Original articleClinical features and long-term outcome of a group of Japanese children with inflammatory central nervous system disorders and seropositivity to myelin-oligodendrocyte glycoprotein antibodies
Introduction
Inflammatory demyelinating disorders of the central nervous system include a heterogeneous group of diseases such as multiple sclerosis (MS), acute disseminated encephalomyelitis (ADEM), isolated optic neuritis (ON), and neuromyelitis optica spectrum disorders (NMOSD). In clinical practice, the differential diagnosis between ADEM, isolated ON, and a clinically isolated syndrome representing the first MS attack is often challenging.
Myelin-oligodendrocyte glycoprotein (MOG) and aquaporin-4 (AQP4) have been extensively analyzed as targets for humoral immune reactions in central nervous system (CNS) demyelinating diseases, and the results suggested a possible role of these antibodies in the pathogenesis of various demyelinating diseases [1]. Interestingly, high titers of anti-MOG antibodies, assayed by cell-based assays, have been detected in a subgroup of pediatric diseases such as ADEM, MS, ON and NMOSD in different cohorts [2]. However, no study has been conducted in an Asian pediatric cohort with inflammatory demyelinating central nervous system diseases, and the follow-up period in the previous studies was not long enough to compare the long-term prognosis with typical adult MS or AQP4 antibody-positive NMOSD. In this study, we investigated anti-MOG and anti-AQP antibodies in a group of Japanese pediatric patients with inflammatory central nervous system disorders, and the final diagnosis as well as long-term prognosis was investigated retrospectively.
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Methods
We retrospectively analyzed stored sera from 17 consecutive patients (18 years old or younger) who had been diagnosed as having inflammatory CNS demyelinating diseases at Tohoku University Hospital from 1992 to 2007. Serum samples were obtained 4–51 days (median: 8 days) after onset and before therapy. The brain magnetic resonance imaging (MRI) was studied at the onset in all patients and spinal MRI was studied in 15 patients at onset. The other diseases with white matter lesions, such as
Results
Nine of 17 (52%) patients diagnosed with inflammatory CNS demyelinating diseases were positive for anti-MOG antibodies, among whom male predominance was evident (7 males and 2 females). A clinical summary of patients is shown in Table 1. Two patients were treated with interferon-beta (case 9 and 14). Three of eight patients diagnosed as ADEM, three of four patients diagnosed as isolated ON, three of four patients diagnosed as pediatric MS were positive for anti-MOG antibodies, while one patient
Discussion
This study presented good outcome and long-term prognosis of anti-MOG antibody associated diseases, although their clinical manifestations or symptoms are various among each patient as reported in the previous study [2], [6].
This study has several limitations. First, since we could not repeat the test of anti-MOG antibodies, changes in its titers over time were not evaluated. Second, the retrospective nature of this study and small sample size limited the systematic evaluation of MRI and
Acknowledgement
We thank Brent Bell for reading the manuscript.
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