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KMID : 0391520160240040223
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Journal of the Korean Child Neurology Society
2016 Volume.24 No. 4 p.223 ~ p.231
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Childhood Onset Chronic Inflammatory Demyelinating Polyneuropathy: Treatment and Outcome
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Kim Woo-Joong
Choi Sun-Ah
Kim Soo-Yeon
Kim Hun-Min
Lim Byung-Chan
Hwang Hee
Choi Ji-Eun
Kim Ki-Joong
Chae Jong-Hee
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Abstract
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Purpose: To investigate the clinical features of childhood chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) as well as the treatment responses and outcomes.
Methods: We retrospectively analyzed clinical features and treatment outcomes of 10 cases of childhood CIDP followed up for more than a year. Intravenous immunoglobulin (IVIG), IV steroid and plasmapheresis was considered as the first line therapy and used according to patient’s response. Immunosuppresant agent was added when patients did not respond to the first line therapy.
Results: 10 CIDP cases (4 male and 6 female) were enrolled, and the median age of onset was 8 years (range 3-15 years). The mean duration of follow up was 46.2 months (range 12-82 months). Our patients manifested more frequently with subacute onset (n=8, 80%) and polyphasic course (n=6, 60%). All ten patients had lower extremity weakness at presentation (100%), six patients complained of upper extremity weakness (60%), and three patients reported sensory changes (30%). In the monophasic course (n=4), plasmapheresis showed a better treatment response (good 50%, partial 50%) compared to IVIG (good 0%, partial 75%) and steroids (good 0%, partial 0%), especially in progressive phases. In the polyphasic course (n=6), IVIG (good 50%, partial 50%) and plasmapheresis (good 40%, partial 60%) showed similar treatment responses. Five patients were refractory to first line treatment and received immunosuppressants; four patients received cyclosporine and achieved successful disease control without significant side effects. The overall long-term outcomes were favorable, with eight patients (80%) showing minimal symptoms and no relapse within 6 months.
Conclusion: Our results suggest that early administration of plasmapheresis in progressive monophasic course and cyclosporine in refractory polyphasic course may be effective in childhood CIDP.
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KEYWORD
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Childhood CIDP, Refractory CIDP, Treatment outcome, Plasmapheresis, Cyclosporine
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