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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Background: Both Guillain–Barré syndrome (GBS) and chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) are neurodegenerative and inflammatory demyelination disorders. Sporadic reports showed that the increased levels of thyroid function and autoantibodies are associated with GBS, CIDP, or both, but no systematic study has been reported. We assessed the differences of thyroid function and autoantibodies between GBS and CIDP in a Chinese cohort.Methods: A total of 256 patients were enrolled in this study. 175 clinically confirmed GBS and CIDP patients were selected. Meanwhile, 81 patients hospitaliz...
Source: Frontiers in Neurology - Category: Neurology Source Type: research
Authors: Nieto-Aristizábal I, Vivas ÁJ, Ruiz-Montaño P, Aragón CC, Posso-Osorio I, Quiñones J, Rivillas JA, Tobón GJ Abstract Introduction: Therapeutic plasma exchange (TPE) is commonly used as treatment of certain autoimmune neurological diseases (ANDs), and its main objective is the removal of pathogenic autoantibodies. Our aim was to describe the clinical profile and the experience with the usage of TPE in patients with ANDs at our institution. Methods: This is an observational retrospective study, including medical records of patients with diagnosis of ANDs who rece...
Source: Autoimmune Diseases - Category: Allergy & Immunology Tags: Autoimmune Dis Source Type: research
Source: Neurology Neuroimmunology and Neuroinflammation - Category: Neurology Tags: Correction Source Type: research
Peripheral nerve disorders are common and often treatable. The ‘default’ presentation of a polyneuropathy is a chronic, length-dependent, sensorimotor axonopathy. Recognizing deviations from this default, informed by the clinical features and investigations, can help identify the cause of a neuropathy in most cases. For inflammatory causes, such as Guillain –Barré syndrome and chronic inflammatory demyelinating polyradiculoneuropathy, there are effective immunomodulatory treatments. For other neuropathies, management consists of supportive care and treatment of the underlying cause, to prevent or limit progression.
Source: Medicine - Category: Internal Medicine Authors: Tags: Neuromuscular disorders in neurology Source Type: research
Conclusion Our data (1) confirm and extend previous observations that antiparanodal IgG2/3 but not IgG4 antibodies can occur in acute-onset neuropathies manifesting as monophasic GBS, (2) suggest association of IgG3 to a favorable response to IVIg, and (3) lend support to the hypothesis that in some patients, an IgG subclass switch from IgG3 to IgG4 may be the correlate of a secondary progressive or relapsing course following a GBS-like onset.
Source: Neurology Neuroimmunology and Neuroinflammation - Category: Neurology Authors: Tags: Autoimmune diseases, All Neuromuscular Disease, Peripheral neuropathy, Guillain-Barre syndrome, Chronic inflammatory demyelinating polyneuropathy Article Source Type: research
The discovery of autoantibodies against paranodal proteins such as neurofascin-155 (NF155), contactin-1 (CNTN1), or contactin-associated protein-1 (CASPR1) in inflammatory neuropathies has led to the description of subsets of patients with specific phenotypic features.1 These antibodies mostly belong to the immunoglobulin G (IgG)4 subclass, but IgG1, IgG2, or IgG3 autoantibodies have also been described.2–4 Different autoantibody isotypes are associated with different effector mechanisms causing nerve damage5,6; testing them may have implications to inform therapeutic choices or to predict prognosis.
Source: Neurology Neuroimmunology and Neuroinflammation - Category: Neurology Authors: Tags: Autoimmune diseases, All Neuromuscular Disease, Peripheral neuropathy, Guillain-Barre syndrome, Chronic inflammatory demyelinating polyneuropathy Editorial Source Type: research
CONCLUSIONS AND CLINICAL IMPLICATIONS: Our study proves that the use of plasmapheresis conducted by filtration in the treatment of autoimmune neurological diseases is safe and well tolerated. PMID: 32557528 [PubMed - as supplied by publisher]
Source: Neurologia i Neurochirurgia Polska - Category: Neurology Authors: Tags: Neurol Neurochir Pol Source Type: research
This article spans the common, well-defined inflammatory demyelinating polyradiculoneuropathies (Guillain-Barr é syndrome and chronic inflammatory demyelinating polyradiculoneuropathy) to the rarer, acquired demyelinating neuropathy variants (Miller-Fisher syndrome and multifocal motor neuropathy), vasculitic neuropathies, and sensory neuronopathies (dorsal root ganglionopathies). These case studies illustr ate the characteristic clinical patterns of the immune-mediated neuropathies encountered in neurologic practice. Recommendations for diagnostic evaluation and treatment approach accompany each case. Prompt recogn...
Source: Neurologic Clinics - Category: Neurology Authors: Source Type: research
Patients with nodal/paranodal antibodies represent a specific subgroup of inflammatory peripheral neuropathies, whose clinical presentation with a prolonged subacute phase, additional symptoms such as ataxia and tremor, and poor treatment response to IV immunoglobulin (IVIG) often differs from classic Guillain-Barré syndrome (GBS) or chronic inflammatory demyelinating polyneuropathy (CIDP).1
Source: Neurology Neuroimmunology and Neuroinflammation - Category: Neurology Authors: Tags: Guillain-Barre syndrome, Chronic inflammatory demyelinating polyneuropathy Clinical/Scientific Notes Source Type: research
Alemtuzumab is approved for the treatment of relapsing-remitting MS and is used off-label for patients with chronic lymphocytic leukemia and as induction and antirejection therapy in kidney transplant recipients.1 Guillain-Barré syndrome (GBS) or chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) complicating alemtuzumab treatment was reported in 9 patients with hematologic malignancy or MS.1–3 The risk of GBS or CIDP in solid organ transplant recipients treated with alemtuzumab is unknown.
Source: Neurology Neuroimmunology and Neuroinflammation - Category: Neurology Authors: Tags: All Clinical Neurology, Guillain-Barre syndrome, Chronic inflammatory demyelinating polyneuropathy, All Demyelinating disease (CNS), Multiple sclerosis Clinical/Scientific Notes Source Type: research
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