Relapsing demyelinating CNS disease in a Korean pediatric population: multiple sclerosis versus neuromyelitis optica.
OBJECTIVE
Our objective was to characterize the clinical and radiologic features of Korean pediatric patients with relapsing central nervous system (CNS) demyelination disease.


METHODS
Twenty-one patients with relapsing CNS demyelinating events were classified as having multiple sclerosis (MS, 18 patients) or neuromyelitis optica (NMO, three patients) according to the international consensus definitions. Retrospective analysis of clinical and radiologic features was conducted. Anti-aquaporin-4 antibody (AQP4 Ab) test was performed in six patients (including three NMO patients) who showed selective involvement of optic nerve and spinal cord.


RESULTS
Median age at the initial episode in patients with MS was 7.0 years (range, 4.4-13.6 years). Three of 18 MS patients (3/18, 17%) showed selective involvement of the optic nerve and spinal cord during the clinical course. Five patients (31%) at the initial episode and nine patients (50%) at relapse met the McDonald magnetic resonance imaging criteria for dissemination in space. Oligoclonal bands detected with a silver staining method were positive in only one patient of 16 patients tested. Two NMO patients positive for AQP4 Ab showed frequent relapses and early disabilities that were unresponsive to interferon treatment.


CONCLUSIONS
We conclude that Korean pediatric patients with relapsing CNS demyelination disease were characterized by preferential involvement of the optic nerve or spinal cord. The AQP4 Ab test seems to be useful for predicting clinical courses in the setting of heterogeneous opticospinal presentations.
Lim
Byung Chan
BC
Department of Pediatrics, Seoul National University Children's Hospital, Seoul, Korea.
Hwang
Hee
H
Kim
Ki Joong
KJ
Hwang
Yong Seung
YS
Cheon
Jung-Eun
JE
Kim
In-One
IO
Kim
Ho Jin
HJ
Chae
Jong-Hee
JH
D000293:Adolescent; D051401:Aquaporin 4 / Q000276:immunology; D044466:Asian People / Q000706:statistics & numerical data*; D001323:Autoantibodies / Q000097:blood; D015415:Biomarkers / Q000097:blood / Q000134:cerebrospinal fluid; D002648:Child; D002675:Child, Preschool; D038524:Diffusion Magnetic Resonance Imaging; D005260:Female; D006801:Humans; D007155:Immunologic Factors / Q000627:therapeutic use; D000068576:Interferon beta-1b; D016899:Interferon-beta / Q000627:therapeutic use; D008297:Male; D009103:Multiple Sclerosis / Q000175:diagnosis / Q000188:drug therapy / Q000208:ethnology*; D009471:Neuromyelitis Optica / Q000175:diagnosis / Q000188:drug therapy / Q000208:ethnology*; D043025:Oligoclonal Bands / Q000134:cerebrospinal fluid; D011237:Predictive Value of Tests; D056910:Republic of Korea / Q000453:epidemiology; D012189:Retrospective Studies; D016896:Treatment Outcome
C496030:AQP4 protein, human; D051401:Aquaporin 4; D001323:Autoantibodies; D015415:Biomarkers; D007155:Immunologic Factors; D043025:Oligoclonal Bands; D000068576:Interferon beta-1b; D016899:Interferon-beta