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Journal of Neurology

Erschienen in:

24.11.2017 | Original Communication

Pediatric Bickerstaff brainstem encephalitis: a systematic review of literature and case series

verfasst von: Jonathan Douglas Santoro, Daniel V. Lazzareschi, Cynthia Jane Campen, Keith P. Van Haren

Erschienen in: Journal of Neurology | Ausgabe 1/2018

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Abstract

Objective

To characterize the phenotype of pediatric Bickerstaff’s brainstem encephalitis (BBE) and evaluate prognostic features in the clinical course, diagnostic studies, and treatment exposures.

Methods

We systematically reviewed PubMed, Web of Science, and SCOPUS databases as well as medical records at the Lucile Packard Children’s Hospital to identify cases of pediatric BBE. Inclusion required all of the following criteria: age ≤ 20 years, presence of somnolence or alterations in mental status at the time of presentation or developed within 7 days of presentation, ataxia, and ophthalmoplegia.

Results

We reviewed 682 manuscripts, identifying a total of 47 pediatric BBE cases. We also describe five previously unreported cases. The phenotype of these pediatric patients was similar to previously published literature. Sixty-eight percent of patients demonstrated positive anti-GQ1b antibody titers, yet the presence of these antibodies was not associated with longer times to recovery. Patients with neuroimaging abnormalities featured a longer median time to recovery, but this was not statistically significant (p = 0.124). Overall, patients treated with any form of immunotherapy (intravenous immunoglobulin, steroids, or plasmapheresis) demonstrated shorter median time to resolution of symptoms compared to supportive therapy, although this trend was not statistically significant (p = 0.277). Post-hoc t tests revealed a trend towards use of immunotherapy against supportive care alone (p = 0.174).

Conclusion

Our study identified clinical, radiologic, and treatment features that may hold prognostic value for children with BBE. The role of immunotherapy remains under investigation but may prove of utility with further, randomized controlled studies in this rare disease.

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Fisher M (1956) An unusual variant of acute idiopathic polyneuritis—syndrome of ophthalmoplegia, ataxia, and areflexia. N Engl J Med 255(2):57–65PubMedCrossRef

Ito M, Kuwabara S, Odaka M, Misawa S, Koga M, Hirata K, Yuki N (2008) Bickerstaff’s brainstem encephalitis and Fisher syndrome form a continuous spectrum: clinical analysis of 581 cases. J Neurol 255(5):674–682PubMedCrossRef

Mori M, Kuwabara S, Fukutake T, Yuki N, Hattori T (2001) Clinical features and prognosis of Miller Fisher syndrome. Neurology 56(8):1104–1106PubMedCrossRef

Koga M, Kusunoki S, Kaida K, Uehara R, Nakamura Y, Kohriyama T, Kanda T (2012) Nationwide survey of patients in Japan with Bickerstaff brainstem encephalitis: epidemiological and clinical characteristics. J Neurol Neurosurg Psychiatry 83(12):1210–1215PubMedCrossRef

Hacohen Y, Nishimoto Y, Fukami Y, Lang B, Waters P, Lim MJ, Yuki N et al (2016) Paediatric brainstem encephalitis associated with glial and neuronal autoantibodies. Dev Med Child Neurol 58(8):836–841PubMedCrossRef

Ajena D, Ferrari S, Romito S, Zagila F, Biban P, Squintani G (2013) A pediatric case of Miller Fisher syndrome with central involvement. Neurol Sci. 34(9):1689–1690PubMedCrossRef

Al-Din AN, Anderson M, Bickerstaff ER, Harvey I (1982) Brainstem encephalitis and the syndrome of Miller Fisher: a clinical study. Brain 105(Pt 3):481–495PubMedCrossRef

Arias AP, Santoni A, Goenaga B, Martinez MJ, Gonzalez N (2015) Sindrome de Bickerstaff: caso Clinico. Arch Argent Pediatr 113(4):226–229

Barontini F, Sitá D (1983) The nosological position of Fisher’s syndrome—ophthalmoplegia, ataxia and areflexia. J Neurol 229(1):33–44PubMedCrossRef

10.

Bell W, Van Allen M, Blackman J (1970) Fisher syndrome in childhood. Dev Med Child Neurol 12(6):758–766PubMedCrossRef

11.

Bradshaw DY, Jones HR (2001) Pseudomeningoencephalitic presentation of pediatric Guillain Barre syndrome. J*** Child Neurol 16(7):505–508CrossRef

12.

Campos-Segovia A, Perez-Villena A, Martinez J, Sanchez CZ, Blanco-Martin AB, Aurrecoechea JF, Izquierdo SM, Jimenez-Martinez J (2016) Encefalitis de tronco de Bickerstaff atipica: 2 casos pediatricos. Neurol Argent 8(3):181–186CrossRef

13.

Damasceno A, Franca MC Jr, Pimenta DS, Deus-Silva L, Nucci A, Damasceno BP (2008) Bickerstaff’s encephalitis, Guillain-Barré syndrome and idiopathic intracranial hypertension: are they related conditions? Arg Neuropsiquiatr 66(3B):744–746CrossRef

14.

Eggenberger ER, Coker S, Menezes M (1993) Pediatric Miller Fisher syndrome requiring intubation: a case report. Clin Pediatr (Phila) 32(6):372–375CrossRef

15.

Fargas A, Roig M, Vazquez E, Fitó A (1998) Brainstem involvement in a child with ophthalmoplegia, ataxia, areflexia syndrome. Pediatr Neurol 18(1):73–75PubMedCrossRef

16.

Fargas i Busquets A, Roig Quilis M, Gratacòs Vinyola M, Macaya Ruiz A, del Toro Riera M, Fitó Costa A (1998) Ophthalmoplegia-ataxia-areflexia in pediatrics three new patients and review of the literature. An Esp Pediatr 48(5):483–488PubMed

17.

Fox RJ, Kasner SE, Galetta SL, Chalela JA (2000) Treatment of Bickerstaff’s brainstem encephalitis with immune globulin. J Neurol Sci 178(2):88–90PubMedCrossRef

18.

Genizi J, Kasis I, Schif A, Shahar E (2007) Effect of high-dose methyl-prednisolone on brainstem encephalopathy and basal ganglia impairment complicating cat scratch disease. Brain Dev. 29(6):377–379PubMedCrossRef

19.

Gologorsky RC, Barakos JA, Sahebkar F (2013) Rhomb- and Bickerstaff encephalitis: two clinical phenotypes? Pediatr Neurol 48(3):244–248PubMedCrossRef

20.

Han C, Wang Y, Jia J, Ji X, Fredrickson V, Ding Y, Sun W et al (2012) Bickerstaff’s brainstem encephalitis, Miller–Fisher syndrome and Guillain-Barre syndrome overlap in an asthma patient with negative anti-ganglioside antibodies. BMC Res Notes 5:295PubMedPubMedCentralCrossRef

21.

Kanzaki A, Yabuki S, Yuki N (1995) Bickerstaff’s brainstem encephalitis associated with cytomegalovirus infection. J Neurol Neurosurg Psychiatry 58(2):260–261PubMedPubMedCentralCrossRef

22.

Kikuchi M, Tagawa Y, Iwamoto H, Hoshino H, Yuki N (1997) Bickerstaff’s brainstem encephalitis associated with IgG anti-GQ1b antibody subsequent to Mycoplasma pneumoniae infection: favorable response to immunoadsorption therapy. J Child Neurol 12(6):403–405PubMedCrossRef

23.

Kimoto K, Koga M, Odaka M, Hirata K, Takahashi M, Li J, Gilbert M, Yuki N (2006) Relationship of bacterial strains to clinical syndromes of Campylobacter-associated neuropathies. Neurology 67(10):1837–1843PubMedCrossRef

24.

Matsumoto H, Kobayashi O, Tamura K, Ohkawa T, Sekine I (2002) Miller Fisher syndrome with transient coma: comparison with Bickerstaff brainstem encephalitis. Brain Dev 24(2):98–101PubMedCrossRef

25.

Matsuo M, Odaka M, Koga M, Tsuchiya K, Hamasaki Y, Yuki N (2004) Bickerstaff’s brainstem encephalitis associated with IgM antibodies to GM1b and GalNAc-GD1a. J Neurol Sci 217(2):225–228PubMedCrossRef

26.

Meyer Sauteur PM, Relly C, Hackenberg A, Stahr N, Berger C, Bloemberg GV, Jacobs E et al (2014) Mycoplasma pneumoniae intrathecal antibody responses in Bickerstaff brainstem encephalitis. Neuropediatrics 45(1):61–63PubMed

27.

Park JY, Ko KO, Lim JW, Cheon EJ, Yoon JM, Kim HJ (2014) A pediatric case of Bickerstaff’s brainstem encephalitis. Korean J Pediatr 57(12):542–545PubMedPubMedCentralCrossRef

28.

Pavone P, Le Pira A, Greco F, Vitaliti G, Smilari PL, Parano E, Falsaperla R (2014) Bickerstaff’s brainstem encephalitis (BBE) in childhood: rapid resolution after intravenous immunoglobulins treatment. Eur Rev Med Pharmacol Sci 18(17):2496–2499PubMed

29.

Ragosta K (1993) Miller Fisher syndrome, a brainstem encephalitis, mimics brain death. Clin Pediatr (Phila) 32(11):685–687CrossRef

30.

Reyes AR, Garcés P, Reig R, Martinéz E, Sebastián I, Herrera M (2001) Benign encephalitis of the brainstem. Rev Neurol 32(4):341–344PubMed

31.

Rho YI (2014) Overlapping Guillain-Barré syndrome and Bickerstaff’s brainstem encephalitis associated with Epstein Barr virus. Korean J Pediatr 57(10):457–460PubMedPubMedCentralCrossRef

32.

Schain RJ, Wilson G (1971) Brainstem encephalitis with radiographic evidence of medullary enlargement. Neurology 21(5):537–539PubMedCrossRef

33.

Sonmez FM, Odemis E, Ahmetoglu A, Ayvaz A (2004) Brainstem encephalitis and acute disseminated encephalomyelitis following mumps. Pediatr Neurol 30(2):132–134PubMedCrossRef

34.

Steer AC, Starr M, Kornberg AJ (2006) Bickerstaff brainstem encephalitis associated with Mycoplasma pneumoniae infection. J Child Neurol 21(6):533–534PubMedCrossRef

35.

Suzuki K, Meguro K, Nakayama J, Aoki T, Tsurushima H (1997) MRI of an infant with Fisher syndrome. Childs Nerv Syst 13(2):95–96PubMedCrossRef

36.

Tachi N, Tsuzuki Y, Minami R (1986) A case of brainstem encephalitis with CT scan abnormality mimicking Fisher syndrome. Brain Dev 8(5):538–541PubMedCrossRef

37.

Tsapis M, Laugel V, Koob M, de Saint Martin A, Fischbach M (2010) A pediatric case of Fisher–Bickerstaff spectrum. Pediatr Neurol 42(2):147–150PubMedCrossRef

38.

Uribe SMR, Suárez HF, Sandoval RP, Mellado TP (2013) Anti-GQ1b syndrome: a report of four cases. Rev Med Chil 141(9):1211–1215CrossRef

39.

Venkateshwara Prasad KN, Venkatesh KS, Devi NG (2013) Bickerstaff brainstem encephalitis in pediatrics—a case report. J Pediatr Neurosci 8(3):263–264PubMedPubMedCentralCrossRef

40.

Villalobos Chaves F, Casado Chocán JL, Moreno Rojas A, Serrano Castro V (1995) Miller–Fisher syndrome and encephalitis of the brainstem. Neurologia 10(2):115–116PubMed

41.

Wali GM (1991) Bickerstaff’s brainstem encephalitis associated with typhoid fever. Postgrad Med J 67(793):1011–1012PubMedPubMedCentralCrossRef

42.

Yuki N, Odaka M, Hirata K (2000) Bickerstaff’s brainstem encephalitis subsequent to Campylobacter jejuni enteritis. J Neurol Neurosurg Psychiatry 68(5):680–681PubMedPubMedCentralCrossRef

43.

Odaka M, Yuki N, Hirata K (2001) Anti-GQ1b IgG antibody syndrome: clinical and immunological range. J Neurol Neurosurg Psychiatry 70(1):50–55PubMedPubMedCentralCrossRef

44.

Tan IL, Mowry EM, Steele SU, Pardo CA, McArthur J, Nath A, Venkatesan A (2013) Brainstem encephalitis: etiologies, treatment and predictors of outcome. J Neurol 260(9):2312–2319PubMedPubMedCentralCrossRef

45.

Overell JR, Hseih ST, Odaka M, Yuki N, Willison HJ (2007) Treatment for Fisher syndrome, Bickerstaff’s brain-stem encephalitis and related disorders. Cochrane Database of Systematic Reviews (Art. no.: CD004761)

Titel: Pediatric Bickerstaff brainstem encephalitis: a systematic review of literature and case series
verfasst von: Jonathan Douglas Santoro
Daniel V. Lazzareschi
Cynthia Jane Campen
Keith P. Van Haren
Publikationsdatum: 24.11.2017
Verlag: Springer Berlin Heidelberg
Erschienen in: Journal of Neurology / Ausgabe 1/2018
Print ISSN: 0340-5354
Elektronische ISSN: 1432-1459
DOI: https://doi.org/10.1007/s00415-017-8684-8

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