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      Guillain-Barré syndrome, tuberculosis and inflammatory bowel disease: a multiple association

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          Abstract

          Guillain-Barré syndrome (GBS) has been associated with both infective or non-infective aetiologies. GBS is usually preceded by acute respiratory or gastrointestinal infection but its association with tuberculosis has been exceptionally reported. Inflammatory bowel disease (IBD) is associated with clinical manifestations involving the neurological system, peripheral neuropathy is known to be related to IBD and, either demyelinating or axonal involvement of peripheral nerves have been described. We report an unusual case of GBS associated with lymph node tuberculosis and ulcerative colitis.

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          Guillain-Barré syndrome.

          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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            Peripheral neuropathy in patients with inflammatory bowel disease.

            Peripheral neuropathy (PN) in inflammatory bowel disease (IBD) patients has been reported as individual cases or small series; however, its clinical and electrodiagnostic features have not been well characterized. We conducted a retrospective review of patients with PN and either Crohn's disease (CD) or ulcerative colitis (UC). Eighteen patients with CD and 15 patients with UC were identified after other PN causes were excluded. Male predominance and mean age of PN presentation in the fifties was seen in both groups. Demyelinating neuropathy (CIDP or MMN) occurred in close to 30% of the patients, in a higher percentage of women, than in the non-demyelinating patients. One-third of CD and UC patients had small-fibre or large-fibre sensory axonal PN, while approximately 40% of the CD and UC patients had large-fibre axonal sensorimotor PN. PN symptoms began earlier in the course of CD than in UC (P < 0.05). Patients with large-fibre axonal PN were older than patients with small-fibre sensory axonal PN (P < 0.05). Close to 60% of each group received immunotherapy with different agents. Half of those treated with CD and 40% with UC had demyelinating PN. Most of the patients who completed immunotherapy in both groups improved; all the patients with demyelinating neuropathy had either moderate or major improvement. The PN syndromes in IBD patients are diverse. Demyelinating forms may occur at any time, but early in the IBD course, pure sensory neuropathy is more common. Response to immunotherapy may occur in both demyelinating and axonal neuropathies.
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              Peripheral neuropathy and neurological disorders in an unselected Brazilian population-based cohort of IBD patients.

              Several neurological disorders have been described in inflammatory bowel disease (IBD) patients, but their exact frequency is unknown. We prospectively studied the prevalence of neurological disorders (especially peripheral neuropathy) in a group of 82 patients with Crohn's disease (CD, n = 31) or ulcerative colitis (UC, n = 51) from 2 Brazilian tertiary care university clinics and followed them through a period of at least 1 year. All patients were interviewed and had complete neurological evaluations. Large-fiber sensory or sensorimotor polyneuropathy (PN) was observed in 16.1% of the CD and 19.6% of the UC patients. PN was usually mild, predominantly symmetric, and distal with axonal involvement. One patient had demyelinating PN at the diagnosis of CD. Mild carpal tunnel syndrome was common in female UC patients. Sensory symptoms without electromyography abnormalities, suggestive of small-fiber neuropathy or subclinical myelopathy, affected 29% and 11.8%, respectively. After excluding other known etiological or contributory factors for PN, 13.4% of the IBD patients had otherwise unexplained large-fiber or small-fiber PN (7.3% with large-fiber SM PN). Nondebilitating headache was the most common neurological complaint. Three patients had ischemic strokes, 5 were epileptic, and 1 transient chorea. Neurological disorders, especially PN, are common in our Brazilian cohort of IBD patients. They are diverse, multifactorial, and more common in women. Despite the mild phenotype in most cases, attention should be given by the general practitioner and gastroenterologist since they are frequently undiagnosed. Further studies are necessary to confirm these findings in populations with different genetic and nutritional backgrounds.
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                Author and article information

                Journal
                Int Arch Med
                International Archives of Medicine
                BioMed Central
                1755-7682
                2010
                16 July 2010
                : 3
                : 15
                Affiliations
                [1 ]Internal Medicine Service. Hospital San Agustín, Avilés, Asturias, Spain
                [2 ]Neurology Service. Hospital San Agustín, Avilés, Asturias, Spain
                Article
                1755-7682-3-15
                10.1186/1755-7682-3-15
                2912254
                20637069
                aa9aa936-3bf6-4318-9196-590ae2c3429c
                Copyright ©2010 de la Torre et al; licensee BioMed Central Ltd.

                This is an Open Access article distributed under the terms of the Creative Commons Attribution License ( http://creativecommons.org/licenses/by/2.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

                History
                : 23 May 2010
                : 16 July 2010
                Categories
                Case Report

                Medicine
                Medicine

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