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      The prognostic value of demyelinating electrophysiologic findings and cerebrospinal fluid protein levels in acute inflammatory demyelinating polyneuropathy Translated title: O valor prognóstico dos achados eletrofisiológicos desmielinizantes e os níveis de proteína do líquido cefalorraquidiano na polineuropatia desmielinizante inflamatória aguda

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          Abstract

          ABSTRACT Background: Guillain-Barre syndrome is an acute immune-mediated polyneuropathy characterized by rapidly evolving symptoms and disability. Cerebrospinal fluid analysis and electrophysiological studies are crucial in the diagnosis of this syndrome. Objective: To evaluate the prognostic value of the type and number of demyelinating findings and cerebrospinal fluid protein levels in patients with acute inflammatory demyelinating polyneuropathy. Methods: We retrospectively analyzed electrophysiological data and cerebrospinal fluid of 67 consecutive patients with acute inflammatory demyelinating polyneuropathy from Istanbul, Turkey (2011-2019) studied ≤ 24 hours post-onset. Results: The patients who met a higher number of demyelinating criteria had increased disability scores in the first day and first month, and higher cerebrospinal fluid protein levels were correlated with worse prognosis both on the first day and the first month. However, the disability scores did not correlate with any single specific criterion, and no significant correlation was found between the number of satisfied criteria and cerebrospinal fluid protein levels. Conclusions: The number of demyelinating criteria that are met and high cerebrospinal fluid protein levels at the disease onset may be valuable prognostic markers. More systematic studies conducted with serial nerve conduction studies are required to highlight the roles of the suggested criteria in clinical practice.

          Translated abstract

          RESUMO Introdução: A síndrome de Guillain-Barré é uma polineuropatia imunomediada aguda caracterizada por sintomas e incapacidade em rápida evolução. A análise do líquido cefalorraquidiano e os estudos eletrofisiológicos são cruciais no diagnóstico dessa síndrome. Objetivo: Avaliar o valor prognóstico do tipo e número de achados desmielinizantes e dos níveis de proteínas do líquido cefalorraquidiano em pacientes com polineuropatia desmielinizante inflamatória aguda. Métodos: Analisamos retrospectivamente dados eletrofisiológicos e líquido cefalorraquidiano de 67 pacientes consecutivos com polineuropatia desmielinizante inflamatória aguda de Istambul, Turquia (2011-2019), estudados ≤24 horas após o início. Resultados: Os pacientes que atenderam a um número maior de critérios desmielinizantes apresentaram escores de incapacidade aumentados no primeiro dia e no primeiro mês, e níveis mais altos de proteína do líquido cefalorraquidiano foram correlacionados com pior prognóstico no primeiro dia e no primeiro mês. No entanto, os escores de incapacidade não se correlacionaram com nenhum critério específico e não foi encontrada correlação significativa entre o número de critérios satisfeitos e os níveis de proteína do líquido cefalorraquidiano. Conclusões: O número de critérios desmielinizantes atendidos e altos níveis de proteína no líquido cefalorraquidiano no início da doença podem ser marcadores prognósticos valiosos. Estudos mais sistemáticos conduzidos com estudos de condução nervosa em série são necessários para destacar os papéis dos critérios sugeridos na prática clínica.

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          Autoimmune T cell responses in the central nervous system.

          Autoreactive T cell responses have a crucial role in central nervous system (CNS) diseases such as multiple sclerosis. Recent data indicate that CNS autoimmunity can be mediated by two distinct lineages of CD4+ T cells that are defined by the production of either interferon-gamma or interleukin-17. The activity of these CD4+ T cell subsets within the CNS influences the pathology and clinical course of disease. New animal models show that myelin-specific CD8+ T cells can also mediate CNS autoimmunity. This Review focuses on recent progress in delineating the pathogenic mechanisms, regulation and interplay between these different T cell subsets in CNS autoimmunity.
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            Guillain-Barré syndrome and variants.

            Guillain-Barré syndrome (GBS) is characterized by rapidly evolving ascending weakness, mild sensory loss, and hyporeflexia or areflexia. Acute inflammatory demyelinating polyneuropathy was the first to be recognized over a century ago and is the most common form of GBS. Axonal motor and sensorimotor variants have been described in the last three decades and are mediated by molecular mimicry targeting peripheral nerve motor axons. Other rare phenotypic variants have been recently described with pure sensory variant, restricted autonomic manifestations, and the pharyngeal-cervical-brachial pattern. It is important to recognize GBS and its variants because of the availability of equally effective therapies in the form of plasmapheresis and intravenous immunoglobulins.
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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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                Author and article information

                Journal
                anp
                Arquivos de Neuro-Psiquiatria
                Arq. Neuro-Psiquiatr.
                Academia Brasileira de Neurologia - ABNEURO (São Paulo, SP, Brazil )
                0004-282X
                1678-4227
                August 2020
                : 78
                : 8
                : 481-487
                Affiliations
                [2] Istanbul orgnameTurkish Health Sciences University orgdiv1Istanbul Training and Research Hospital orgdiv2Department of Neurology Turkey
                [3] Istanbul orgnameBezmiâlem Vak?f Üniversitesi orgdiv1Faculty of Medicine orgdiv2Department of Neurology Turkey
                [4] Bursa orgnameBursa Yüksek İhtisas Education and Research Hospital orgdiv1Department of Neurology Turkey
                [1] Sakarya orgnameSakarya University orgdiv1Sakarya Training and Research Hospital orgdiv2Department of Neurology Turkey
                Article
                S0004-282X2020000800481 S0004-282X(20)07800800481
                10.1590/0004-282x20200042
                c012f971-96d8-4db4-a557-4a76f51dd7a0

                This work is licensed under a Creative Commons Attribution 4.0 International License.

                History
                : 12 April 2020
                : 06 April 2020
                Page count
                Figures: 0, Tables: 0, Equations: 0, References: 25, Pages: 7
                Product

                SciELO Brazil

                Categories
                Articles

                Prognóstico,Guillain-Barre Syndrome,Síndrome de Guillain-Barré,Prognosis,Electromyography,Eletromiografia

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