https://imcjms.com/public Ibrahim Medical College Journal of Medical Science https://imcjms.com/public/registration/journal_full_text/113 Mon, 10 Oct 2016 12:08:20 +0000 Guillain-Barré Syndrome (GBS) otherwise known as Acute Inflammatory Polyneuritis, characterized by acute progressive limb weakness and aretlexia, is the prototype of a post infectious autoimmune disease. Two-thirds of the cases of GBS emerge from viral or bacterial infection. In August 2006, a 20 year old man presented at ICU, BIRDEM Hospital with a history of brief icteric illness followed by progressive bilateral symmetrical hypotonic aretlexic muscular weakness, bilateral infra-nuclear facial palsy and bulbar weakness. Later on, he was diagnosed as a case of GBS and acute hepatitis E. Up till now, only three cases of GBS associated with hepatitis E have been reported in the medical literature world wide. This is probably the 4th case to be reported. Address for Correspondence: Prof. Rawshan Ara Khanam, Department of Internal Medicine, Ibrahim Medical College & BIRDEM, 122 Kazi Nazrul Islam Avenue, Shahbag, Dhaka-1000   The incidence of the typical GBS has been reported to be relatively uniform between 0.6 and 4 cases per 100 000 per year thought-out the world1. In two thirds of cases there are occurrences of gastroenteritis or flu like illness within six weeks of onset of GBS2-3. Campylobacter jejuni is reported to be the most frequent antecedent pathogen followed by cytomegalovirus4. Association of GBS with viral hepatitis is well documented. Most of the reported associations are with acute hepatitis B5-6 and hepatitis A7. Few cases also have been reported with Hepatitis C8. Association of GBS with HEV hepatitis is rare. Only three such cases have been published so far9-11. Case Report On admission, he was conscious and mildly icteric. Respiratory rate was 24/min, pulse 100/min, blood pressure 150/80 mm of Hg and temperature 99° F. Systemic examination revealed mild tenderness in right hypochondrium and soft enlarged tender liver about 2 cm from the right costal margin. Neurological examination showed bilateral lower motor neuron (LMN) type facial palsy with evidence of dysphagia. Muscle power was 2/5 in all four limbs with diminished muscle tone. All the deep tendon and superficial reflexes were absent. Planter responses were equivocal. All sensory functions including touch, temperature and vibration were intact and there was no sign of meningeal irritation. Ultrasonography revealed hepatomegaly suggestive of acute hepatitis. Nerve conduction studies revealed both demyelinating and axonopathic sensory motor polyneuropathy. CSF study showed albumin 190 mg/dl with “0” cell count – suggestive of albuminocytologic dissociation.   GBS is a disease of peripheral nervous system, which is caused by aberrant immune response, directed against some components of peripheral nerves12. The targeted antigens may be gangliosides present on plasma membrane of cell (e.g. GM-1, GD-1a), Swchawnn cell neurons (nerve growth cone region). There are four common sub types based on clinical and neuro-physiological studies e.g. 1. Acute Inflammatory Demyelinating Polyneuropathy (AIDP), 2. Acute Motor Axonal Neuropathy (AMAN), 3. Acute Motor Sensory Axonal Neuropathy (AMSAN), 4. Millar Fisher’s Syndrome13. In rare cases, where nerve conduction studies cannot be done because of lack of recordable action potential, nerve biopsy can differentiate the subtypes. However this is done only for research purposes16-17. CSF studies shows raised protein and paucity of cell in 80% cases (albuminocytologic dissociation), in 20% cases CSF study is usually normal in first few days of illness18-19. In this case, albuminocytologic dissociation was present when the CSF was studied four days after admission.   1.  Hubhcs RAC, Rees JH. Clinical and epidemiological features of Guillain-Barré Syndrome. J Infect Dis 1997; 176(suppl): S92-98. 3.  Guillain- Barré Syndrome study group. Guillain-Barré Syndrome: an Italian multicentre case control study. Neurol Sci 2000; 21: 229-34. 5.  Berger JR, Ayyar R, Sharemata WA. Guillain-Barré Syndrome complicating acute hepatitis B. A case with detailed electrophysiological and immunological studies. Arch Neurol 1981; 38: 366-8. 7.  Chitamber SD. Fadnis RS, Joshi MS, Habbu A, Bhatia SG. J Med Virol 2006; 78(8): 1011-4. 9.  Sood A, Midha V, Sood N, Guillain-Barré Syndrome with Acute Hepatitis E. AM J Gastroenterol 2000; 95(12): 3667-8. 11.Kamani P, Baijal R, Amarapurkar D, Gupte P, Patel N, Kumar PH, Agal S. Guillain-Barré Syndrome associated with Acute Hepatitis E. Indian J Gastroenterol 2005; 24: 216. 13.Rchard AC Hughes, David R Cornblath, Lancet 2005; 336: 1653-66. 15.Ho TW, Mishu B Li CY, et al. Guillain-Barré Syndrome in northern China. Relationship to campylobacter jejuni infection and anti glycolipid antibodies. Brain 1995; 118: 597-605. 17.Barciano J, Figols J, Garcia A, et al. Fulminant Guillain-Barré Syndrome with universal excitability of peripheral nerves. A clinicopathological study. Muscle Nerve 1997; 20: 846-57. 19.Hughcs RAC. Guillain-Barré Syndrome. Heidelberg: springerverlag; 1990. 21.Van der Mechc FGA, Schmitz PIM, Dutch. Guillain-Barre Study group. A randomized trial comparing intravenous immunoglobulin and plasma exchange in Guillain-Barre Syndrome, N Engl J Med 1992; 326: 1123-29. 2026 Ibrahim Medical College. All rights reserved.