Bilateral Ptosis and Limb Myokymia: Regional Variant of Guillain-Barré Syndrome?

Biltateral ptosis and limb myokymia in Guillain-Barré Syndrome?

  • Felix Cheng Dept. of Neurology, Jacobs School of Medicine and Biomedical Sciences, Univ. at Buffalo/SUNY
  • Gil Wolfe Dept. of Neurology, Univ. at Buffalo/SUNY
  • Richard Barohn Dept. of Neurology, University of Missouri School of Medicine
  • Jonathan Katz
Keywords: Guillain-Barré syndrome, ptosis, antiganglioside antibodies, myokymia


Isolated bilateral ptosis as an initial sign of  Guillain-Barré Syndrome (GBS) with subsequent limb paralysis or paresis has been reported.  However, to our knowledge, bilateral ptosis with subsequent limb myokymia but without ophthalmoplegia, facial or extremity weakness has not been observed.  Our patient developed severe bilateral ptosis over several days. There was no facial or extremity weakness although limb myokymia was seen.  Laboratory studies excluded common causes of ptosis and were suggestive of GBS. We believe our patient extends the clinical spectrum of GBS, and that binocular ptosis represents a mild regional variant of this neuropathy.


1. Govoni V, Granieri E: Epidemiology of the Guillain-Barré syndrome. Curr Opin Neurol 2001;14:605–613.
2. Hughes RA, Cornblath DR: Guillain-Barré syndrome. Lancet 2005;366:1653–1666.
3. Ropper AH: Unusual clinical variants and signs in Guillain-Barré syndrome. Arch Neurol 1986;43:1150-1152.
4. Govoni V, Granieri E, Tola MR, Casetta I, Ruppi P, Vaghi L: The frequency of clinical variants of Guillain-Barré syndrome in Ferrara, Italy. J Neurol 1999;246:1010–1014.
5. Ropper AH, Wijdicks EFM, Truax BT: Guillain-Barré Syndrome. Philadelphia, FA Davis, 1991: pp 18-225.
6. Teng HW, Sung JY. Ptosis as the initial presentation of Guillain-Barré syndrome. J Emerg Med. 2012;43:e283–5.
7. Talebian A, Soltani B, Talebian M. Bilateral ptosis as the first presentation of Guillain-Barré syndrome. Iran J Child Neurol. 2016;10:70–72.
8. Gutmann L: AAEM minimonograph #37: facial and limb myokymia. Muscle Nerve 1991;14:1043-1049.
9. Roth G, Magistris MR: Neuropathies with prolonged conduction block, single and grouped fasciculations, localized limb myokymia. Electroenceph Clin Neurophysiol 1987;67:428-438.
10. Mateer JE, Gutmann L, McComas CF: Myokymia in Guillain-Barré syndrome. Neurology 1983;33:374-376.
11. Albers JW, Allen AA, Bastron JA, Daube JR: Limb myokymia. Muscle Nerve 1981;4:494-504.
12. Hughes RA, Rees JH: Clinical and epidemiologic features of Guillain-Barré syndrome. J Infect Dis 1997;176:92–98.
13. Parry GJ: Guillain-Barré Syndrome.New York, Thieme Medical Publishers, 1993. pp 10-32.
14. Yuki N, Odaka M, Hirata K. Acute ophthalmoparesis (without ataxia) associated with anti-GQ1b IgG antibody: clinical features. Ophthalmology 2001;108:196-200
15. Lee SH, Lim GH, Kim JS, et al. Acute ophthalmoplegia (without ataxia) associated with anti-GQ1b antibody. Neurology 2008;71:426–9.
16. Ropper AH: Further regional variants of acute immune polyneuropathy. Arch Neurol 1994;51:671-675.
17. 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.
18. Illa I, Ortiz N, Gallard E, Juarez C, Grau JM, Dalakas MC: Acute axonal Guillain-Barré syndrome with IgG antibodies against motor axons following parenteral gangliosides. Ann Neurol 1995;38:218-224.
19. Ilyas AA, Mithen FA, Dalakas MC, Chen Z, Cook SD: Antibodies to acidic glycolipids in Guillain-Barré syndrome and chronic inflammatory demyelinating polyneuropathy. J Neurol Sci 1992;107:111-121.
20. Nobile-Orazio E, Carpo M, Meucci N, et al: Guillain-Barré syndrome associated with high titers of anti-GM1 antibodies. J Neurol Sci 1992;109:200-206.
21. Rees JH, Gregson NA, Hughes RAC: Anti-ganglioside GM1 antibodies in Guillain-Barré syndrome and their relationship to Campylobacter jejuni infection. Ann Neurol 1995;38:809-816.
How to Cite
Cheng, F., Wolfe, G., Barohn, R., & Katz, J. (2020). Bilateral Ptosis and Limb Myokymia: Regional Variant of Guillain-Barré Syndrome? Biltateral ptosis and limb myokymia in Guillain-Barré Syndrome?. RRNMF Neuromuscular Journal, 1(2), 22-24. Retrieved from
Clinic Stuff (Case Reports)