[P1-128] A Nationwide Survey of Guillain-Barré Syndrome, Fisher’s Syndrome, and Bickerstaff's Brainstem Encephalitis in Children in Japan
[Introduction] Guillain-Barré Syndrome (GBS), Fisher syndrome (FS) and Bickerstaff’s brainstem encephalitis (BBE) are well-known but rare diseases. There was no nationwide survey of these childhood diseases in Japan. The purpose of this survey is to clarify epidemiology, clinical courses and prognosis of these diseases in children in Japan.
[Methods] A questionnaire about pediatric patients with GBS, FS and BBE in 2014 was sent to 1,066 child neurologists in Japan, and then we asked them more precisely by e-mail or telephone.
[Results] Response rate was 42%. 32 GBS patients, 2 FS patients and 2 BBE patients were reported. Median age was 5 years old (1-16 years). 17 males and 19 females were included. Incidence rates of GBS, FS and BBE were 0.32, 0.022 and 0.022 per 100,000 person-years respectively. At onset, 35.7% patients could walk, 50% patients could not walk, 14.3% patients needed mechanical ventilation. AIDP (Acute inflammatory demyelinating polyneuropathy), AMAN (Acute motor axonal neuropathy), AMSAN (acute motor sensory axonal neuropathy) and unclassified were 46.4%, 28.6%, 3.6% and 21.4% respectively. 57% patients, including all 8 AMAN type patients, were positive for antiganglioside antibodies. Anti GM1 IgM antibody was the most frequently detected (42.9% among all antiganglioside antibody positive patients).
[Conclusions] Incidence rate of GBS in Japanese children was lower compared with world-wide reports and decreased from the previous report in Japan. Positive rate of anti-ganglioside antibodies was the same as other reports. FS and BBE were very rare diseases. Continuous survey will be necessarily to know the longitudinal trend GBS in pediatric patients in Japan.
[Methods] A questionnaire about pediatric patients with GBS, FS and BBE in 2014 was sent to 1,066 child neurologists in Japan, and then we asked them more precisely by e-mail or telephone.
[Results] Response rate was 42%. 32 GBS patients, 2 FS patients and 2 BBE patients were reported. Median age was 5 years old (1-16 years). 17 males and 19 females were included. Incidence rates of GBS, FS and BBE were 0.32, 0.022 and 0.022 per 100,000 person-years respectively. At onset, 35.7% patients could walk, 50% patients could not walk, 14.3% patients needed mechanical ventilation. AIDP (Acute inflammatory demyelinating polyneuropathy), AMAN (Acute motor axonal neuropathy), AMSAN (acute motor sensory axonal neuropathy) and unclassified were 46.4%, 28.6%, 3.6% and 21.4% respectively. 57% patients, including all 8 AMAN type patients, were positive for antiganglioside antibodies. Anti GM1 IgM antibody was the most frequently detected (42.9% among all antiganglioside antibody positive patients).
[Conclusions] Incidence rate of GBS in Japanese children was lower compared with world-wide reports and decreased from the previous report in Japan. Positive rate of anti-ganglioside antibodies was the same as other reports. FS and BBE were very rare diseases. Continuous survey will be necessarily to know the longitudinal trend GBS in pediatric patients in Japan.