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      Guillain-Barre syndrome masquerading as acute respiratory failure in an infant

      case-report

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          Abstract

          Guillain-Barré syndrome (GBS) is a rare entity in infants. We report a case of GBS in a 5-month-old girl. The child presented with cough, loose stools, breathing difficulty, and listlessness. The child was treated as pneumonia with respiratory failure. Due to difficulty in weaning from ventilation with areflexia, marked hypotonia, and reduced power in all four limbs; possibilities of spinal muscular atrophy, poliomyelitis, and myopathies were kept. Nerve conduction velocity study was suggestive of mixed sensory-motor, severe axonal, and demyelinating polyradiculoneuropathy. Cerebrospinal fluid study revealed albuminocytological dissociation. Child was diagnosed as GBS and treated with intravenous immunoglobulin. Child recovered completely on follow-up. GBS should be considered as a differential diagnosis in acute onset respiratory failure with neuromuscular weakness in infants.

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          Guillain-Barré syndrome: epidemiology, pathophysiology and management.

          Satoshi Kuwabara (2003)
          Guillain-Barré syndrome (GBS) is clinically defined as an acute peripheral neuropathy causing limb weakness that progresses over a time period of days or, at the most, up to 4 weeks. GBS occurs throughout the world with a median annual incidence of 1.3 cases per population of 100 000, with men being more frequently affected than women. GBS is considered to be an autoimmune disease triggered by a preceding bacterial or viral infection. Campylobacter jejuni, cytomegalovirus, Epstein-Barr virus and Mycoplasma pneumoniae are commonly identified antecedent pathogens. In the acute motor axonal neuropathy (AMAN) form of GBS, the infecting organisms probably share homologous epitopes to a component of the peripheral nerves (molecular mimicry) and, therefore, the immune responses cross-react with the nerves causing axonal degeneration; the target molecules in AMAN are likely to be gangliosides GM1, GM1b, GD1a and GalNAc-GD1a expressed on the motor axolemma. In the acute inflammatory demyelinating polyneuropathy (AIDP) form, immune system reactions against target epitopes in Schwann cells or myelin result in demyelination; however, the exact target molecules in the case of AIDP have not yet been identified. AIDP is by far the most common form of GBS in Europe and North America, whereas AMAN occurs more frequently in east Asia (China and Japan). The prognosis of GBS is generally favourable, but it is a serious disease with a mortality of approximately 10% and approximately 20% of patients are left with severe disability. Treatment of GBS is subdivided into: (i) the management of severely paralysed patients with intensive care and ventilatory support; and (ii) specific immunomodulating treatments that shorten the progressive course of GBS, presumably by limiting nerve damage. High-dose intravenous immunoglobulin (IVIg) therapy and plasma exchange aid more rapid resolution of the disease. The predominant mechanisms by which IVIg therapy exerts its action appear to be a combined effect of complement inactivation, neutralisation of idiotypic antibodies, cytokine inhibition and saturation of Fc receptors on macrophages. Corticosteroids alone do not alter the outcome of GBS.
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            Epidemiologic study of Guillain-Barré syndrome in children <15 years of age in Latin America.

            Juan Castro, Priscila Cilene Leon Bueno de Camargo, Julio C. Castillo … (1997)
            In 1986, surveillance of acute flaccid paralysis (AFP) cases among children <15 years of age was implemented in Latin America as part of the initiative to eradicate poliomyelitis from the Western Hemisphere. Data on AFP, including Guillain-Barré syndrome (GBS), could be analyzed from a regional registry system and from specific GBS studies in seven countries. Between 1989 and 1991, 3112 cases of GBS were reported in Latin America, representing 52% of all nonpolio AFP cases. From the studies in seven countries, a total of 1527 GBS cases (49%) were studied, representing an overall annual incidence rate of 0.91/100,000 children <15 years old. Follow-up investigations showed a persistent muscular weakness at 60 days, 6 months, and 1 year after onset in 61%, 14%, and 10% of children, respectively. This study confirms that with the disappearance of polio, GBS arises as the most common cause of AFP.
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              A 3-month-old baby with H1N1 and Guillain-Barré syndrome.

              Alexandra Vasconcelos, Francisco Abecasis, Rita Monteiro … (2012)
              Majority of children with pandemic influenza A (H1N1)pdm09 experience mild illness with full recovery without treatment. A previously healthy two and a half month-old girl was admitted to our paediatric intensive care unit because of severe respiratory failure with A (H1N1)pdm09 infection. Despite initial clinical improvement all attempts to extubate to non-invasive ventilation were unsuccessful and 2 to 3 weeks after symptom onset she started periods of cardiovascular instability and a progressive neurological deterioration with distal symmetrical progressive motor weakness and areflexia. All investigations were normal except elevated liver enzymes and cerebrospinal fluid examination that revealed elevated protein without pleocytosis. A possible diagnosis of Guillain-Barré syndrome (GBS) was considered and electromyogram was compatible with axonal form of GBS. To our knowledge this is the youngest case of GBS acquired postnatally and the first in children associated with H1N1 virus.
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                Author and article information

                Journal
                Journal ID (nlm-ta): J Pediatr Neurosci
                Journal ID (iso-abbrev): J Pediatr Neurosci
                Journal ID (publisher-id): JPN
                Title: Journal of Pediatric Neurosciences
                Publisher: Medknow Publications & Media Pvt Ltd (India )
                ISSN (Print): 1817-1745
                ISSN (Electronic): 1998-3948
                Publication date (Print): Oct-Dec 2015
                Volume: 10
                Issue: 4
                Pages: 399-400
                Affiliations
                [1]Pediatric Intensive Care Unit, B. L. Kapur Super Speciality Hospital, New Delhi, India
                Author notes
                Address for correspondence: Dr. Pradeep Kumar Sharma, Flat No. 48, Pocket 7, Sector 21, Rohini, New Delhi - 110 086, India. E-mail: drsharma025@ 123456gmail.com
                Article
                Publisher ID: JPN-10-399
                DOI: 10.4103/1817-1745.174461
                PMC ID: 4770662
                PubMed ID: 26962356
                SO-VID: 5829c470-99d7-43a1-8add-bc792396d20d
                Copyright © Copyright: © Journal of Pediatric Neurosciences
                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.

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                Subject: Case Report

                ScienceOpen disciplines: Neurosciences
                Keywords: acute flaccid paralysis,guillain-barré syndrome,infant,respiratory failure
                Data availability:
                ScienceOpen disciplines: Neurosciences
                Keywords: acute flaccid paralysis, guillain-barré syndrome, infant, respiratory failure

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