Advertisement

Neurological Sciences

, Volume 40, Issue 1, pp 67–73 | Cite as

Atypical clinical manifestations of Miller Fisher syndrome

  • Jae Ho Jung
  • Eun Hye Oh
  • Jin-Hong Shin
  • Dae-Seong Kim
  • Seo-Young Choi
  • Kwang-Dong Choi
  • Jae-Hwan ChoiEmail author
Original Article
  • 142 Downloads

Abstract

Miller Fisher syndrome (MFS) is characterized by a clinical triad of ophthalmoplegia, ataxia, and areflexia, and is closely associated with serum anti-GQ1b antibody. Although the clinical triad is the cardinal diagnostic clue, a variety of other symptoms and signs beyond the triad have been reported. To elucidate the frequency and characteristics of atypical clinical manifestations of MFS, we recruited 38 patients with MFS and evaluated the symptoms or signs beyond the classic triad. Eleven (29%) of 38 patients had atypical clinical manifestations of MFS such as headache (n = 6), delayed facial palsy (n = 3), divergence insufficiency (n = 2), and taste impairment (n = 2). Headache was localized to the periorbital (n = 3), temporal (n = 2), or whole (n = 1) area. Only one of them showed bilateral papilledema and an elevated opening pressure in cerebrospinal fluid analysis. Delayed facial palsy developed after the other signs have reached nadir (n = 1) or started to improve (n = 2), and did not follow a pattern of descending paralysis with other cranial neuropathies. Two patients showed divergence insufficiency without external ophthalmoplegia, and another two had taste impairment over the entire tongue without the other signs of facial and glossopharyngeal nerve involvements. Our study shows that approximately 30% of MFS patients can have atypical clinical manifestations beyond the classic triad. These results reflect the broad clinical spectrum of MFS, and might be associated with the presence of additional antiganglioside antibodies besides anti-GQ1b in patients with MFS.

Keywords

Miller Fisher syndrome Headache Delayed facial palsy Anti-GQ1b antibody 

Notes

Compliance with ethical standards

All experiments followed the tenets of the Declaration of Helsinki and were approved by the Institutional Review Board of Pusan National University Yangsan Hospital. Informed contents were obtained after the nature and possible consequence of this study had been explained to participants.

Conflict of interest

The authors declare that they have no competing interests.

References

  1. 1.
    FISHER M (1956) An unusual variant of acute idiopathic polyneuritis (syndrome of ophthalmoplegia, ataxia and areflexia). N Engl J Med 255:57–65CrossRefGoogle Scholar
  2. 2.
    Wakerley BR, Uncini A, Yuki N (2014) Guillain-Barré and Miller Fisher syndromes - new diagnostic classification. Nat Rev Neurol 10:537–544CrossRefGoogle Scholar
  3. 3.
    Sekiguchi Y, Mori M, Misawa S, Sawai S, Yuki N, Beppu M, Kuwabara S (2016) How often and when Fisher syndrome is overlapped by Guillain-Barré syndrome or Bickerstaff brainstem encephalitis? Eur J Neurol 23:1058–1063CrossRefGoogle Scholar
  4. 4.
    Hu Q, Li H, Tian J, Zhang B (2018) Bulbar paralysis associated with Miller-Fisher syndrome and its overlaps in Chinese patients. Neurol Sci 39:305–311CrossRefGoogle Scholar
  5. 5.
    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 43:1911–1917CrossRefGoogle Scholar
  6. 6.
    Chiba A, Kusunoki S, Obata H, Machinami R, Kanazawa I (1997) Ganglioside composition of the human cranial nerves, with special reference to pathophysiology of Miller Fisher syndrome. Brain Res 745:32–36CrossRefGoogle Scholar
  7. 7.
    Koga M, Yuki N, Hirata K (2000) Pain in Miller Fisher syndrome. J Neurol 247:720–721CrossRefGoogle Scholar
  8. 8.
    Friedman DI, Potts E (2007) Headache associated with Miller Fisher syndrome. Headache 47:1347–1348CrossRefGoogle Scholar
  9. 9.
    Kim JK, Kim B-J, Shin HY, Shin KJ, Nam TS, Seok JI, Suh BC, Oh J, Kim YJ, Bae JS (2015) Does delayed facial involvement implicate a pattern of “descending reversible paralysis” in Fisher syndrome? Clin Neurol Neurosurg 135:1–5CrossRefGoogle Scholar
  10. 10.
    Tan CY, Yuki N, Shahrizaila N (2015) Delayed facial palsy in Miller Fisher syndrome. J Neurol Sci 358:409–412CrossRefGoogle Scholar
  11. 11.
    Biotti D, Boucher S, Ong E, Tilikete C, Vighetto A (2013) Optic neuritis as a possible phenotype of anti-GQ1b/GT1a antibody syndrome. J Neurol 260:2890–2891CrossRefGoogle Scholar
  12. 12.
    Ohe Y, Shintani D, Kato Y, Tanahashi N (2012) Fisher syndrome with taste impairment. Intern Med 51:2977–2979CrossRefGoogle Scholar
  13. 13.
    Yagi Y, Yokote H, Watanabe Y, Amino T, Kamata T, Kusunoki S (2015) Taste impairment in Miller Fisher syndrome. Neurol Sci 36:809–810CrossRefGoogle Scholar
  14. 14.
    Mori M, Kuwabara S, Fukutake T, Yuki N, Hattori T (2001) Clinical features and prognosis of Miller Fisher syndrome. Neurology 56:1104–1106CrossRefGoogle Scholar
  15. 15.
    Meyer W, Schneider B, Nobile Orazio E, Klotz M, Schlumberger W, Stoker W (2002) EUROLINE anti-ganglioside-profile: a new membrane test for detection of antibodies against gangliosides. Autoimmun Rev 1:71Google Scholar
  16. 16.
    Farmakidis C, Inan S, Milstein M, Herskovitz S (2015) Headache and pain in Guillain-Barré syndrome. Curr Pain Headache Rep 19:40CrossRefGoogle Scholar
  17. 17.
    Lolekha P, Phanthumchinda K (2009) Miller-Fisher syndrome at King Chulalongkorn Memorial Hospital. J Med Assoc Thail 92:471–477Google Scholar
  18. 18.
    Gold DR, Zee DS (2015) Neuro-ophthalmology and neuro-otology update. J Neurol 262:2786–2792CrossRefGoogle Scholar
  19. 19.
    Pinchoff BS, Slavin ML, Rosenstein D, Hyman R (1986) Divergence paralysis as the initial sign in the Miller Fisher syndrome. Am J Ophthalmol 101:741–742CrossRefGoogle Scholar
  20. 20.
    Oguro H, Yamaguchi S, Abe S, Bokura H, Kobayashi S (2005) Acute divergence paralysis in the Miller Fisher syndrome. Rinsho Shinkeigaku 45:524–526PubMedGoogle Scholar
  21. 21.
    Mays LE (1984) Neural control of vergence eye movements: convergence and divergence neurons in midbrain. J Neurophysiol 51:1091–1108CrossRefGoogle Scholar
  22. 22.
    Hüfner K, Frenzel C, Kremmyda O, Adrion C, Bardins S, Glasauer S, Brandt T, Strupp M (2015) Esophoria or esotropia in adulthood: a sign of cerebellar dysfunction? J Neurol 262:585–592CrossRefGoogle Scholar
  23. 23.
    Kim YK, Kim JS, Jeong SH, Park KS, Kim SE, Park SH (2009) Cerebral glucose metabolism in Fisher syndrome. J Neurol Neurosurg Psychiatry 80:512–517CrossRefGoogle Scholar
  24. 24.
    Blumenfeld H (2010) Neuroanatomy through clinical cases, 2nd edn. Sunderland: Sinauer AssociatesGoogle Scholar

Copyright information

© Springer-Verlag Italia S.r.l., part of Springer Nature 2018

Authors and Affiliations

  1. 1.Department of OphthalmologySeoul National University HospitalSeoulSouth Korea
  2. 2.Department of Neurology, Pusan National University School of Medicine, Research Institute for Convergence of Biomedical Science and TechnologyPusan National University Yangsan HospitalYangsanSouth Korea
  3. 3.Department of Neurology, Pusan National University School of Medicine and Biomedical Research InstitutePusan National University HospitalBusanSouth Korea

Personalised recommendations