Clinical subtypes and characteristics of pediatric chronic inflammatory demyelinating polyneuropathy

Abstract

Objective:We investigated the clinical features of eight pediatric patients with chronic inflammatory demyelinating polyneuropathy （CIDP）to clarify the clinical window for early diagnosis. Methods:The subjects were eight patients who were diagnosed as having CIDP based on neuropathy progressing over two months, evidence of demyelination on nerve conduction study or nerve biopsy, and exclusion of any other disorders. The patients were divided into three groups, according to the area of nerve involvement: typical type, distal acquired demyelinating symmetric type（DADS）, and multifocal acquired demyelinating sensory and motor type（MADSAM）. Then, we retrospectively investigated their clinical symptoms, electrophysiological features, and responses to medication from their medical records. Results: Four patients were of the typical type, three DADS, and one MADSAM. Patients with the atypical type, i. e. DADS and MADSAM, had a significantly longer period till the diagnosis compared to patients with the typical type（p＝0.029）. As for the electrophysiological aspect, demyelination was found in all cases and conduction block in three cases. Two of atypical cases had no signs of albuminocytologic dissociation in the cerebrospinal fluid nor nerve root hypertrophy. In addition, all atypical cases needed nerve biopsy. All typical cases and two out of the four atypical cases achieved remission by means of immunomodulation therapy. Conclusions: Since a definite diagnosis of pediatric CIDP is difficult especially in patients with slowly progressive neuropathy, electrophysiological and pathological examinations should be actively used.