Journal of Neurology

, Volume 255, Issue 5, pp 674–682

Bickerstaff's brainstem encephalitis and Fisher syndrome form a continuous spectrum

Clinical analysis of 581 cases
  • M. Ito
  • S. Kuwabara
  • M. Odaka
  • S. Misawa
  • M. Koga
  • K. Hirata
  • N. Yuki*
ORIGINAL COMMUNICATION

Abstract

Whether Bickerstaff's brainstem encephalitis (BBE) is a distinct disease or a subtype of Fisher syndrome (FS) is unclear as there have been no clinical studies with sufficiently large numbers of patients with FS or BBE. Our aim was to clarify the nosological relationship. Medical records of patients suffering acute ophthalmoplegia and ataxia within four weeks of onset were reviewed. BBE was the diagnosis for patients with impaired consciousness, FS for those with clear consciousness and areflexia. Clinical features, neuroimages, and laboratory findings were analyzed. Patients were grouped as having BBE (n = 53), FS (n = 466), or as unclassified (n = 62). The BBE and FS groups had similar features; positive serum anti-GQ1b IgG antibody (68 % versus 83 %), antecedent Campylobacter jejuni infection (23 % versus 21 %), CSF albuminocytological dissociation (46 % versus 76 %), brain MRI abnormality (11 % versus 2 %), and abnormal EEG findings (57 % versus 25 %). BBE (n = 4) and FS (n = 28) subgroups underwent detailed electrophysiological testing. Both groups frequently showed absent soleus H-reflexes, but normal sensory nerve conduction (75 % versus 74 %) and a 1-Hz power spectrum peak on postural body sway analysis (67 % versus 72 %). Common autoantibodies, antecedent infections, and MRI and neurophysiological results found in this large study offer conclusive evidence that Bickerstaff's brainstem encephalitis and Fisher syndrome form a continuous spectrum with variable CNS and PNS involvement.

Key words

Anti-GQ1b antibody Bickerstaff's brainstem encephalitis Campylobacter jejuni Fisher syndrome Guillain-Barré syndrome 

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References

  1. 1.
    Al-Din AN, Anderson M, Bickerstaff ER, Harvey I (1982) Brainstem encephalitis and the syndrome of Miller Fisher: a clinical study. Brain 105:481–495PubMedCrossRefGoogle Scholar
  2. 2.
    Asbury AK, Cornblath DR (1990) Assessment of current diagnostic criteria for Guillain-Barré syndrome. Ann Neurol 27(Suppl):S21–S24PubMedCrossRefGoogle Scholar
  3. 3.
    Bergin PS, Bronstein AM, Murray NM, Sancovic S, Zeppenfeld DK (1995) Body sway and vibration perception thresholds in normal aging and in patients with polyneuropathy. J Neurol Neurosurg Psychiatry 58:335–340PubMedGoogle Scholar
  4. 4.
    Bickerstaff ER (1957) Brain-stem encephalitis: further observations on a grave syndrome with benign prognosis. Br Med J i:5032:1384–1387Google Scholar
  5. 5.
    Bickerstaff ER, Cloake PCP (1951) Mesencephalitis and rhombencephalitis. Br Med J ii:4723:77–81Google Scholar
  6. 6.
    Chiba A, Kusunoki S, Obata H, Machinami R, Kanazawa I (1993) Serum anti-GQ1b IgG antibody is associated with ophthalmoplegia in Miller Fisher syndrome and Guillain-Barré syndrome: clinical and immunohistochemical studies. Neurology 43:1911–1917PubMedGoogle Scholar
  7. 7.
    Chiba A, Kusunoki S, Shimizu T, Kanazawa I (1992) Serum IgG antibody to ganglioside GQ1b is a possible marker of Miller Fisher syndrome. Ann Neurol 31:677–679PubMedCrossRefGoogle Scholar
  8. 8.
    Echaniz-Laguna A, Battaglia F, Heymann R, Tranchant C, Warter JM (2000) The Miller Fisher syndrome: neurophysiological and MRI evidence of both peripheral and central origin in one case. J Neurol 247:980–982PubMedCrossRefGoogle Scholar
  9. 9.
    Faraci FM, Choi J, Baumbach GL, Mayhan WG, Heistad DD (1989) Microcirculation of the area postrema. Permeability and vascular responses. Circ Res 65:417–425PubMedGoogle Scholar
  10. 10.
    Fisher M (1956) An unusual variant of acute idiopathic polyneuritis (syndrome of ophthalmoplegia, ataxia and areflexia). N Engl J Med 255:57–65PubMedCrossRefGoogle Scholar
  11. 11.
    Fross RD, Daube JR (1987) Neuropathy in the Miller Fisher syndrome: clinical and electrophysiologic findings. Neurology 37:1493–1498PubMedGoogle Scholar
  12. 12.
    Gilbert M, Karwaski MF, Bernatchez S, Young NM, Taboada E, Michniewicz J, Cunningham AM, Wakarchuk WW (2002) The genetic bases for the variation in the lipo-oligosaccharide of the mucosal pathogen, Campylobacter jejuni: biosynthesis of sialylated ganglioside mimics in the core oligosaccharide. J Biol Chem 277:327–337PubMedCrossRefGoogle Scholar
  13. 13.
    Hughes RAC, Raphaël JC, Swan AV, van Doorn PA (2006) Intravenous immunoglobulin for Guillain-Barré syndrome. Cochrane Database Syst Rev:CD002063Google Scholar
  14. 14.
    Jamal GA, Ballantyne JP (1988) The localization of the lesion in patients with acute ophthalmoplegia, ataxia and areflexia (Miller Fisher syndrome): a serial multimodal neurophysiological study. Brain 111:95–114PubMedCrossRefGoogle Scholar
  15. 15.
    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 neuro pathies. Neurology 67:1837–1843PubMedCrossRefGoogle Scholar
  16. 16.
    Koga M, Gilbert M, Li J, Koike S, Takahashi M, Furukawa K, Hirata K, Yuki N (2005) Antecedent infections in Fisher syndrome: a common pathogenesis of molecular mimicry. Neurology 64:1605–1611PubMedCrossRefGoogle Scholar
  17. 17.
    Koga M, Koike S, Hirata K, Yuki N (2005) Ambiguous value of Haemophilus influenzae isolation in Guillain- Barré and Fisher syndromes. J Neurol Neurosurg Psychiatry 76:1736–1738PubMedCrossRefGoogle Scholar
  18. 18.
    Koga M, Takahashi M, Masuda M, Hirata K, Yuki N (2005) Campylobacter gene polymorphism as a determinant of clinical features of Guillain-Barré syndrome. Neurology 65:1376–1381PubMedCrossRefGoogle Scholar
  19. 19.
    Kusunoki S, Chiba A, Kanazawa I (1999) Anti-GQ1b IgG antibody is associated with ataxia as well as ophthalmoplegia. Muscle Nerve 22: 1071–1074PubMedCrossRefGoogle Scholar
  20. 20.
    Kuwabara S, Asahina M, Nakajima M, Mori M, Fukutake T, Hattori T, Yuki N (1999) Special sensory ataxia in Miller Fisher syndrome detected by postural body sway analysis. Ann Neurol 45:533–536PubMedCrossRefGoogle Scholar
  21. 21.
    Mori M, Kuwabara S, Fukutake T, Hattori T (2007) IV immunoglobulin treatment for Fisher syndrome. Neurology 68:1144–1146PubMedCrossRefGoogle Scholar
  22. 22.
    Mori M, Kuwabara S, Fukutake T, Yuki N, Hattori T (2001) Clinical features and prognosis of Miller Fisher syndrome. Neurology 56:1104–1106PubMedGoogle Scholar
  23. 23.
    Najim Al-Din AS, Anderson M, Eeg-Olofsson O, Trontelj JV (1994) Neuro-ophthalmic manifestations of the syndrome of ophthalmoplegia, ataxia and areflexia: observations on 20 patients. Acta Neurol Scand 89:87–94PubMedCrossRefGoogle Scholar
  24. 24.
    Najim Al-Din AS, Trontelj JV, Anderson M, Eeg-Olofsson O (1993) Ataxia and areflexia in SOAA. Acta Neurol Scand 88:363–369PubMedCrossRefGoogle Scholar
  25. 25.
    Odaka M, Yuki N, Tatsumoto M, Tateno M, Hirata K (2004) Ataxic Guillain- Barré syndrome associated with anti-GM1b and anti-GalNAc-GD1a antibodies. J Neurol 251:24–29PubMedCrossRefGoogle Scholar
  26. 26.
    Odaka M, Yuki N, Yamada M, Koga M, Takemi T, Hirata K, Kuwabara S (2003) Bickerstaff 's brainstem encephalitis: clinical features of 62 cases and a subgroup associated with Guillain-Barré syndrome. Brain 126:2279–2290PubMedCrossRefGoogle Scholar
  27. 27.
    Ogawara K, Kuwabara S, Yuki N (2002) Fisher syndrome or Bickerstaff brainstem encephalitis? : anti-GQ1b IgG antibody syndrome involving both the peripheral and central nervous systems. Muscle Nerve 26:845–849PubMedCrossRefGoogle Scholar
  28. 28.
    Overell J, Hsieh S, Odaka M, Yuki N, Willison H (2007) Treatment for Fisher syndrome, Bickerstaff 's brainstem encephalitis and related disorders. Cochrane Database Syst Rev CD004761Google Scholar
  29. 29.
    Raphaël JC, Chevret S, Hughes RAC, Annane D (2002) Plasma exchange for Guillain-Barré syndrome. Cochrane Database Syst Rev CD001798Google Scholar
  30. 30.
    Ropper AH (1983) The CNS in Guillain-Barré syndrome. Arch Neurol 40:397–398PubMedGoogle Scholar
  31. 31.
    Sauron B, Bouche P, Cathala HP, Chain F, Castaigne P (1984) Miller Fisher syndrome: clinical and electrophysiologic evidence of peripheral origin in 10 cases. Neurology 34:953–956PubMedGoogle Scholar
  32. 32.
    van Breemen VL, Clemente CD (1955) Silver deposition in the central nervous system and the hematoencephalic barrier studied with the electron microscope. J Biophys Biochem Cytol 1:161–166PubMedCrossRefGoogle Scholar
  33. 33.
    Yuki N (2001) Infectious origins of, and molecular mimicry in, Guillain- Barré and Fisher syndromes. Lancet Infect Dis 1:29–37PubMedCrossRefGoogle Scholar
  34. 34.
    Yuki N, Koga M (2006) Bacterial infections in Guillain-Barré and Fisher syndromes. Curr Opin Neurol 19:451–457PubMedCrossRefGoogle Scholar
  35. 35.
    Yuki N, Sato S, Tsuji S, Hozumi I, Miyatake T (1993) An immunologic abnormality common to Bickerstaff ’s brain stem encephalitis and Fisher's syndrome. J Neurol Sci 118:83–87PubMedCrossRefGoogle Scholar
  36. 36.
    Yuki N, Susuki K, Hirata K (2000) Ataxic form of Guillain-Barré syndrome associated with anti-GD1b IgG antibody. J Neurol Neurosurg Psychiatry 69:136–137PubMedCrossRefGoogle Scholar
  37. 37.
    Yuki N, Tagawa Y, Irie F, Hirabayashi Y, Handa S (1997) Close association of Guillain-Barré syndrome with antibodies to minor monosialogangliosides GM1b and GM1α. J Neuroimmunol 74:30–34PubMedCrossRefGoogle Scholar

Copyright information

© Steinkopff-Verlag 2008

Authors and Affiliations

  • M. Ito
    • 1
  • S. Kuwabara
    • 2
  • M. Odaka
    • 1
  • S. Misawa
    • 2
  • M. Koga
    • 1
  • K. Hirata
    • 1
  • N. Yuki*
    • 1
    • 3
  1. 1.Dept. of NeurologyDokkyo Medical UniversityShimotsuga, TochigiJapan
  2. 2.Dept. of NeurologyChiba University School of MedicineChibaJapan
  3. 3.GBS LaboratoryTokyoJapan

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