Diagnosis and treatment of Guillain-Barr é Syndrome in childhood and adolescence: An evidence- and consensus-based guideline.

Diagnosis and treatment of Guillain-Barré Syndrome in childhood and adolescence: An evidence- and consensus-based guideline. Eur J Paediatr Neurol. 2020 Jan 07;: Authors: Korinthenberg R, Trollmann R, Felderhoff-Müser U, Bernert G, Hackenberg A, Hufnagel M, Pohl M, Hahn G, Mentzel HJ, Sommer C, Lambeck J, Mecher F, Hessenauer M, Winterholler C, Kempf U, Jacobs BC, Rostasy K, Müller-Felber W Abstract This evidence- and consensus-based practical guideline for the diagnosis and treatment of Guillain-Barré Syndrome (GBS) in childhood and adolescence has been developed by a group of delegates from relevant specialist societies and organisations; it is the result of an initiative by the German-Speaking Society of Neuropediatrics (GNP), and is supported by the Association of Scientific Medical Societies (AWMF, Arbeitsgemeinschaft der Wissenschaftlichen Medizinischen Fachgesellschaften). A systematic analysis of the literature revealed that only a few adequately-controlled studies exist for this particular age group, while none carries a low risk of bias. For this reason, the diagnostic and therapeutic recommendations largely rely on findings in adult patients with GBS, for which there are a higher number of suitable studies available. Consensus was established using a written, multi-step Delphi process. A high level of consensus could be reached for the crucial steps in diagnosis and treatment. We recommend basing the diagnostic approach...
Source: European Journal of Paediatric Neurology - Category: Neurology Authors: Tags: Eur J Paediatr Neurol Source Type: research

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Conditions:   Guillain-Barre Syndrome;   CIDP Intervention:   Sponsors:   University Hospital, Montpellier;   CHU de la Timone, Marseille, France;   CHU Carémeau, Nîmes, France;   CHU de Bordeaux, France;   CH de Perpignan, France;   CH de Narbonne, France;   CH de Béziers France Recruiting
Source: ClinicalTrials.gov - Category: Research Source Type: clinical trials
Gangliosides are sialylated glycosphingolipids, highly abundant in our nervous system. Antibodies targeting gangliosides are usually developed as a consequence of molecular mimicry following infections. Antiganglioside antibodies are implicated in many neurological disorders such as acute and chronic polyradiculoneuropathies which includes different variants of Guillain-Barr é syndrome, chronic inflammatory demyelinating polyradiculoneuropathy and multifocal motor neuropathy. Presence of such antibodies in paraneoplastic peripheral neuropathy, neurodegenerative disorders, multiple sclerosis, myasthenia gravis and am...
Source: Journal of the Neurological Sciences - Category: Neurology Authors: Tags: Review Article Source Type: research
Abstract Therapeutic plasma exchange (TPE) has been used as a treatment modality in many autoimmune disorders, including neurological conditions, such as Guillain-Barre syndrome (GBS) and chronic inflammatory demyelinating polyradiculoneuropathy (CIDP). The American Society for Apheresis (ASFA) publishes its guidelines on the use of therapeutic apheresis every 3 years based on published evidence to assist physicians with both the medical and technical aspects of apheresis consults. The ASFA Guidelines included the use of TPE in both GBS and CIDP as an acceptable first-line therapy, either alone and/or in conjuncti...
Source: Presse Medicale - Category: General Medicine Authors: Tags: Presse Med Source Type: research
Therapeutic plasma exchange (TPE) involves the extracorporeal separation of plasma from the cellular components of blood with replacement fluid, such as human albumin or fresh frozen plasma. A number of studies across the world revealed that more than one third of TPE procedures were performed for neurological disorders.Myasthenia gravis (MG), Guillain-Barr é syndrome (GBS) and chronic inflammatory demyelinating polyneuropathy (CIDP) were the most frequently cited indications for TPE, followed by multiple sclerosis (MS).
Source: Transfusion and Apheresis Science - Category: Hematology Authors: Source Type: research
A 26-year-old female presented with acute onset distal paraparesis, upper limb tremor and bilateral facial palsy. Neurophysiology revealed a sensorimotor demyelinating polyneuropathy and lumbar puncture revealed an albuminocytologic dissociation. Neuroaxis MRI revealed bilateral facial nerve and cauda equina enhancement. Initially diagnosed as Guillain-Barr é Syndrome, poor response to intravenous immunoglobulin, persistent deterioration, anti-neurofascin-155 antibodies and clinical response to steroid therapy led to diagnosis of acute-onset chronic inflammatory demyelinating polyneuropathy (CIDP).
Source: Journal of Neuroimmunology - Category: Allergy & Immunology Authors: Tags: Short Communication Source Type: research
Conclusions Our results indicate that anti–pan-NF-associated neuropathy differs from anti–NF-155-associated neuropathy, and epitope and subclass play a major role in the pathogenesis and severity of anti–NF-associated neuropathy and should be determined to correctly classify patients, also in respect to possible differences in therapeutic response.
Source: Neurology Neuroimmunology and Neuroinflammation - Category: Neurology Authors: Tags: Autoimmune diseases, All Clinical Neurology, Guillain-Barre syndrome, Chronic inflammatory demyelinating polyneuropathy Article Source Type: research
A previously healthy 2-year and 9-month old boy was brought to the emergency department for a 6-day history of weakness in the legs and frequent falls, rendering him unable to walk 1 day before admission. He did not have pain, dysphagia, bladder dysfunction, or sensory symptoms. There was no history of trauma, but he developed diarrhea 3 days before symptom onset. Family history was negative for consanguinity or neurologic diseases. At examination, he had bilateral leg weakness requiring substantial aid to walk a few steps and was unable to stand up from the floor. He had absent tendon reflexes in the lower extremities and...
Source: Neurology Neuroimmunology and Neuroinflammation - Category: Neurology Authors: Tags: Guillain-Barre syndrome, Chronic inflammatory demyelinating polyneuropathy, All Pediatric, EMG Clinical/Scientific Notes Source Type: research
ConclusionsTonic pupils have previously been described in different neurologic conditions, including GBS, but not yet in acute onset CIDP or in variants with predominantly distal demyelination. It differs from the classical Adie ’s pupil because it lacks the light-near dissociation. This case report expands the range of possible autonomic signs in acute onset CIDP, which could help physicians establish optimal treatment strategies earlier on.
Source: Neurological Sciences - Category: Neurology Source Type: research
Intravenous immunoglobulin therapy is FDA approved for the immune-mediated peripheral nerve disorders Guillain-Barre syndrome, chronic inflammatory demyelinating polyneuropathy, and multifocal motor neuropathy. Immunoglobulin therapy has been used increasingly with significant efficacy in the treatment of patients with disabling autoimmune forms of dysautonomia, which are most often small fiber (autonomic and/or sensory) polyneuropathies. It is recognized by most who treat these disorders, however, that patients with autonomic dysfunction treated with intravenous immunoglobulin therapy develop aseptic meningitis or severe ...
Source: European Neurology - Category: Neurology Source Type: research
Authors: Peter JG, Heckmann JM, Novitzky N Abstract Polyvalent immunoglobin, derived from pooled human plasma, can be administered via the intravenous, subcutaneous or intramuscular route. Therapy is standard of care in the treatment of a number of immune-mediated pathologies across disciplines. By volume, the majority is used in neurology (~40%). In primary immunodeficiencies, therapy reconstitutes humoral immunity at replacement doses (0.4 - 0.6 g/kg/month), decreasing infections, and is usually lifelong. However, high doses, usually 2 g/kg total dose over five days, are required for immunomodulati...
Source: South African Medical Journal - Category: African Health Tags: S Afr Med J Source Type: research
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