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      Clinical features of Guillain–Barré syndrome patients with elevated serum creatine kinase levels

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          Abstract

          Background

          It is not well defined whether Guillain–Barré syndrome (GBS) patients with elevated serum creatine kinase (CK) levels have characteristic clinical features and are related to the subgroups of GBS.

          Methods

          We retrospectively studied 51 consecutive patients with GBS, who visited our hospital, and compared clinical, laboratory and electrophysiological findings between patients with and without elevated CK levels.

          Results

          Of 51 patients, 14 patients (27%) showed an elevation of serum CK levels. When compared with patients with the normal CK levels, the ratios of male, antecedent infections, and anti-GM1 antibody positivity were significantly higher in patients with elevated CK levels. The ratios of hypoesthesia, cranial nerve involvement, and urinary retention were significantly less in patients with elevated CK levels. There were no significant differences in disability at peak between two groups. In the electrophysiological examination, sensory nerve abnormalities were not observed. Although some patients with elevated CK levels showed prolongation of distal motor latencies (DMLs) and increase of durations in the initial examination, development of the prolongation of DMLs and increase of durations was not observed in the follow-up examinations. The findings were consistent with acute motor axonal neuropathy (AMAN) with reversible conduction failure (RCF) but not acute inflammatory demyelinating polyneuropathy (AIDP).

          Conclusions

          The results suggest that the GBS patients with elevated CK levels represent not a group of AIDP but a group of AMAN with axonal degeneration or RCF even though the initial electrophysiological examination shows AIDP pattern.

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          Most cited references29

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          An acute axonal form of Guillain-Barré polyneuropathy.

          Five patients with a clinical diagnosis of acute Guillain-Barré polyneuropathy (GBP) had electrically inexcitable motor nerves. All were quadriplegic. One patient died and 3 of the 4 survivors showed poor recovery. Autopsy studies on the patient who died showed severe axonal degeneration in nerve roots and distal nerves without inflammation or demyelination. Electrophysiological studies in these patients suggested that the predominant process was axonal degeneration. These cases may represent a separate clinicopathological entity, and constitute a variant of GBP characterized by an acute axonal neuropathy.
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            Pathology of the motor-sensory axonal Guillain-Barré syndrome.

            The concept of a severe motor-sensory neuropathy of acute onset caused by an immune attack on the axon ("axonal" Guillain-Barré syndrome) has been advanced primarily based on electrodiagnostic and limited pathological data, but remains controversial. At autopsy some cases demonstrate unusually severe inflammatory demyelinating neuropathy. There are conflicting data about whether antecedent Campylobacter jejuni infection is associated with "axonal" Guillain-Barré syndrome. We report 4 individuals from Hebei Province, China, who died 7, 7, 18, and 60 days after onset of a syndrome diagnosed clinically as Guillain-Barré syndrome. High titers of antibodies recognizing C. jejuni, consistent with recent infection, were found in the 2 patients tested. At autopsy the 3 with early disease had ongoing wallerian-like degeneration of fibers in the ventral and dorsal roots and in the peripheral nerves, with only minimal demyelination or lymphocytic infiltration. All 3 had numerous macrophages in the periaxonal space of myelinated internodes, and rare intraaxonal macrophages as well. Examination of the patient having the syndrome for 60 days confirmed the extensive loss of large fibers in the spinal roots and nerves, and the paucity of demyelination and remyelination. These observations confirm predictions that some patients with severe motor-sensory Guillain-Barré syndrome, as defined clinically, have predominantly axonal lesions of both motor and sensory fibers, even in the early stages of the disease, and that axonal Guillain-Barré syndrome can follow C. jejuni infection. The pathology supports the possibility that such cases of motor-sensory axonal Guillain-Barré syndrome represent the most severe end of a spectrum of immune attack directed toward epitopes on the axon.
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              IgG anti-GM1 antibody is associated with reversible conduction failure and axonal degeneration in Guillain-Barré syndrome.

              To investigate the pathophysiological role of anti-GM1 antibody in Guillain-Barré syndrome (GBS), we reviewed sequential nerve conduction studies of 345 nerves in 34 GBS patients. Statistically significant correlation between IgG anti-GM1 antibodies and electrodiagnoses was found. Sixteen IgG anti-GM1-positive patients were classified as having acute motor or acute motor sensory axonal neuropathy (AMAN or AMSAN) (12 patients), as having acute inflammatory demyelinating polyneuropathy (AIDP) (3 patients), or as undetermined (1 patient) by electrodiagnostic criteria. Besides axonal features, there was rapid resolution of conduction slowing and block. In 3 patients initially diagnosed as having AIDP, conduction slowing was resolved within days, and 1 of them and 3 AMAN patients showed markedly rapid increases in amplitudes of distal compound muscle action potentials that were not accompanied by prolonged duration and polyphasia. The time courses of conduction abnormalities were distinct from those in IgG anti-GM1-negative AIDP patients. Rapid resolution of conduction slowing and block, and the absence of remyelinating slow components, suggest that conduction failure may be caused by impaired physiological conduction at the nodes of Ranvier. Reversible conduction failure as well as axonal degeneration constitutes the pathophysiological mechanisms in IgG anti-GM1-positive GBS. In both cases, immune-mediated attack probably occurs on the axolemma of motor fibers.
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                Author and article information

                Contributors
                in1237@osaka-med.ac.jp
                Journal
                BMC Neurol
                BMC Neurol
                BMC Neurology
                BioMed Central (London )
                1471-2377
                27 May 2020
                27 May 2020
                2020
                : 20
                : 214
                Affiliations
                [1 ]GRID grid.444883.7, ISNI 0000 0001 2109 9431, Division of Neurology, Department of Internal Medicine IV, , Osaka Medical College, ; 2-7 Daigaku-machi, Takatsuki, Osaka, 569-8686 Japan
                [2 ]GRID grid.414144.0, ISNI 0000 0004 0384 3492, Department of Internal Medicine, , Hirakata City Hospital, ; 2-14-1 Kinya-honnmachi, Hirakata, Osaka, 573-1013 Japan
                [3 ]GRID grid.260969.2, ISNI 0000 0001 2149 8846, Division of Neurology, Department of Medicine, , Nihon University School of Medicine, ; 30-1 Oyaguchi Kami-cho, Itabashi-ku, Tokyo, 173-8610 Japan
                Author information
                http://orcid.org/0000-0001-5733-9476
                Article
                1796
                10.1186/s12883-020-01796-z
                7251688
                32460711
                6a2a4fb8-66a2-47e1-a1b1-b2aec153f010
                © The Author(s) 2020

                Open AccessThis article is licensed under a Creative Commons Attribution 4.0 International License, which permits use, sharing, adaptation, distribution and reproduction in any medium or format, as long as you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons licence, and indicate if changes were made. The images or other third party material in this article are included in the article's Creative Commons licence, unless indicated otherwise in a credit line to the material. If material is not included in the article's Creative Commons licence and your intended use is not permitted by statutory regulation or exceeds the permitted use, you will need to obtain permission directly from the copyright holder. To view a copy of this licence, visit http://creativecommons.org/licenses/by/4.0/. The Creative Commons Public Domain Dedication waiver ( http://creativecommons.org/publicdomain/zero/1.0/) applies to the data made available in this article, unless otherwise stated in a credit line to the data.

                History
                : 25 January 2020
                : 19 May 2020
                Categories
                Research Article
                Custom metadata
                © The Author(s) 2020

                Neurology
                creatine kinase,guillain-barré syndrome,aidp,aman,reversible conduction failure
                Neurology
                creatine kinase, guillain-barré syndrome, aidp, aman, reversible conduction failure

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