies; D005858:Germany / Q000453:epidemiology; D006801:Humans; D007074:Immunoglobulin G / Q000096:biosynthesis* / Q000097:blood; D008297:Male; D009471:Neuromyelitis Optica / Q000175:diagnosis* / Q000453:epidemiology* / Q000276:immunology; D012008:Recurrence; D044465:White People
C496030:AQP4 protein, human; D051401:Aquaporin 4; D007074:Immunoglobulin G