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Fisher Syndrome

  • Neurologic Ophthalmology and Otology
  • Published:
Current Treatment Options in Neurology Aims and scope Submit manuscript

Opinion statement

Fisher syndrome is characterized by the clinical triad of ophthalmoplegia, ataxia, and areflexia. It is considered a variant form of Guillain-Barré syndrome, which is associated with anti-GQ1b antibodies. During initial examinations of patients, physicians must rule out other neurologic disorders or conditions that resemble Fisher syndrome, such as vitamin B1 deficiency (Wernicke’s encephalopathy), vascular disease, multiple sclerosis, collagen disease, Behçet disease, sarcoidosis, neoplasm of the brainstem, and infectious diseases such as diphtheria, botulism, and viral infections (eg, herpes encephalitis). The acute phase of Fisher syndrome should be carefully observed to see if it occurs concomitantly with Guillain-Barré syndrome or if there is development to Bickerstaff brainstem encephalitis, as these require specific immune treatments. Typically, Fisher syndrome has a fairly good natural course. Although several reports have suggested the possible efficacy of immunotherapies such as plasmapheresis and intravenous immunoglobulins (IVIg) in treating Fisher syndrome, there have been no randomized controlled studies. Large retrospective studies have suggested that neither plasmapheresis nor IVIg alters the clinical outcome of patients with Fisher syndrome, probably because of the good spontaneous recovery in these patients. Therefore, Fisher syndrome alone does not necessarily require immunotherapy. To accelerate the start of recovery, IVIg can be given, but it is important to first obtain informed consent from patients after the potential risks of blood products are explained. When overlap with Guillain-Barré syndrome or development to Bickerstaff brainstem encephalitis occurs, plasma exchange or IVIg should be administered as early as possible because Guillain-Barré syndrome can cause respiratory failure or severe weakness with axonal degeneration, and Bickerstaff brainstem encephalitis may not have as good a natural course as Fisher syndrome alone. There have been no prospective, controlled studies (randomized or nonrandomized) of the use of immunotherapy to treat Fisher syndrome. To evaluate the efficacy of immunotherapies used to treat Fisher syndrome, large prospective studies are required.

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References and Recommended Reading

Papers of particular interest, published recently, have been highlighted as: • Of importance ••Of major importance

  1. Collier J: Peripheral neuritis. Edinburgh Med J 1932, 39:601–618.

    Google Scholar 

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

    Article  CAS  PubMed  Google Scholar 

  3. Chiba A, Kusunoki S, Shimizu T, Kanazawa I: Serum IgG antibody to ganglioside GQ1b is a possible marker of Miller Fisher syndrome. Ann Neurol 1992, 31:677–679.

    Article  CAS  PubMed  Google Scholar 

  4. Mori M, Kuwabara S, Fukutake T, et al.: Clinical features and prognosis of Miller Fisher syndrome. Neurology 2001, 56:1104–1106.

    CAS  PubMed  Google Scholar 

  5. Sauron B, Bouche P, Cathala HP, et al.: Miller Fisher syndrome: clinical and electrophysiologic evidence of peripheral origin in 10 cases. Neurology 1984, 34:953–956.

    CAS  PubMed  Google Scholar 

  6. Lyu RK, Tang LM, Cheng SY, et al.: Guillain-Barré syndrome in Taiwan: a clinical study of 167 patients. J Neurol Neurosurg Psychiatry 1997, 63:494–500.

    Article  CAS  PubMed  Google Scholar 

  7. Yuan CL, Wang YJ, Tsai CP: Miller fisher syndrome: a hospital-based retrospective study. Eur Neurol 2000, 44:79–85.

    Article  CAS  PubMed  Google Scholar 

  8. San-Juan OD, Martinez-Herrera JF, Garcia JM, et al.: Miller fisher syndrome: 10 years’ experience in a third-level center. Eur Neurol 2009, 62:149–154.

    Article  PubMed  Google Scholar 

  9. Berlit P, Rakicky J: The Miller Fisher syndrome. Review of the literature. J Clin Neuroophthalmol 1992, 12:57–63.

    CAS  PubMed  Google Scholar 

  10. Kuitwaard K, van Koningsveld R, Ruts L, et al.: Recurrent Guillain-Barré syndrome. J Neurol Neurosurg Psychiatry 2009, 80:56–59.

    Article  CAS  PubMed  Google Scholar 

  11. Ropper AH, Wijdicks EFM, Truax BT: Guillain-Barré Syndrome. Philadelphia: FA Davis; 1991:106–121.

    Google Scholar 

  12. Rees JH, Soudain SE, Gregson NA, Hughes RAC: Campylobacter jejuni infection and Guillain-Barré syndrome. N Eng J Med 1995, 333:1374–1379.

    Article  CAS  Google Scholar 

  13. Emilia-Romagna Study Group on Clinical and Epidemiological Problems in Neurology: Guillain-Barré syndrome variants in Emilia-Romagna, Italy, 1992–3: incidence, clinical features, and prognosis. J Neurol Neurosurg Psychiatry 1998, 65:218–224.

    Article  Google Scholar 

  14. Funakoshi K, Kuwabara S, Odaka M, et al.: Clinical predictors of mechanical ventilation in Fisher/Guillain-Barré overlap syndrome. J Neurol Neurosurg Psychiatry 2009, 80:60–64.

    Article  CAS  PubMed  Google Scholar 

  15. Odaka M, Yuki N, Yamada M, et al.: Bickerstaff’s brainstem encephalitis: clinical features of 62 cases and a subgroup associated with Guillain-Barré syndrome. Brain 2003, 126:2279–2290.

    Article  PubMed  Google Scholar 

  16. Yuki N, Sato S, Tsuji S, et al.: Frequent presence of anti-GQ1b antibody in Fisher’s syndrome. Neurology 1993, 43:414–417.

    CAS  PubMed  Google Scholar 

  17. Yuki N, Odaka M, Hirata K: Acute ophthalmoparesis (without ataxia) associated with anti-GQ1b IgG antibody: clinical features. Ophthalmology 2001, 108:196–200.

    Article  CAS  PubMed  Google Scholar 

  18. Radzwill AJ, Steck AJ, Borruat FX, Bogousslavsky J: Isolated internal ophthalmoplegia associated with IgG anti-GQ1b antibody. Neurology 1998, 50:307.

    Google Scholar 

  19. Mori M, Kuwabara S, Koga M, et al.: IgG anti-GQ1b positive acute ataxia without ophthalmoplegia. J Neurol Neurosurg Psychiatry 1999, 67:668–670.

    Article  CAS  PubMed  Google Scholar 

  20. Yuki N, Susuki K, Hirata K: Ataxic Guillain-Barré syndrome with anti-GQ1b antibody: relation to Miller Fisher syndrome. Neurology 2000, 54:1851–1853.

    CAS  PubMed  Google Scholar 

  21. O’Leary CP, Veitch J, Durward WF, et al.: Acute oropharyngeal palsy is associated with antibodies to GQ1b and GT1a gangliosides. J Neurol Neurosurg Psychiatry 1996, 61:649–651.

    Article  PubMed  Google Scholar 

  22. Yuki N, Taki T, Takahashi M, et al.: Molecular mimicry between GQ1b ganglioside and lipopolysaccharides of Campylobacter jejuni isolated from patients with Fisher’s syndrome. Ann Neurol 1994, 36:791–793.

    Article  CAS  PubMed  Google Scholar 

  23. Koga M, Yuki N, Tai T, Hirata K: Miller Fisher syndrome and Haemophilus influenzae infection. Neurology 2001, 57:686–691.

    CAS  PubMed  Google Scholar 

  24. Houliston RS, Yuki N, Hirama T, et al.: Recognition characteristics of monoclonal antibodies that are cross-reactive with gangliosides and lipooligosaccharide from Campylobacter jejuni strains associated with Guillain-Barré and Fisher syndromes. Biochemistry 2007, 46:36–44.

    Article  CAS  PubMed  Google Scholar 

  25. Houliston RS, Koga M, Li J, et al.: A Haemophilus influenzae strain associated with Fisher syndrome expresses a novel disialylated ganglioside mimic. Biochemistry 2007, 46:8164–8171.

    Article  CAS  PubMed  Google Scholar 

  26. Kornberg AJ, Pestronk A, Blume GM, et al.: Selective staining of the cerebellar molecular layer by serum IgG in Miller-Fisher and related syndromes. Neurology 1996, 47:1317–1320.

    CAS  PubMed  Google Scholar 

  27. Tüzün E, Kürtüncü M, Lang B, et al.: Bickerstaff’s encephalitis and Miller Fisher syndrome associated with voltage-gated potassium channel and novel anti-neuronal antibodies. Eur J Neurol 2010, 17(10):1304–1307.

    Article  PubMed  Google Scholar 

  28. Ito M, Kuwabara S, Odaka M, et al.: Bickerstaff’s brainstem encephalitis and Fisher syndrome form a continuous spectrum: clinical analysis of 581 cases. J Neurol 2008, 255:674–682.

    Article  CAS  PubMed  Google Scholar 

  29. Kuwabara S, Asahina M, Nakajima M, et al.: Special sensory ataxia in Miller Fisher syndrome detected by postural body sway analysis. Ann Neurol 1999, 45:533–536.

    Article  CAS  PubMed  Google Scholar 

  30. Chiba A, Kusunoki S, Obata H, Machinami R, Kanazawa I: Serum anti-GQ1b IgG antibody is associated with ophthalmoplegia in Miller Fisher syndrome and Guillain-Barré syndrome: clinical and immunohistochemical studies. Neurology 1993, 43:1911–1917.

    CAS  PubMed  Google Scholar 

  31. Liu JX, Willison HJ, Pedrosa-Domellöf F: Immunolocalization of GQ1b and related gangliosides in human extraocular neuromuscular junctions and muscle spindles. Invest Ophthalmol Vis Sci 2009, 50:3226–3232.

    Article  PubMed  Google Scholar 

  32. Lo YL, Fook-Chong S, Chan LL, et al.: Electrophysiological evidence of cerebellar fiber system involvement in the Miller Fisher syndrome. J Neurol Sci 2010, 288:49–53.

    Article  CAS  PubMed  Google Scholar 

  33. Kim YK, Kim JS, Jeong SH, et al.: Cerebral glucose metabolism in Fisher syndrome. J Neurol Neurosurg Psychiatry 2009, 80:512–517.

    Article  CAS  PubMed  Google Scholar 

  34. Ogawara K, Kuwabara S, Yuki N: Fisher syndrome or Bickerstaff brainstem encephalitis? Anti-GQ1b IgG antibody syndrome involving both the peripheral and central nervous systems. Muscle Nerve 2002, 26:845–849.

    Article  PubMed  Google Scholar 

  35. Roberts M, Willison H, Vincent A, Newsom-Davis J: Serum factor in Miller-Fisher variant of Guillain-Barré syndrome and neurotransmitter release. Lancet 1994, 343:454–455.

    Article  CAS  PubMed  Google Scholar 

  36. Halstead SK, Humphreys PD, Zitman FM, et al.: C5 inhibitor rEV576 protects against neural injury in an in vitro mouse model of Miller Fisher syndrome. J Peripher Nerv Syst 2008, 13:228–235.

    Article  PubMed  Google Scholar 

  37. Halstead SK, Zitman FM, Humphreys PD, et al.: Eculizumab prevents anti-ganglioside antibody-mediated neuropathy in a murine model. Brain 2008, 131:1197–1208.

    Article  PubMed  Google Scholar 

  38. Kaida K, Kusunoki S: Antibodies to gangliosides and ganglioside complexes in Guillain-Barré syndrome and Fisher syndrome: mini-review. J Neuroimmunol 2010, 223:5–12.

    Article  CAS  PubMed  Google Scholar 

  39. Arakawa Y, Yoshimura M, Kobayashi S, et al.: The use of intravenous immunoglobulin in Miller Fisher syndrome. Brain Dev 1993, 15:231–233.

    Article  CAS  PubMed  Google Scholar 

  40. Zifko U, Drlicek M, Senautka G, Grisold W: High dose immunoglobulin therapy is effective in the Miller Fisher syndrome. J Neurol 1994, 241:178–179.

    Article  CAS  PubMed  Google Scholar 

  41. Mori M, Kuwabara S, Fukutake T, Hattori T: Intravenous immunoglobulin therapy for Miller Fisher syndrome. Neurology 2007, 68:1144–1146.

    Article  CAS  PubMed  Google Scholar 

  42. Overell JR, Hsieh ST, Odaka M, et al.: Treatment for Fisher syndrome, Bickerstaff’s brainstem encephalitis and related disorders. Cochrane Database Syst Rev 2007, (1):CD004761.

  43. Donofrio PD, Berger A, Brannagan 3rd TH, et al.: Consensus statement: the use of intravenous immunoglobulin in the treatment of neuromuscular conditions report of the AANEM ad hoc committee. Muscle Nerve 2009, 40:890–900.

    Article  CAS  PubMed  Google Scholar 

  44. Littlewood R, Bajada S: Successful plasmapheresis in the Miller-Fisher syndrome. Br Med J (Clin Res Ed) 1981, 282:778.

    Article  CAS  Google Scholar 

  45. Ohtsuka K, Nakamura Y, Tagawa Y, Yuki N: Immunoadsorption therapy for Fisher syndrome associated with IgG anti-GQ1b antibody. Am J Ophthalmol 1998, 125:403–406.

    Article  CAS  PubMed  Google Scholar 

  46. Mori M, Kuwabara S, Fukutake T, Hattori T: Plasmapheresis and Miller Fisher syndrome: analysis of 50 consecutive cases. J Neurol Neurosurg Psychiatry 2002, 72:680.

    Article  CAS  PubMed  Google Scholar 

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Authors and Affiliations

  1. Department of Neurology, Graduate School of Medicine, Chiba University, 1-8-1 Inohana, Chuo-ku, Chiba, 260-8670, Japan

    Masahiro Mori MD, PhD & Satoshi Kuwabara MD, PhD

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  1. Masahiro Mori MD, PhD
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  2. Satoshi Kuwabara MD, PhD
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Correspondence to Masahiro Mori MD, PhD.

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Mori, M., Kuwabara, S. Fisher Syndrome. Curr Treat Options Neurol 13, 71–78 (2011). https://doi.org/10.1007/s11940-010-0103-8

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  • DOI: https://doi.org/10.1007/s11940-010-0103-8

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