DOI QR코드

DOI QR Code

Overlapping Guillain-Barr$\acute{e}$ syndrome and Bickerstaff's brainstem encephalitis associated with Epstein Barr virus

  • Rho, Young Il (Department of Pediatrics, Chosun University School of Medicine)
  • Received : 2013.04.10
  • Accepted : 2013.10.16
  • Published : 2014.10.10

Abstract

A flaccid tetraparesis in Bickerstaff's brainstem encephalitis (BBE) is presumed to be a sign of overlapping Guillain-Barr$\acute{e}$ syndrome (GBS). In addition, BBE and Fisher syndrome, which are clinically similar and are both associated with the presence of the immunoglobulin G anti-GQ1b antibody, represent a specific autoimmune disease with a wide spectrum of symptoms that include ophthalmoplegia and ataxia. A 2-year-old boy presented with rapidly progressive ophthalmoplegia, ataxia, hyporeflexia, weakness of the lower extremities, and, subsequently, disturbance of consciousness. He experienced bronchitis with watery diarrhea and had laboratory evidence of recent infection with Epstein-Barr virus (EBV). He was diagnosed as having overlapping GBS and BBE associated with EBV and received treatment with a combination of immunoglobulin and methylprednisolone, as well as acyclovir, and had recovered completely after 3 months. In addition, he has not experienced any relapse over the past year. We suggest that combinations of symptoms and signs of central lesions (disturbance of consciousness) and peripheral lesions (ophthalmoplegia, facial weakness, limb weakness, and areflexia) are supportive of a diagnosis of overlapping GBS and BBE and can be helpful in achieving an early diagnosis, as well as for the administration of appropriate treatments.

Keywords

References

  1. 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(Pt 10):2279-90. https://doi.org/10.1093/brain/awg233
  2. Yuki N, Wakabayashi K, Yamada M, Seki K. Overlap of Guillain-Barre syndrome and Bickerstaff's brainstem encephalitis. J Neurol Sci 1997;145:119-21. https://doi.org/10.1016/S0022-510X(96)00251-1
  3. Kamasaki A, Kinoshita I, Koga M, Yamaguchi T, Yuki N. A case of brainstem encephalitis associated with Epstein-Barr virus infection: differentiation of acute disseminated encephalomyelitis and Bickerstaff's brainstem encephalitis. No To Shinkei 2001;53:951-5.
  4. Steer AC, Starr M, Kornberg AJ. Bickerstaff brainstem encephalitis associated with Mycoplasma pneumoniae infection. J Child Neurol 2006;21:533-4. https://doi.org/10.1177/08830738060210061401
  5. Kanzaki A, Yabuki S, Yuki N. Bickerstaff's brainstem encephalitis associated with cytomegalovirus infection. J Neurol Neurosurg Psychiatry 1995;58:260-1. https://doi.org/10.1136/jnnp.58.2.260
  6. Tagawa Y, Yuki N. Bickerstaff's brainstem encephalitis associated with shingles. J Neurol 2000;247:218-9. https://doi.org/10.1007/s004150050567
  7. Bickerstaff ER, Cloake PC. Mesencephalitis and rhombencephalitis. Br Med J 1951;2:77-81. https://doi.org/10.1136/bmj.2.4723.77
  8. Yuki N, Sato S, Tsuji S, Hozumi I, Miyatake T. An immunologic abnormality common to Bickerstaff's brain stem encephalitis and Fisher's syndrome. J Neurol Sci 1993;118:83-7. https://doi.org/10.1016/0022-510X(93)90250-3
  9. Reddel SW, Barnett MH, Yan WX, Halmagyi GM, Pollard JD. Chronic ophthalmoplegia with anti-GQ1b antibody. Neurology 2000;54:1000-2. https://doi.org/10.1212/WNL.54.4.1000
  10. Bruno M, Sicurella L. Bickerstaff's brainstem encephalitis: case report. Riv It Neurobiologia 2009;55:19-23.
  11. Tsukamoto H, Sonoo M, Hatanaka Y, Kobayashi M, Shimizu T. Utility of somatosensory evoked potentials in the early diagnosis of Bickerstaff's brainstem encephalitis. Int Congr Ser 2005:1278;99-102. https://doi.org/10.1016/j.ics.2004.11.027
  12. Zifko U, Drlicek M, Senautka G, Grisold W. High dose immunoglobulin therapy is effective in the Miller Fisher syndrome. J Neurol 1994;241:178-9.
  13. Dalakas MC. Mechanism of action of intravenous immunoglobulin and therapeutic considerations in the treatment of autoimmune neurologic diseases. Neurology 1998;51(6 Suppl 5):S2-8.
  14. Kim JY, Kim YO, Son YJ, Woo YJ. A case of Bickerstaff's brainstem encephalitis in childhood. Korean J Pediatr 2010;53:607-11. https://doi.org/10.3345/kjp.2010.53.4.607

Cited by

  1. Guillain-Barré syndrome: causes, immunopathogenic mechanisms and treatment vol.12, pp.11, 2016, https://doi.org/10.1080/1744666x.2016.1193006
  2. Pediatric Bickerstaff brainstem encephalitis: a systematic review of literature and case series vol.265, pp.1, 2014, https://doi.org/10.1007/s00415-017-8684-8
  3. To be, or not to be… Guillain-Barré Syndrome vol.20, pp.12, 2021, https://doi.org/10.1016/j.autrev.2021.102983