Nationwide Survey of Childhood Guillain-Barré Syndrome, Fisher Syndrome, and Bickerstaff Brainstem Encephalitis in Japan

Abstract

Background: Guillain-Barre syndrome (GBS), Fisher syndrome (FS), and Bickerstaff brainstem encephalitis (BBE) are immune-mediated neuropathies presenting with symptoms such as muscle weakness, ophthalmoplegia, ataxia, and consciousness disturbances. Although the epidemiology of GBS and BBE in patients of all ages has been reported, childhood data have not been well-investigated. We aimed to determine the clinical features, therapeutics, and prognoses of childhood GBS, FS, and BBE in Japan. \n \nMethods: We sent questionnaires to 1068 pediatric neurologists in Japan from 2014 to 2016 to determine the number of children less than 15 years old with GBS, FS, or BBE and their age and sex. We subsequently performed a secondary survey to investigate the clinical features, laboratory data, treatment, and prognosis. This study was approved by the local ethics committee of the Graduate School of Medicine, Chiba University. \n \nFindings: Five-hundred thirty-eight pediatric neurology specialists (50·4%) responded to the first survey. The total number of children with GBS, FS, and BBE in Japan from 2014 to 2016 were 87, 10, and 6, respectively, suggesting annual incidence rates of was 0·19, 0·023, and 0·0071 per 100,000 children, respectively, in the pediatric population (0-14 years). GBS was classified as acute inflammatory demyelinating neuropathy (39·7%), acute motor axonal neuropathy (24·4%), or acute motor-sensory axonal neuropathy (11·5%), with a male-to-female ratio of 1·29:1·0 and a wide distribution of onset ages. The disease severities of GBS, FS, and BBE were variable, but all children were able to walk within one year, indicating an excellent childhood prognosis. No in-hospital deaths occurred in this study. \n \nInterpretation: The prognoses of childhood GBS, FS, and BBE were generally favorable, as long as the patient was promptly treated with either intravenous immunoglobulin or plasma exchange. \n \nClinical Trial Registration Details: The research scheme was registered at the UMIN Clinical Trials Registry (UMIN-CTR) as UMIN000018739. \n \nFunding Information: This work was supported by the institutional budget of the Chiba University Graduate School of Medicine (grant reference J09KF00284). \n \nDeclaration of Interests: The authors declare no conflict of interest. \n \nEthics Approval Statement: The study protocol was approved by the institutional ethics committee of the Graduate School of Medicine, Chiba University (No. 319), and the Collaborative Research Committee of the Japanese Society of Child Neurology (No. 13-04).