Very-Early-Onset Treatment-Refractory Pediatric Chronic Inflammatory Demyelinating Polyradiculoneuropathy Responsive to Multimodal Therapy

Abstract

Chronic inflammatory demyelinating polyradiculoneuropathy is a treatable pediatric polyneuropathy, with both a typical form and variants. The approach to treatment-refractory chronic inflammatory demyelinating polyradiculoneuropathy is not fully characterized, especially in children with very-early-onset chronic inflammatory demyelinating polyradiculoneuropathy, namely, those with symptom onset before 4 years of age. We herein report the case of a 30-month-old child with treatment-refractory, very-early-onset chronic inflammatory demyelinating polyradiculoneuropathy who failed to respond to initial intravenous immunoglobulin and then corticosteroid monotherapy. He was ultimately switched to combined pulsed intravenous methylprednisolone and plasma exchange, followed by induction and maintenance rituximab therapy. The patient exhibited a significant and sustained clinical response to this regimen.