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      Guillain–Barré syndrome in pregnancy: A conservatively managed case

      case-report

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          Abstract

          Guillain–Barré syndrome (GBS) is an autoimmune disease. Estimated population incidence ranges from 0.62 to 2.66 cases per 100,000 person-years across all age groups. We report a case of GBS in a 22-year-old primigravida who presented at 36 weeks of the period of gestation (POG), with complaints of bilateral progressive lower limb numbness and weakness for 2 weeks duration. Magnetic resonance imaging of the brain was done to exclude other possible causes. Diagnosis of GBS was made according to the Brighton criteria, which our patient falls into Level 2. She received intensive care management. The patient improved rapidly without any specific management. She went to labor spontaneously and delivered a healthy baby with a birth weight of 2.8 kg at 38 weeks of POG. She continued to receive supportive therapy and improved significantly.

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          Guillain-Barré syndrome.

          Richard Hughes, David Cornblath (2005)
          Guillain-Barré syndrome consists of at least four subtypes of acute peripheral neuropathy. Major advances have been made in understanding the mechanisms of some of the subtypes. The histological appearance of the acute inflammatory demyelinating polyradiculoneuropathy (AIDP) subtype resembles experimental autoimmune neuritis, which is predominantly caused by T cells directed against peptides from the myelin proteins P0, P2, and PMP22. The role of T-cell-mediated immunity in AIDP remains unclear and there is evidence for the involvement of antibodies and complement. Strong evidence now exists that axonal subtypes of Guillain-Barré syndrome, acute motor axonal neuropathy (AMAN), and acute motor and sensory axonal neuropathy (AMSAN), are caused by antibodies to gangliosides on the axolemma that target macrophages to invade the axon at the node of Ranvier. About a quarter of patients with Guillain-Barré syndrome have had a recent Campylobacter jejuni infection, and axonal forms of the disease are especially common in these people. The lipo-oligosaccharide from the C jejuni bacterial wall contains ganglioside-like structures and its injection into rabbits induces a neuropathy that resembles acute motor axonal neuropathy. Antibodies to GM1, GM1b, GD1a, and GalNac-GD1a are in particular implicated in acute motor axonal neuropathy and, with the exception of GalNacGD1a, in acute motor and sensory axonal neuropathy. The Fisher's syndrome subtype is especially associated with antibodies to GQ1b, and similar cross-reactivity with ganglioside structures in the wall of C jejuni has been discovered. Anti-GQ1b antibodies have been shown to damage the motor nerve terminal in vitro by a complement-mediated mechanism. Results of international randomised trials have shown equivalent efficacy of both plasma exchange and intravenous immunoglobulin, but not corticosteroids, in hastening recovery from Guillain-Barré syndrome. Further research is needed to discover treatments to prevent 20% of patients from being left with persistent and significant disability.
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            EFNS guidelines for the use of intravenous immunoglobulin in treatment of neurological diseases: EFNS task force on the use of intravenous immunoglobulin in treatment of neurological diseases.

            B. Udd, R Schaik, N Scolding … (2008)
            Despite high-dose intravenous immunoglobulin (IVIG) is widely used in treatment of a number of immune-mediated neurological diseases, the consensus on its optimal use is insufficient. To define the evidence-based optimal use of IVIG in neurology, the recent papers of high relevance were reviewed and consensus recommendations are given according to EFNS guidance regulations. The efficacy of IVIG has been proven in Guillain-Barré syndrome (level A), chronic inflammatory demyelinating polyradiculoneuropathy (level A), multifocal mononeuropathy (level A), acute exacerbations of myasthenia gravis (MG) and short-term treatment of severe MG (level A recommendation), and some paraneoplastic neuropathies (level B). IVIG is recommended as a second-line treatment in combination with prednisone in dermatomyositis (level B) and treatment option in polymyositis (level C). IVIG should be considered as a second or third-line therapy in relapsing-remitting multiple sclerosis, if conventional immunomodulatory therapies are not tolerated (level B), and in relapses during pregnancy or post-partum period (good clinical practice point). IVIG seems to have a favourable effect also in paraneoplastic neurological diseases (good practice point) [corrected],stiff-person syndrome (level A), some acute-demyelinating diseases and childhood refractory epilepsy (good practice point).
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              IVIG treatment and prognosis in Guillain-Barré syndrome.

              Pieter A van Doorn, Krista Kuitwaard, Christa Walgaard … (2010)
              Guillain-Barré syndrome (GBS) is an acute, immune-mediated polyneuropathy that often leads to severe weakness. Intravenous immunoglobulin (IVIG) is a proven effective treatment for GBS (class 1 evidence). However, about 25% of patients need artificial ventilation and 20% are still unable to walk unaided after 6 months. Important clinical factors associated with poor outcome are age, presence of preceding diarrhea and the severity of disability in the early course of disease. These clinical factors were combined in a clinical prognostic scoring scale, the Erasmus GBS Outcome Scale (EGOS). GBS patients being unable to walk unaided are currently treated with a standard single IVIg dose (0.4 g/kg bodyweight for 5 days). A recent retrospective study in 174 GBS patients enrolled in one of our randomized controlled clinical trials showed that patients with a minor increase of serum IgG level after standard single IVIg dose recovered significantly slower. Additionally, fewer patients reached the ability to walk unaided at six months after correction for the known clinical prognostic factors (multivariate analysis; P < 0.022). It is yet unknown why some GBS patients only have a minor increase after standard IVIg treatment. By using the EGOS it is possible to select GBS patients with a poor prognosis. These patients potentially may benefit from a second IVIg dose. A standard dose of IVIG is not sufficiently effective in many GBS patients. Whether these patients might benefit from a second IVIg dose needs further investigation.
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                Author and article information

                Journal
                Journal ID (nlm-ta): J Family Med Prim Care
                Journal ID (iso-abbrev): J Family Med Prim Care
                Journal ID (publisher-id): JFMPC
                Title: Journal of Family Medicine and Primary Care
                Publisher: Medknow Publications & Media Pvt Ltd (India )
                ISSN (Print): 2249-4863
                ISSN (Electronic): 2278-7135
                Publication date (Print): Jul-Sep 2016
                Volume: 5
                Issue: 3
                Pages: 688-690
                Affiliations
                [1 ] Department of Obstetrics and Gynaecology, University Professorial Unit, De Soysa Maternity Hospital for Women, Colombo 8, Sri Lanka
                [2 ] Department of Obstetrics and Gynaecology, Faculty of Medicine, University of Colombo, Colombo, Sri Lanka
                Author notes
                Address for correspondence: Dr. T. N. M. S. Fernando, University Professorial Unit, De Soysa Maternity Hospital for Women, Colombo 8, Sri Lanka. E-mail: nadeesha.sampath@ 123456gmail.com
                Article
                Publisher ID: JFMPC-5-688
                DOI: 10.4103/2249-4863.197303
                PMC ID: 5290785
                PubMed ID: 28217608
                SO-VID: e8bac24d-e395-4102-a24f-963ef6f43403
                Copyright © Copyright: © Journal of Family Medicine and Primary Care
                License:

                This is an open access article distributed under the terms of the Creative Commons Attribution-NonCommercial-ShareAlike 3.0 License, which allows others to remix, tweak, and build upon the work non-commercially, as long as the author is credited and the new creations are licensed under the identical terms.

                History
                Categories
                Subject: Case Report

                Keywords: conservative management,guillain-barré syndrome,intravenous immunoglobulin,pregnancy
                Data availability:
                Keywords: conservative management, guillain-barré syndrome, intravenous immunoglobulin, pregnancy

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