Clinical characteristics and treatment outcome of childhood onset chronic inflammatory demyelinating polyneuropathy

Abstract

Introduction: Chronic inflammatory demyelinating polyneuropathy (CIDP) is a clinically heterogeneous group of sensory and motor peripheral neuropathy, presumed to occur due to immune related reactions. Because childhood CIDP is very rare, there are very few published studies to help clinicians in treating refractory cases. Aim of this study is to investigate the clinical features and treatment outcome of childhood CIDP. Methods: Clinical features and treatment outcomes of 14 cases of childhood CIDP (mean age = 8.6 ± 3.8years old) followed up for more than a year (mean duration = 47.7±29.6 months)were analyzed. Patients were initially treated with either intravenous immunoglobulin (IVIG) (78.6%) or steroids (21.4%). Plasmapheresis was considered when both treatments were proven ineffective. Results: In contrast to adult CIDP, which commonly showed insidious onset with monophasic courses, patients from this study manifested more frequently with subacute onset (n=10, 71.4%) and polyphasic course (n=8, 57.1%). In the monophasic group (n=6, 42.9%), plasmapheresis (n=5) showed a better treatment response (good 80%, partial 20%, none 0%) compared to IVIG (n=6) (good 0%, partial 50%, none 50%) and steroids (n=5) (good 0%, partial 40%, none 60%), especially in progressive phases. In the polyphasic group (n=8), IVIG (n=8) (good 50%, partial 37.5%, none 12.5%) and plasmapheresis (n=4) (good 0%, partial 100%, none 0%) showed comparable treatment responses. Six polyphasic patients (75%) were refractory to first line treatment and received immunosuppressant

All four patients who received cyclosporine achieved significant disease control. The overall long-term outcomes were favorable, with 6 patients (42.8%) showing minimal symptoms and no relapse within 6 months. Conclusions: This study results suggest that administration of plasmapheresis in progressive monophasic course and cyclosporine in refractory polyphasic course may be effective in childhood CIDP.