Abstract:

Objective: To investigate the clinical characteristics and prognosis of chronic inflammatory demyelinating polyneuropathy (CIDP) in children. Methods: Clinical data were collected and analyzed from patients with CIDP who visited the Department of Neurology, Children's Hospital of Fudan University between January 1,2008 and January 31,2017. PubMed databases were retrieved from January 1991 to February 2017 with CIDP cases in children. According to the peak time of the disease(4-8 weeks for subacute onset group, >8weeks for chronic onset group), the retrieved literature cases and the cases in the study were aggregated and divided into two groups, They were compared in clinical manifestation, laboratory examination, treatment and prognosis. Results: Firstly, there were 15 CIDP cases who met the criteria of inclusion and exclusion, including 6 males and 9 females; 4 cases between 1-3 years old, 4 cases between 4-7 years old, 7 cases between 8-15 years old; the age of disease onset was 1-11 years old;7 patients had a history of prodromal infection; development to the peak of the disease required 4 weeks to 13 months, with an average (12.93±13.57) weeks. At the beginning of the disease all cases showed dyskinesia;4 cases with sensory disorder; the peak of the disease MRS score: 3 points in 8 cases, 4 points in 6 cases and 5 points in 1 case; the course of disease was monophase in 3 cases, progressive in 5 cases and recurrent in 7 cases;all cases had nerve electrophysiological changes; in all cases dissociation of protein from cell in CSF occurred. 14 cases were treated with glucocorticoid, 1 case was treated with intravenous immunoglobin; 1 case was discharged from hospital voluntarily and 14 cases were hospitalized between 7-17 days with the clinical symptoms improved;during the follow-up period, 7 cases were relapsed.Secondly, 87 cases were retrieved from PubMed, together with our 15 cases，102 cases were analyzed. There were 38 patients with subacute-onset disease and 64 patients with chronic-onset disease. Patients exhibited symptoms in less than 2 months with more often sensory abnormalities (57.1% vs 23.5%，P=0.002), and cranial nerve abnormalities (25.7% vs 7.8%，P=0.023). There was no significant difference between the two groups in gender, age of disease onset and movement disorders. Patients with subacute-onset relapsed more than patients with chronic-onset(62.2% vs 34.0%，P=0.010).There were more monophasic evolution in chronic-onset disease than in subacute-onset disease (50.8% vs 27.8%,P=0.026).Conclusion: Children with subacute onset of CIDP are slightly more than adults, and their sensory abnormalities and cranial nerve abnormalities are more frequent than children with chronic onset of CIDP, and they are prone to relapse during disease development. Dyskinesia is common in children with CIDP, and cranial neuropathy and respiratory failure are unusual. Nerve electrophysiological changes and dissociation of protein from cell in CSF are common in CIDP, and early diagnosis and initial treatment are important.

Key words: Chronic inflammatory demyelinating polyradiculoneuropathy, Clinical analysis;Children