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      A Case of Acute Motor Sensory Axonal Neuropathy: A Variant of Guillain-Barré Syndrome, with Possible Syndrome of Irreversible Lithium-Effectuated Neurotoxicity

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          Abstract

          Acute Motor Sensory Axonal Neuropathy (AMSAN) is a rare and severe variant of Guillain-Barré syndrome (GBS) that has a prolonged recovery course. GBS is often suspected due to ascending muscle weakness, sensation difficulties, respiratory compromise, and antecedent diarrhea. The diagnosis of GBS is supported by cerebrospinal fluid analysis showing albuminocytologic dissociation. Electromyogram and nerve conduction study confirm the diagnosis and allow for further classification by variant. Treatment involves either IV immune globulins or plasmapheresis, and patients typically recover. However, depending on the variant and severity, patients may ultimately require prolonged mechanical ventilation with tracheostomy. In these cases, they may continue to have persistent muscle and sensation abnormalities requiring long-term care. We present a unique case of a 38-year-old female patient with decade-long use of lithium for bipolar disorder that presented with acute lithium toxicity. Though she was ultimately diagnosed with AMSAN, the Syndrome of Irreversible Lithium-Effectuated Neurotoxicity (SILENT) may have also contributed to her persistent neurological sequelae.

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          Most cited references7

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          Outcome and its predictors in Guillain-Barre syndrome.

          Despite the use of plasma exchanges and intravenous immunoglobulins, Guillain-Barré syndrome (GBS) still carries non-negligible morbidity and mortality. Furthermore, the psychosocial consequences of GBS may persist longer than expected. Various aetiological, clinical, electrophysiological and immunological factors may carry prognostic predictive value. The objective of this article was to perform a summary of the current knowledge-base on outcome and its determinants in adequately-treated adult-onset GBS. Relevant prospective literature was reviewed through a Medline search of English-language articles published between 1966 and March 2012. GBS causes severe persistent disability in 14% of patients at 1 year. Loss of full strength, persistent pain and need for professional change occurs in about 40%. Mortality is of about 4% within the first year. Analysis of prognostic predictors consistently demonstrates the negative impact of higher age, preceding diarrhoea, greater disability/weaker muscles at admission, short interval between symptom-onset and admission, mechanical ventilation and absent/low amplitude compound muscle action potentials. Further outcome studies will soon be underway and may in future contribute to adequately integrate all potential factors in more reliable predictive models.
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            Plasma exchange for Guillain-Barré syndrome.

            Guillain-Barré syndrome (GBS) is an acute paralysing disease caused by peripheral nerve inflammation. This is an update of a review first published in 2001 and last updated in 2012.
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              The syndrome of irreversible lithium-effectuated neurotoxicity.

              Persistent sequelae of lithium intoxication gained clinical attention in the 1980s and were named Syndrome of Irreversible Lithium-Effectuated Neurotoxicity (SILENT). The authors review the published cases of SILENT reported in the literature and discuss various clinical manifestations. The authors' inclusion criteria included persistence of sequelae for at least 2 months after the cessation of lithium administration. They conducted a MEDLINE and Pub Med search for journal articles from the year 1965 to 2004. They also cross-referenced available papers. The authors identified 90 cases of SILENT in peer-reviewed publications. Persistent cerebellar dysfunction was the most commonly reported sequela. Other atypical presentations have also been reported. Although the biologic mechanism remains unclear, the authors hypothesize that the putative cause of SILENT is demyelination caused by lithium at multiple sites in the nervous system, including the cerebellum. Recent advances in the understanding of the molecular basis of lithium-induced neurotoxicity may be able to provide a means of defining a pathway associated with the long-term prophylactic properties of lithium, distinct from its toxicity profile. This identification of differential gene expression patterns that distinguish between therapeutic and toxic actions of lithium may help in the discovery of new drugs for mood stabilization. Clinically and heuristically, it is important to raise the awareness of this syndrome so that clinicians are able to avoid it. A precise definition, operational diagnostic criteria, and a descriptive name will aid in the early identification and prevention of SILENT.
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                Author and article information

                Contributors
                Journal
                Case Rep Med
                Case Rep Med
                CRIM
                Case Reports in Medicine
                Hindawi
                1687-9627
                1687-9635
                2020
                20 April 2020
                : 2020
                : 4683507
                Affiliations
                1Internal Medicine, University Hospitals Regional Hospitals, Richmond Heights, OH 44143, USA
                2Lake Erie College of Osteopathic Medicine, Erie, OH 16509, USA
                Author notes

                Academic Editor: Michael S. Firstenberg

                Author information
                https://orcid.org/0000-0002-1271-1315
                Article
                10.1155/2020/4683507
                7189311
                32373177
                62f32a9c-e410-42e8-bff1-f3dce6b9b613
                Copyright © 2020 David Y. Liu et al.

                This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

                History
                : 24 October 2019
                : 12 March 2020
                : 3 April 2020
                Funding
                Funded by: University Hospitals Regional Hospitals Internal Medicine Residency Program
                Categories
                Case Report

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