[PS2-1B-1 ] Epidemiology of Guillain-Barré syndrome, Fisher syndrome, and Bickerstaff brainstem encephalitis of children in Japan
Introduction: Guillain-Barré syndrome (GS), Fisher syndrome (FS), and Bickerstaff brainstem encephalitis (BE) are immune-mediated peripheral neurological disorders, exhibiting muscle weakness, ophthalmoplegia, or consciousness disturbance, respectively. Although adult epidemiology of these diseases have been well reported, little has been focused on children. This study aimed to disclose the epidemiology and clinical manifestations, therapy, and clinical outcome of children with these disorders. Materials and Methods: We investigated incidence of children with GBS, FS, BE during 2014, by sending questionnaires to 1066 specialists for pediatric neurology in Japan. This study was approved by local ethics committees of Chiba University and Japanese Society for Child Neurology.
Results: We obtained clinical data from 432 pediatric neurology specialist (42%). The number of patients with GBS, FS, BE in Japan during 2014, were 33, 2, and 2, respectively, indicating that the incidence rates according to the pediatric population of Japan were 0.32 to 100,000, 0.02 to 100,000 and 0.02 to 100,000, respectively. The disease course was variable, but prognosis was generally favorable, except for only two children who remained gait disturbance.
Conclusion: This is the first nationwide surveillance of childhood GBS, FS, and BE in Japan. The incidence rate as 0.32 to 100,000 of GBS was relatively lower than those from previous reports. These are quite rare diseases even in children, but if these are diagnosed promptly and treated adequately, prognosis of these diseases would be favorable.
Results: We obtained clinical data from 432 pediatric neurology specialist (42%). The number of patients with GBS, FS, BE in Japan during 2014, were 33, 2, and 2, respectively, indicating that the incidence rates according to the pediatric population of Japan were 0.32 to 100,000, 0.02 to 100,000 and 0.02 to 100,000, respectively. The disease course was variable, but prognosis was generally favorable, except for only two children who remained gait disturbance.
Conclusion: This is the first nationwide surveillance of childhood GBS, FS, and BE in Japan. The incidence rate as 0.32 to 100,000 of GBS was relatively lower than those from previous reports. These are quite rare diseases even in children, but if these are diagnosed promptly and treated adequately, prognosis of these diseases would be favorable.