Acute Facial Dyplegia and Rhabdomyolisis: Case Report and Review of Literature
Clinical Medicine Research
Volume 8, Issue 4, July 2019, Pages: 85-92
Received: Aug. 14, 2019; Accepted: Aug. 26, 2019; Published: Sep. 12, 2019
Views 14      Downloads 9
Maria Sofia Cotelli, Neurology Unit, Azienda Socio Sanitaria Territoriale Valcamonica-Esine, Brescia, Italy
Filippo Manelli, Emergency Unit, Azienda Socio Sanitaria Territoriale Valcamonica-Esine, Brescia, Italy
Marinella Turla, Neurology Unit, Azienda Socio Sanitaria Territoriale Valcamonica-Esine, Brescia, Italy
Article Tools
Follow on us
In 1916, Guillain, Barré and Strohl reported on two cases of acute flaccid paralysis with high cerebrospinal fluid protein levels and normal cell counts-novel findings that identified the disease we now know as Guillain–Barré syndrome (GBS). 100 years on, we have made great progress with the clinical and pathological characterization of GBS. GBS is an acute/subacute-onset polyradiculoneuropathy typically presenting with sensory symptoms and weakness over several days, often leading to quadriparesis. Approximately 70% of patients report a recent preceding upper or lower respiratory tract infection or gastrointestinal illness. The interplay between the microbial and host factors that dictate whether and how the immune response shifts towards autoreactivity is still unclear, and nothing is known about the genetic and environmental factors that affect an individual's susceptibility to the disease. Facial Diplegia with Paresthesias is a rare localized variant of GBS in which patient presents with simultaneous facial diplegia, distal limb paresthesias and minimal or no motor weakness. Treatment with intravenous immunoglobulin or plasma exchange is the optimal management approach, alongside supportive care. A common misconception is that the Guillain–Barré syndrome has a good prognosis-but up to 20% of patients remain severely disabled and approximately 5% die, despite immunotherapy. We report the case of a woman with acute facial dyplegia and rhabdomyolisis improved after immunoglobulin treatment.
Facial Dyplegia, Hyperckemia, Guillain Barrè Syndrome
To cite this article
Maria Sofia Cotelli, Filippo Manelli, Marinella Turla, Acute Facial Dyplegia and Rhabdomyolisis: Case Report and Review of Literature, Clinical Medicine Research. Special Issue: Neurology Emergency. Vol. 8, No. 4, 2019, pp. 85-92. doi: 10.11648/j.cmr.20190804.13
Copyright © 2019 Authors retain the copyright of this article.
This article is an open access article distributed under the Creative Commons Attribution License ( which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.
Harms M Inpatient management of guillain-barré syndrome. Neurohospitalist. 2011 Apr; 1 (2): 78-84.
Hayden RDM, Cornblath DR, Hughes RAC, et al. Electrophysiological classification of Guillain-Barré Syndrome: clinical associations and outcomes. Ann Neurol. 1998; 44 (5): 780–788.
Landry JB. Note sur la paralysie ascendante aigue. Gaz Hebd Med Chir. 1859; 6: 472–474, 486–488.
Osler W. The principles and practice of medicine: designed for the use of practitioners and students of medicine. New York: Appleton; 1892.
Guillain G, Barré J, Strohl A. Sur un syndrome de radiculonévrite avec hyperalbuminose du liquide céphalo-rachidien sans réaction cellulaire. Remarques sur les caractères cliniques et graphiques des réflexes tendineux. Bull Mem Soc Med Hop Paris. 1916; 40: 1462–70.
Haymaker WE, Kernohan JW The Landry-Guillain-Barré syndrome; a clinicopathologic report of 50 fatal cases and a critique of the literature. Medicine (Baltimore). 1949 Feb; 28 (1): 59-141.
Asbury AK, Arnason BG, Adams RD The inflammatory lesion in idiopathic polyneuritis. Its role in pathogenesis. Medicine (Baltimore). 1969 May; 48 (3): 173-215.
McGrogan A, Madle GC, Seaman HE, de Vries CS. The epidemiology of Guillain-Barré syndrome worldwide: A systematic literature review. Neuroepidemiology. 2009; 32 (2): 150–163.
Rees JH, Thompson RD, Smeeton NC, Hughes RAC. Epidemiological study of Guillain-Barré syndrome in south east England. J Neurol Neurosurg Pscyh. 1998; 64 (1): 74–77.
Guillain-Barré Study Group Guillain-Barré syndrome: an Italian multicentre case-control study. Neurol Sci. 2000; 21 (4): 229–234.
Alshekhlee A, Hussain Z, Sultan B, Katirji B. Guillain Barre syndrome: incidence and mortality rates in US hospitals. Neurology. 2008; 70 (18): 1608–1613.
Bersano A, Carpo M, Allaria S, Franciotta D, Citterio A, Nobile-Orazio E. Long term disability and social status change after Guillain-Barré syndrome. J Neurol. 2006; 253 (2): 214–218.
Londono XA, Lewis RA Gullain-Barrè Syndrome Semin Neurol 2012; 32: 179–186.
Hadden RDM, Karch H, Hartung H-P, et al; Plasma Exchange/Sandoglobulin Guillain-Barré Syndrome Trial Group. Preceding infections, immune factors, and outcome in Guillain-Barré syndrome. Neurology 2001; 56 (6): 758–765.
Van Koningsveld R, Rico R, Gerstenbluth I, et al. Gastroenteritis associated Guillain-Barré syndrome on the Caribbean island Curaçao. Neurology 2001; 56 (11): 1467–1472.
Schonberger LB, Bregman DJ, Sullivan-Bolyai JZ, Keenlyside RA, Ziegler DW, Retailliau HF, et al. Guillain-Barre syndrome following vaccination in the National Influenza Immunization Program, United States, 1976-1977. Am J Epidemiol. 1979; 110 (2): 105-23.
Centers for Disease C, Prevention. Preliminary results: surveillance for Guillain-Barre syndrome after receipt of influenza A (H1N1) 2009 monovalent vaccine - United States, 2009-2010. MMWR Morb Mortal Wkly Rep. 2010; 59 (21): 657-61.
Salmon DA, Proschan M, Forshee R, Gargiullo P, Bleser W, Burwen DR, et al. Association between Guillain-Barre syndrome and influenza A (H1N1) 2009 monovalent inactivated vaccines in the USA: a meta-analysis. Lancet. 2013; 381 (9876) 1461-8.
Sanz Fadrique R, Martín Arias L, Molina-Guarneros JA1, Jimeno Bulnes N, García Ortega P. Guillain-Barré syndrome and influenza vaccines: current evidence. Rev Esp Quimioter. 2019 Aug; 32 (4): 288-295. Epub 2019 Jun 20.
Fujimura H The Guillain-Barrè Syndrome Chapter 21. Handbook of Clinical Neurology, Vol. 115 (3rd series) Peripheral Nerve Disorders G. Said and C. Krarup, Editors © 2013 Elsevier B. V.
Bourque PR, Chardon JW, Massie R Autoimmune peripheral neuropathies. Clin Chim Acta. 2015 Sep 20; 449: 37-42.
Hiraga A, Mori M, Ogawara K, Kojima S, KanesakaT, Misawa T, Hattori T, Kuwabara S Recovery patterns and long term prognosis for axonal Guillain–Barré syndrome 2005 May; 76 (5): 719–722.
Kimoto K, Koga M, Odaka M, Hirata K, Takahashi M, Li J, Gilbert M, Yuki N. Relationship of bacterial strains to clinical syndromes of Campylobacter-associated neuropathies. Neurology. 2006 Nov 28; 67 (10): 1837-43.
Yuki N, Ho TW, Tagawa Y, et al. Autoantibodies to GM1b and GalNAc-GD1a: relationship to Campylobacter jejuni infection and acute motor axonal neuropathy in China. J Neurol Sci 1999; 164: 134–8.
Ang CW, Jacobs BC, Laman JD. The Guillain-Barré syndrome: a true case of molecular mimicry. Trends Immunol 2004; 25: 61–6.
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: 1911–7.
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: 677–9.
Nishimoto Y, Odaka M, Hirata K, Yuki N. Usefulness of anti-GQ1b IgG antibody testing in Fisher syndrome compared with cerebrospinal fluid examination. J Neuroimmunol 2004; 148: 200–5.
Liu JX, Willison HJ, Pedrosa-Domellof F. Immunolocalization of GQ1b and related gangliosides in human extraocular neuromuscular junctions and muscle spindles. Invest Ophthalmol Vis Sci 2009; 50: 3226–32.
Wakerley BR, Uncini A, Yuki N, and the GBS Classification Group. Guillain-Barré and Miller Fisher syndromes – new diagnostic classification. Nat Rev Neurol 2014; 10: 537–44.
Wakerley BR, Yuki N. Pharyngeal-cervical-brachial variant of Guillain-Barre syndrome J Neurol Neurosurg Psychiatry. 2014 Mar; 85 (3): 339-44.
Nagashima T, Koga M, Odaka M, Hirata K, Yuki N. Continuous spectrum of pharyngeal-cervical-brachial variant of Guillain- Barré syndrome. Arch Neurol 2007; 64 (10): 1519–1523.
Kashihara K, Shiro Y, Koga M, Yuki N. IgG anti-GT1a antibodies which do not cross react with GQ1b ganglioside in a pharyngealcervical- brachial variant of Guillain-Barré syndrome. J Neurol Neurosurg Psychiatry 1998; 65 (5): 799.
Lee SS, Lee SH. Does sensory Guillain-Barré syndrome exist without any abnormalities in motor nerve conduction? Neurology. 2006; 66: 947–948.
Yang J, Huan M, Jiang H, Song C, Zhong L, Liang Z Pure sensory Guillain-Barré syndrome: A case report and review of the literature Exp Ther Med. 2014 Nov; 8 (5): 1397–1401.
Wakerley BR, Yuki N. Isolated facial diplegia in Guillain-Barré syndrome: Bifacial weakness with paresthesias. Muscle Nerve. 2015; 52 (6): 927–32.
Susuki K, Koga M, Hirata K, Isogai E, Yuki N. A Guillain-Barré syndrome variant with prominent facial diplegia. J Neurol. 2009; 256 (11): 1899–905.
Dal Verme A, Acosta P, Margan M, Pagnini C, Dellepiane E, Peralta C. Facial diplegia with atypical paresthesia. A variant of Guillain-Barré syndrome. Medicina. 2015; 75 (3): 178–80.
Akarsu EO, Yalcın D, Surmeli R, Demir A, Sunter G, Diler Y. A rare variant of guillain-barre syndrome: facial diplegia paresthesia. Turk J Neurol. 2015; 21: 171–74.
Koike H, Atsuta N, Adachi H, et al. Clinicopathological features of acute autonomic and sensory neuropathy. Brain 2010; 133 (10): 2881–2896.
Esposito S, Longo MR. Guillain-Barré syndrome. Autoimmun Rev. 2017 Jan; 16 (1): 96-101.
Soliven B. Animal models of autoimmune neuropathy. ILAR J 2014; 54: 282–90.
Sudo M, Miyaji K, Späth PJ, Morita-Matsumoto K, Yamaguchi Y, Yuki N. Polyclonal IgM and IgA block in vitro complement deposition mediated by anti-ganglioside antibodies in autoimmune neuropathies. Int Immunopharmacol 2016; 40: 11–5.
Kuwabara S. Guillain–Barré syndrome. Epidemiology, pathophysiology andmanagement. Drugs 2004; 64: 597–610.
Dimachkie MM, Barohn RJ Guillain-Barré Syndrome and Variants Neurol Clin. 2013 May; 31 (2): 491-510.
Asbury AK, Cornblath DR. Assessment of current diagnostic criteria for Guillain-Barré syndrome. Ann Neurol. 1990; 27 (Suppl): S21–S24.
Albers JW, Donofrio PD, McGonagle TK. Sequential electrodiagnostic abnormalities in acute inflammatory demyelinating polyradiculoneuropathy. Muscle Nerve. 1985; 8 (6): 528–539.
Van den Berg B, Walgaard C, Drenthen J, Fokke C, Jacobs BC, van Doorn PA. Guillain-Barré syndrome: pathogenesis, diagnosis, treatment and prognosis. Nat Rev Neurol. 2014 Aug; 10 (8): 469-82.
Fokke, C. et al. Diagnosis of Guillain–Barré syndrome and validation of Brighton criteria. Brain 2014; 137, 33–43.
Nishimoto Y, Odaka M, Hirata K. Usefulness of anti-GQ1b IgG antibody testing in Fisher syndrome compared with cerebrospinal fluid examination. J Neuroimmunol 2014; 148: 200–205.
Mori, M. & Kuwabara, S. Fisher syndrome. Curr. Treat. Options Neurol 2011; 13: 71–78.
Kuwabara, S. & Yuki, N. Axonal Guillain–Barré syndrome: concepts and controversies. Lancet Neurol. 2013; 12: 1180–1188.
Fulbright RK, Erdum E, Sze G et-al. Cranial nerve enhancement in the Guillain-Barré syndrome. AJNR Am J Neuroradiol. 1995; 16 (4): 923-5.
Alkan O, Yildirim T, Tokmak N et-al. Spinal MRI findings of guillain-barré syndrome. J Radiol Case Rep. 2009; 3 (3): 25-8.
Plasma Exchange/Sandoglobulin Guillain- Barré Syndrome Trial Group. Randomised trial of plasma exchange, intravenous immunoglobulin, and combined treatments in Guillain-Barré syndrome. Lancet 1997; 349: 225-30.
The French Cooperative Group on Plasma Exchange in Guillain-Barré Syndrome. Appropriate number of plasma exchanges in Guillain-Barré syndrome. Ann Neurol 1997; 41: 298-306.
Hughes RA, Wijdicks EF, Barohn R, Benson E, Cornblath DR, Hahn AF, Meythaler JM, Miller RG, Sladky JT, Stevens JC. Quality Standards Subcommittee of the American Academy of Neurology. Practice parameter: immunotherapy for Guillain-Barré syndrome: report of the Quality Standards Subcommittee of the American Academy of Neurology. Neurology. 2003 Sep 23; 61 (6): 736–740.
Torres PA, Helmstetter JA, Kaye AM, Kaye AD Rhabdomyolysis: pathogenesis, diagnosis, and treatment. Ochsner J. 2015 Spring; 15 (1): 58-69.
Ropper, A. H. & Shahani, B. T. Pain in Guillain-Barre syndrome. Arch Neurol 1984, 41: 511-514.
Science Publishing Group
1 Rockefeller Plaza,
10th and 11th Floors,
New York, NY 10020
Tel: (001)347-983-5186