Guillain-Barré Syndrome Donofrio, Peter D
Continuum (Minneapolis, Minn.),
2017-October, 2017-Oct, 2017-10-00, Volume:
23, Issue:
5, Peripheral Nerve and Motor Neuron Disorders
Journal Article
ABSTRACTPurpose of ReviewThis article reviews the current state of Guillain-Barré syndrome (GBS), including its clinical presentation, evaluation, pathophysiology, and treatment.Recent FindingsGBS is ...an acute/subacute-onset polyradiculoneuropathy typically presenting with sensory symptoms and weakness over several days, often leading to quadriparesis. Approximately 70% of patients report a recent preceding upper or lower respiratory tract infection or gastrointestinal illness. Approximately 30% of patients require intubation and ventilation because of respiratory failure. Nerve conduction studies in the acute inflammatory demyelinating polyradiculoneuropathy (AIDP) form of GBS typically show evidence for a multifocal demyelinating process, including conduction block or temporal dispersion in motor nerves. Sural sparing is a common phenomenon when testing sensory nerves. CSF analysis commonly shows an elevated protein, but this elevation may not be present until the third week of the illness. Patients with AIDP are treated with best medical management and either IV immunoglobulin (IVIg) or plasma exchange.SummaryGBS is a common form of acute quadriparesis; a high level of suspicion is needed for early diagnosis. With appropriate therapy, most patients make a very good to complete recovery.
OBJECTIVE:This study estimates current and projects future neurologist supply and demand under alternative scenarios nationally and by state from 2012 through 2025.
METHODS:A microsimulation supply ...model simulates likely career choices of individual neurologists, taking into account the number of new neurologists trained each year and changing demographics of the neurology workforce. A microsimulation demand model simulates utilization of neurology services for each individual in a representative sample of the population in each state and for the United States as a whole. Demand projections reflect increased prevalence of neurologic conditions associated with population growth and aging, and expanded coverage under health care reform.
RESULTS:The estimated active supply of 16,366 neurologists in 2012 is projected to increase to 18,060 by 2025. Long wait times for patients to see a neurologist, difficulty hiring new neurologists, and large numbers of neurologists who do not accept new Medicaid patients are consistent with a current national shortfall of neurologists. Demand for neurologists is projected to increase from ∼18,180 in 2012 (11% shortfall) to 21,440 by 2025 (19% shortfall). This includes an increased demand of 520 full-time equivalent neurologists starting in 2014 from expanded medical insurance coverage associated with the Patient Protection and Affordable Care Act.
CONCLUSIONS:In the absence of efforts to increase the number of neurology professionals and retain the existing workforce, current national and geographic shortfalls of neurologists are likely to worsen, exacerbating long wait times and reducing access to care for Medicaid beneficiaries. Current geographic differences in adequacy of supply likely will persist into the future.
Background
Little literature exists describing resident training in peripheral electrodiagnosis (EDX).
Methods
U.S. residency programs in neurology and physical medicine and rehabilitation (PM&R) ...were surveyed by the AANEM (American Association of Neuromuscular and Electrodiagnostic Medicine) on specific features of EDX training.
Results
Ninety‐seven programs responded to the survey. Training duration was 4–8 weeks in most neurology programs; training averaged 22 weeks in PM&R programs. EDX experience was required in all PM&R and in 90% of neurology programs. Results varied greatly for the residency years of training, pulling of residents for other responsibilities, participation in continuity clinics, number of teaching physicians, number of needle examinations performed, organization of nerve conduction training, written/oral examinations, muscle/nerve biopsy reviews, and training materials.
Conclusions
This survey demonstrated large variability in training of neurology and PM&R residents in peripheral EDX.
Intravenous immune globulin (IVIG) is an immune‐modulating biologic therapy that is increasingly being used in neuromuscular disorders despite the paucity of high‐quality evidence for various ...specific diseases. To address this, the AANEM created the 2009 consensus statement to provide guidance on the use of IVIG in neuromuscular disorders. Since then, there have been several randomized controlled trials for IVIG, a new FDA‐approved indication for dermatomyositis and a revised classification system for myositis, prompting the AANEM to convene an ad hoc panel to update the existing guidelines.New recommendations based on an updated systemic review of the literature were categorized as Class I‐IV. Based on Class I evidence, IVIG is recommended in the treatment of chronic inflammatory demyelinating polyneuropathy, Guillain‐Barré Syndrome (GBS) in adults, multifocal motor neuropathy, dermatomyositis, stiff‐person syndrome and myasthenia gravis exacerbations but not stable disease. Based on Class II evidence, IVIG is also recommended for Lambert‐Eaton myasthenic syndrome and pediatric GBS. In contrast, based on Class I evidence, IVIG is not recommended for inclusion body myositis, post‐polio syndrome, IgM paraproteinemic neuropathy and small fiber neuropathy that is idiopathic or associated with tri‐sulfated heparin disaccharide or fibroblast growth factor receptor‐3 autoantibodies. Although only Class IV evidence exists for IVIG use in necrotizing autoimmune myopathy, it should be considered for anti‐hydroxy‐3‐methyl‐glutaryl‐coenzyme A reductase myositis given the risk of long‐term disability. Insufficient evidence exists for the use of IVIG in Miller‐Fisher syndrome, IgG and IgA paraproteinemic neuropathy, autonomic neuropathy, chronic autoimmune neuropathy, polymyositis, idiopathic brachial plexopathy and diabetic lumbosacral radiculoplexopathy.
Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is the most common treatable chronic autoimmune neuropathy. Multiple diagnostic criteria have been established, with the primary goal ...of identifying neurophysiologic hallmarks of acquired demyelination. Treatment modalities have expanded to include numerous immunomodulatory therapies, although the best evidence continues to be for corticosteroids, plasma exchange, and intravenous immunoglobulin (IVIg). This review describes the pathology, epidemiology, pathogenesis, diagnosis, and treatment of CIDP.
Textbook of Peripheral Neuropathy is a practical but authoritative reference for clinicians in anymedical specialty who are evaluating and treating patients with signs and symptoms of a ...peripheralneuropathy. Reviewing the full spectrum of clinically significant neuropathies, the book contains chapterson common and rare forms including mononeuropathy in the upper and lower extremities, mononeuritismultiplex, diffuse and symmetric polyneuropathies, brachial and lumbrosacral plexopathies, and spinalroot disordersódisorders that can mimic diffuse and/or focal neuropathies, complicating diagnosis andevaluation. Coverage encompasses both inherited and acquired diseases, including neuropathies arisingfrom physical injury, diabetes, alcoholism, toxins, autoimmune responses, nutritional deficiencies, vascularand metabolic disorders, medication-induced neuropathies, and idiopathic conditions. The textbookprovides an evidence-based approach to testing, differential diagnosis, and treatment, and should serveas a trusted resource for healthcare professionals confronting the many manifestations of peripheralneuropathy in clinical practice. The chapters are written by internationally renowned expert contributors with deep clinical experienceand contain numerous tables, figures, and algorithms providing clear diagnostic and managementguidelines. Boxed Clinical Pearls and Key Points allow for quick access to pertinent information, making evaluation and review easy and rewarding. Features of Textbook of Peripheral Neuropathy Include: * ¨ * Practical yet comprehensiveóan accessible ìgo-toî reference for clinicians¨ * Covers all clinically relevant peripheral neuropathies¨ * Clinical Pearls and Key Points are set off from the text for quick reference ¨ * Contains clear diagnostic and management guidelines from expert contributors¨ * Structured chapters make it easy to find essential point-of-careinformation
ABSTRACT
Introduction: Electrodiagnostic studies (EDX) are not performed routinely before treatment suspension in CIDP, and no data exist regarding their value in predicting clinical relapse. ...Methods: Serial EDX (baseline and after IGIV‐C therapy) were analyzed from subjects in the ICE clinical trial who responded to IGIV‐C treatment and were subsequently re‐randomized to placebo in an extension phase. Comparisons were made between subjects who relapsed and those who did not. Results: A total of 55% (6/11) of the Relapse group had an increase in total number of demyelinating findings (DF) versus 8% (1/13) in the No Relapse group (P = 0.023). In the Relapse group, 100% had ≥1 new DF and 73% (8/11) had ≥4 new DF versus 60% (8/13) and 8% (1/13), respectively, in the No Relapse group. Conclusions: An increased total number of DF or the occurrence of ≥4 new DF may indicate a higher risk of clinical relapse after treatment cessation in IGIV‐C‐responsive patients. Muscle Nerve 52: 498–502, 2015
The antibody profile against autoantigens previously associated with autoimmune diseases and other human proteins in patients with COVID-19 or multisystem inflammatory syndrome in children (MIS-C) ...remains poorly defined. Here we show that 30% of adults with COVID-19 had autoantibodies against the lung antigen KCNRG, and 34% had antibodies to the SLE-associated Smith-D3 protein. Children with COVID-19 rarely had autoantibodies; one of 59 children had GAD65 autoantibodies associated with acute onset of insulin-dependent diabetes. While autoantibodies associated with SLE/Sjögren's syndrome (Ro52, Ro60, and La) and/or autoimmune gastritis (gastric ATPase) were detected in 74% (40/54) of MIS-C patients, further analysis of these patients and of children with Kawasaki disease (KD), showed that the administration of intravenous immunoglobulin (IVIG) was largely responsible for detection of these autoantibodies in both groups of patients. Monitoring
decay of the autoantibodies in MIS-C children showed that the IVIG-derived Ro52, Ro60, and La autoantibodies declined to undetectable levels by 45-60 days, but gastric ATPase autoantibodies declined more slowly requiring >100 days until undetectable. Further testing of IgG and/or IgA antibodies against a subset of potential targets identified by published autoantigen array studies of MIS-C failed to detect autoantibodies against most (16/18) of these proteins in patients with MIS-C who had not received IVIG. However, Troponin C2 and KLHL12 autoantibodies were detected in 2 of 20 and 1 of 20 patients with MIS-C, respectively. Overall, these results suggest that IVIG therapy may be a confounding factor in autoantibody measurements in MIS-C and that antibodies against antigens associated with autoimmune diseases or other human proteins are uncommon in MIS-C.
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