Unusual presentation of pediatric chronic inflammatory demyelinating polyneuropathy

Abstract

Chronic inflammatory demyelinating polyneuropathy is an immune mediated disorder of more than 2 mo duration. A 5-year-old male child presented with subacute onset of ascending weakness, hyporeflexia and respiratory insufficiency requiring mechanical ventilation. He had suffered from an exactly similar episode 1 yr back. Cerebrospinal fluid showed albuminocytological dissociation. Nerve conduction velocity study showed combined demyelinating and axonal type of polyradiculoneuropathy. Sural nerve biopsy revealed demyelination and axonal degeneration. The child responded well to intravenous immunoglobulin in the acute phase and prednisolone was used to maintain the child in remission.

Keywords

Chronic inflammatory demyelinating polyradiculoneuropathy - Guillian-Barre syndrome - demyelination - intravenous immunoglobulin