Clinical features and long-term outcome of a group of Japanese children with inflammatory central nervous system disorders and seropositivity to myelin-oligodendrocyte glycoprotein antibodies.
BACKGROUND
Myelin-oligodendrocyte glycoprotein and aquaporin-4 have been extensively analyzed as targets for humoral immune reactions in central nervous system (CNS) demyelinating diseases, and the results indicated a possible role of these antibodies in the pathogenesis of various demyelinating diseases.


OBJECTIVE
To investigate the antibody titer levels against myelin-oligodendrocyte glycoprotein and aquaporin-4 in pediatric patients with inflammatory CNS disorders, and to evaluate clinical significance to study anti-myelin-oligodendrocyte glycoprotein antibodies.


METHODS
Sera at onset from patients with acute disseminated encephalomyelitis (ADEM) in 7, optic neuritis (ON) in 5, pediatric MS in 4 and neuromyelitis optica in one were tested for myelin-oligodendrocyte glycoprotein and aquaporin-4 antibodies using cell-based assays with live transfected cells. The duration of the observation periods ranged from 1 to 21 years (median, 10 years). We also described clinical course of patients with positive anti-myelin-oligodendrocyte glycoprotein antibodies.


RESULTS
Among 17 patients diagnosed with inflammatory CNS demyelinating diseases nine (52%) were positive to anti-myelin-oligodendrocyte glycoprotein antibodies. Of note, all cases with positive anti-myelin-oligodendrocyte glycoprotein antibodies showed seronegativity against anti-aquaporin-4 antibodies and had a favorable prognosis.


CONCLUSIONS
This preliminary report showed that anti-myelin-oligodendrocyte glycoprotein antibodies testing at onset could be a useful tool predicting clinical outcome of children with ADEM, ON, and MS.
Department of Pediatrics, Tohoku University School of Medicine, Sendai, Japan. Electronic address: naomi-h@zc4.so-net.ne.jp.
Naomi
N
Hino-Fukuyo
Department of Pediatrics, Tohoku University School of Medicine, Sendai, Japan.
Kazuhiro
K
Haginoya
Department of Neurology, Tohoku University of School of Medicine, Sendai, Japan.
Ichiro
I
Nakashima
Department of Neurology, Tohoku University of School of Medicine, Sendai, Japan.
Douglas Kazutoshi
DK
Sato
Department of Neurology, Tohoku University of School of Medicine, Sendai, Japan.
Toshiyuki
T
Takahashi
Department of Neurology, Tohoku University of School of Medicine, Sendai, Japan.
Tatsuro
T
Misu
Department of Neurology, Tohoku University of School of Medicine, Sendai, Japan.
Kazuo
K
Fujihara
Department of Pediatrics, Tohoku University School of Medicine, Sendai, Japan.
Mieko
M
Hirose
Department of Pediatrics, Tohoku University School of Medicine, Sendai, Japan.
Yosuke
Y
Kakisaka
Department of Pediatrics, Tohoku University School of Medicine, Sendai, Japan.
Mitsugu
M
Uematsu
Department of Pediatrics, Tohoku University School of Medicine, Sendai, Japan.
Tomoko
T
Kobayashi
Department of Pediatrics, Tohoku University School of Medicine, Sendai, Japan.
Shigeo
S
Kure
D000293:Adolescent; D000906:Antibodies / Q000276:immunology*; D051401:Aquaporin 4 / Q000276:immunology*; D002648:Child; D002675:Child, Preschool; D004673:Encephalomyelitis, Acute Disseminated / Q000175:diagnosis / Q000276:immunology*; D005260:Female; D006801:Humans; D007223:Infant; D007564:Japan; D008297:Male; D009103:Multiple Sclerosis / Q000175:diagnosis / Q000276:immunology*; D063308:Myelin-Oligodendrocyte Glycoprotein / Q000276:immunology*; D009471:Neuromyelitis Optica / Q000175:diagnosis / Q000276:immunology*
C496030:AQP4 protein, human; D000906:Antibodies; D051401:Aquaporin 4; D063308:Myelin-Oligodendrocyte Glycoprotein
Acute disseminated encephalomyelitis; Multiple sclerosis; Myelin-oligodendrocyte glycoprotein; Neuromyelitis optica; Optic neuritis