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Nationwide survey of childhood Guillain-Barré syndrome, Fisher syndrome, and Bickerstaff brainstem encephalitis in Japan

      Abstract

      Objective

      Guillain-Barré syndrome (GBS), Fisher syndrome (FS), and Bickerstaff brainstem encephalitis (BBE) are immune-mediated neuropathies presenting with symptoms such as 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.

      Methods

      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.

      Results

      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. GBS was classified as acute inflammatory demyelinating neuropathy (35.6%), acute motor axonal neuropathy (20.7%), or acute motor-sensory axonal neuropathy (10.3%), 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 could walk within one year.

      Conclusion

      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.

      Keywords

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      References

        • Yuki N.
        • Hartung H.-P.
        Guillain-Barré syndrome.
        N Engl J Med. 2012; 366: 2294-2304
        • van Doorn P.A.
        • Ruts L.
        • Jacobs B.C.
        Clinical features, pathogenesis, and treatment of Guillain-Barré syndrome.
        Lancet Neurol. 2008; 7: 939-950
        • Shahrizaila N.
        • Yuki N.
        Bickerstaff brainstem encephalitis and Fisher syndrome: anti-GQ1b antibody syndrome.
        J Neurol Neurosurg Psychiatry. 2013; 84: 576-583
        • Hahn A.F.
        Guillain-Barre syndrome.
        Lancet. 1998; 352: 635-641
        • Yuki N.
        Fisher syndrome and Bickerstaff brainstem encephalitis (Fisher Bickerstaff syndrome).
        J Neuroimmunol. 2009; 215: 1-9
        • Arányi Z.
        • Kovács T.
        • Sipos I.
        • Bereczki D.
        Miller Fisher syndrome: brief overview and update with a focus on electrophysiological findings.
        Eur J Neurol. 2012; 19: e1-e3
        • Odaka M.
        • Yuki N.
        • Yamada M.
        • Koga M.
        • Takemi T.
        • Hirata K.
        • et al.
        Bickerstaff’s brainstem encephalitis: clinical features of 62 cases and a subgroup associated with Guillain-Barre’ syndrome.
        Brain. 2003; 126: 2279-2290
        • Willison H.J.
        • Yuki N.
        Peripheral neuropathies and anti-glycolipid antibodies.
        Brain. 2002; 125: 2591-2625
        • Kaida K.
        • Kusunoki S.
        Antibodies to gangliosides and ganglioside complexes in Guillain-Barre syndrome and Fisher syndrome: mini-review.
        J Neuroimmunol. 2010; 223: 5-12
        • Hughes R.A.
        • Cornblath D.R.
        Guillain-Barre syndrome.
        Lancet. 2005; 366: 1653-1666
        • Van Koningsveld R.
        • Van Doorn P.A.
        • Schmitz P.I.M.
        • Ang C.W.
        • Van der Meche F.G.A.
        Mild forms of Guillain-Barre syndrome in an epidemiologic survey in The Netherlands.
        Neurology. 2000; 54: 620-625
        • Alshekhlee A.
        • Hussain Z.
        • Sultan B.
        • Katirji B.
        Guillain-Barre syndrome: incidence and mortality rates in US hospitals.
        Neurology. 2008; 70: 1608-1613
        • Bogliun G.
        • Beghi E.
        Incidence and clinical features of acute inflammatory polyradiculoneuropathy in Lombardy, Italy, 1996.
        Acta Neurol Scand. 2004; 110: 100-106
        • Hauck L.J.
        • White C.
        • Feasby T.E.
        • Zochodne D.W.
        • Svenson L.W.
        • Hill M.D.
        Incidence of Guillain-Barre syndrome in Alberta, Canada: an administrative data study.
        J Neurol Neurosurg Psychiatry. 2008; 79: 318-320
      1. Lehmann HC, Kohne A, zu Horste GM, Kieseier BC. Incidence of Guillain-Barre syndrome in Germany. J Peripher Nerv Syst 2007;12:285.

        • McGrogan A.
        • Madle G.C.
        • Seaman H.E.
        • de Vries C.S.
        The epidemiology of Guillain-Barré syndrome worldwide. A systematic literature review.
        Neuroepidemiology. 2009; 32: 150-163
        • Sejvar J.J.
        • Baughman A.L.
        • Wise M.
        • Morgan O.W.
        Population incidence of Guillain-Barré syndrome: a systematic review and meta-analysis.
        Neuroepidemiology. 2011; 36: 123-133
        • Saito T.
        • Arimura K.
        • Osame M.
        Nationwide survey of Guillain-Barré syndrome in Japan -Therapeutic preliminary report-. (in Japanese).
        Prog Med. 1999; 19: 2369-2374
        • Hicks C.W.
        • Kay B.
        • Worley S.E.
        • Moodley M.
        A clinical picture of Guillain-Barré syndrome in children in the United States.
        J Child Neurol. 2010; 25: 1504-1510
        • Nachamkin I.
        • Barbosa P.A.
        • Ung H.
        • Lobato C.
        • Rivera A.G.
        • Rodriguez P.
        • et al.
        Patterns of Guillain-Barre syndrome in children: results from a Mexican population.
        Neurology. 2007; 69: 1665-1671
        • Lyu R.K.
        • Tang L.M.
        • Cheng S.Y.
        • Hsu W.C.
        • Chen S.T.
        Guillain-Barré syndrome in Taiwan: a clinical study of 167 patients.
        J Neurol Neurosurg Psychiatry. 1997; 63: 494-500
        • Lin J.J.
        • Hsia S.H.
        • Wang H.S.
        • Lyu R.K.
        • Chou M.L.
        • Hung P.C.
        • et al.
        Clinical variants of Guillain-Barré syndrome in children.
        Pediatr Neurol. 2012; 47: 91-96
        • Nagasawa K.
        • Kuwabara S.
        • Misawa S.
        • Fujii K.
        • Tanabe Y.
        • Yuki N.
        • et al.
        Electrophysiological subtypes and prognosis of childhood Guillain-Barré syndrome in Japan.
        Muscle Nerve. 2006; 33: 766-770
        • Doets A.Y.
        • Verboon C.
        • van den Berg B.
        • Harbo T.
        • Cornblath D.R.
        • Willison H.J.
        • et al.
        Regional variation of Guillain-Barré syndrome.
        Brain. 2018; 141: 2866-2877
        • Arnason G.B.W.
        Acute inflammatory demyelinating polyneuropathy.
        in: Dyek P.K. Griffin J.W. Low P.A. Poduslo J.F. Peripheral neuropathy. 3rd ed. Saunders, Philadelphia1993: 1437-1497
        • Mori M.
        • Kuwabara S.
        • Fukutake T.
        • Yuki N.
        • Hattori T.
        Clinical features and prognosis of Miller Fisher syndrome.
        Neurology. 2001; 56: 1104-1106
        • Koga M.
        • Kusunoki S.
        • Kaida K.
        • Uehara R.
        • Nakamura Y.
        • Kohriyama T.
        • et al.
        Nationwide survey of patients in Japan with Bickerstaff brainstem encephalitis: epidemiological and clinical characteristics.
        J Neurol Neurosurg Psychiatry. 2012; 83: 1210-1215
        • Ito M.
        • Kuwabara S.
        • Odaka M.
        • Misawa S.
        • Koga M.
        • Hirata K.
        • et al.
        Bickerstaff’s brainstem encephalitis and Fisher syndrome form a continuous spectrum: clinical analysis of 581 cases.
        J Neurol. 2008; 255: 674-682
        • Santoro J.D.
        • Lazzareschi D.V.
        • Campen C.J.
        • Van Haren K.P.
        Pediatric Bickerstaff brainstem encephalitis: a systematic review of literature and case series.
        J Neurol. 2018; 265: 141-150
        • Asbury A.K.
        • Cornblath D.R.
        Assessment of current diagnostic criteria for Guillain-Barré syndrome.
        Ann Neurol. 1990; 27: S21-S24
      2. Criteria for diagnosis of Guillain-Barré syndrome. Ann Neurol 1978;3:565–6.

        • Ho T.W.
        • Mishu B.
        • Li C.Y.
        • Gao C.Y.
        • Cornblath D.R.
        • Griffin J.W.
        • et al.
        Guillain-Barré syndrome in northern China. Relationship to Campylobacter jejuni infection and anti-glycolipid antibodies.
        Brain. 1995; 118: 597-605
        • Hughes R.A.
        • Newsom-Davis J.M.
        • Perkin G.D.
        • Pierce J.M.
        Controlled trial prednisolone in acute polyneuropathy.
        Lancet. 1978; 2: 750-753
        • Papri N.
        • Islam Z.
        • Leonhard S.E.
        • Mohammad Q.D.
        • Endtz H.P.
        • Jacobs B.C.
        Guillain-Barré syndrome in low-income and middle-income countries: challenges and prospects.
        Nat Rev Neurol. 2021; 17: 285-296
        • Sejvar J.J.
        • Kohl K.S.
        • Gidudu J.
        • Amato A.
        • Bakshi N.
        • Baxter R.
        • et al.
        Guillain-Barré syndrome and Fisher syndrome: case definitions and guidelines for collection, analysis, and presentation of immunization safety data.
        Vaccine. 2011; 29: 599-612
        • Roodbol J.
        • de Wit M.-C.
        • van den Berg B.
        • Kahlmann V.
        • Drenthen J.
        • Catsman-Berrevoets C.E.
        • et al.
        Diagnosis of Guillain-Barré syndrome in children and validation of the Brighton criteria.
        J Neurol. 2017; 264: 856-861
        • Ogawara K.
        • Kuwabara S.
        • Mori M.
        • Hattori T.
        • Koga M.
        • Yuki N.
        Axonal Guillain-Barré syndrome: relation to anti-ganglioside antibodies and Campylobacter jejuni infection in Japan.
        Ann Neurol. 2000; 48: 624-631
        • Kuwabara S.
        • Yuki N.
        • Koga M.
        • Hattori T.
        • Matsuura D.
        • Miyake M.
        • et al.
        IgG anti-GM1 antibody is associated with reversible conduction failure and axonal degeneration in Guillain-Barré syndrome.
        Ann Neurol. 1998; 44: 202-208
        • Kokubun N.
        • Nishibayashi M.
        • Uncini A.
        • Odaka M.
        • Hirata K.
        • Yuki N.
        Conduction block in acute motor axonal neuropathy.
        Brain. 2010; 133: 2897-2908
        • Ho T.W.
        • Mishu B.
        • Li C.Y.
        • Gao C.Y.
        • Cornblath D.R.
        • Griffin J.W.
        • et al.
        Guillain-Barré syndrome in northern China: relationship to Campylobacter jejuni infection and anti-glycolipid antibodies.
        Brain. 1995; 118: 597-605
        • Hadden R.D.M.
        • Cornblath D.R.
        • Hughes R.A.C.
        • Zielasek J.
        • Hartung H.-P.
        • Toyka K.V.
        • et al.
        Electrophysiological classification of Guillain-Barré syndrome: clinical associations and outcome.
        Ann Neurol. 1998; 44: 780-788
        • Kuwabara S.
        • Yuki N.
        Axonal Guillain-Barré syndrome: concepts and controversies.
        Lancet Neurol. 2013; 12: 1180-1188
        • Willison H.J.
        • Jacobs B.C.
        • van Doorn P.A.
        Guillain-Barré syndrome.
        Lancet. 2016; 388: 717-727
        • Hughes R.A.
        • Swan A.V.
        • Raphaël J.C.
        • Annane D.
        • van Koningsveld R.
        • van Doorn P.A.
        Immunotherapy for Guillain-Barré syndrome: a systematic review.
        Brain. 2007; 130: 2245-2257
        • Gürses N.
        • Uysal S.
        • Cetinkaya F.
        • Icslek I.
        • Kalayci A.G.
        Intravenous immunoglobulin treatment in children with Guillain-Barré syndrome.
        Scand J Infect Dis. 1995; 27: 241-243
        • Korinthenberg R.
        • Schessl J.
        • Kirschner J.
        • Mo¨ntning J.S.
        Intravenous immunoglobulin in the treatment of childhood Guillain-Barré syndrome.
        Pediatrics. 2005; 116: 8-14
        • Wang R.
        • Feng A.
        • Sun W.
        • Wen Z.
        Intravenous immunoglobulin in children with Guillain-Barré syndrome.
        J Appl Clin Pediatr. 2001; 16: 223-224
        • Misawa S.
        • Kuwabara S.
        • Sato Y.
        • Yamaguchi N.
        • Nagashima K.
        • Katayama K.
        • et al.
        Safety and efficacy of eculizumab in Guillain-Barre syndrome: a multicentre, double-blind, randomised phase 2 trial.
        Lancet Neurol. 2018; 17: 519-529
        • Jang Y.
        • Choi J.H.
        • Chae J.H.
        • Lim B.C.
        • Kim S.J.
        • Jung J.H.
        Pediatric Miller Fisher syndrome; characteristic presentation and comparison with adult Miller Fisher syndrome.
        J Clin Med. 2020; 9: 3930
        • Roodbol J.
        • de Wit M.C.
        • Walgaard C.
        • de Hoog M.
        • Catsman-Berrevoets C.E.
        • Jacobs B.C.
        Recognizing Guillain-Barré syndrome in preschool children.
        Neurology. 2011; 76: 807-810
        • Roodbol J.
        • de Wit M.C.
        • Aarsen F.K.
        • Catsman-Berrevoets C.E.
        • Jacobs B.C.
        Long-term outcome of Guillain-Barré syndrome in children.
        J Peripher Nerv Syst. 2014; 19: 121-126