Guillain-Barré syndrome Willison, Hugh J, Prof; Jacobs, Bart C, Prof; van Doorn, Pieter A, Prof
The Lancet,
08/2016, Letnik:
388, Številka:
10045
Journal Article
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Summary Guillain-Barré syndrome is the most common and most severe acute paralytic neuropathy, with about 100 000 people developing the disorder every year worldwide. Under the umbrella term of ...Guillain-Barré syndrome are several recognisable variants with distinct clinical and pathological features. The severe, generalised manifestation of Guillain-Barré syndrome with respiratory failure affects 20–30% of cases. Treatment with intravenous immunoglobulin or plasma exchange is the optimal management approach, alongside supportive care. Understanding of the infectious triggers and immunological and pathological mechanisms has advanced substantially in the past 10 years, and is guiding clinical trials investigating new treatments. Investigators of large, worldwide, collaborative studies of the spectrum of Guillain-Barré syndrome are accruing data for clinical and biological databases to inform the development of outcome predictors and disease biomarkers. Such studies are transforming the clinical and scientific landscape of acute autoimmune neuropathies.
Summary Guillain-Barré syndrome (GBS) is an important cause of acute neuromuscular paralysis. Molecular mimicry and a cross-reactive immune response play a crucial part in its pathogenesis, at least ...in those cases with a preceding Campylobacter jejuni infection and with antibodies to gangliosides. The type of preceding infection and patient-related host factors seem to determine the form and severity of the disease. Intravenous immunoglobulin (IVIg) and plasma exchange are effective treatments in GBS; mainly for practical reasons, IVIg is the preferred treatment. Whether mildly affected patients or patients with Miller Fisher syndrome also benefit from IVIg is unclear. Despite medical treatment, GBS often remains a severe disease; 3–10% of patients die and 20% are still unable to walk after 6 months. In addition, many patients have pain and fatigue that can persist for months or years. Advances in prognostic modelling have resulted in the development of a new and simple prognostic outcome scale that might also help to guide new treatment options, particularly in patients with GBS who have a poor prognosis.
Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is a rare and heterogeneous but treatable immune-mediated neuropathy. Nerve conduction studies are considered essential for a definite ...diagnosis, but poor performance and misinterpretation of the results frequently leads to misdiagnosis. Nerve ultrasound and MRI could be helpful in diagnosis. Whereas typical CIDP is relatively easy to diagnose, atypical variants with distinct phenotypes can be a diagnostic challenge. Intravenous or subcutaneous immunoglobulin, corticosteroids, and plasma exchange are effective treatments, but maintenance treatments are often required for years, and treatment regimens require careful and regular adjustments to avoid undertreatment or overtreatment. Patients who do not improve, or insufficiently improve after treatment, might have specific characteristics related to a distinct disease mechanism caused by immunoglobulin G4 antibodies to nodal or paranodal proteins, and could require alternative treatments. Future studies should focus on curative and individualised treatment regimens to improve the patient's condition and to prevent further nerve damage.
Polyneuropathy is a disabling condition of the peripheral nerves, characterized by symmetrical distal numbness and paresthesia, often accompanied with pain and weakness. Although the disease is often ...encountered in neurological clinics and is well known by physicians, incidence and prevalence rates are not well known. We searched EMBASE, Medline, Web-of-science, Cochrane, PubMed Publisher, and Google Scholar, for populationbased studies investigating the prevalence of polyneuropathy and its risk factors. Out of 5119 papers, we identified 29 eligible studies, consisting of 11 door-to-door survey studies, 7 case-control studies and 11 cohort/database studies. Prevalence of polyneuropathy across these studies varies substantially. This can partly be explained by differences in assessment protocols and study populations. The overall prevalence of polyneuropathy in the general population seems around 1 % and rises to up to 7 % in the elderly. Polyneuropathy seemed more common in Western countries than in developing countries and there are indications that females are more often affected than males. Risk factor profiles differ across countries. In developing countries communicable diseases, like leprosy, are more common causes of neuropathy, whereas in Western countries especially diabetes, alcohol overconsumption, cytostatic drugs and cardiovascular disease are more commonly associated with polyneuropathy. In all studies a substantial proportion of polyneuropathy cases (20-30 %) remains idiopathic. Most of these studies have been performed over 15 years ago. More recent evidence suggests that the prevalence of polyneuropathy in the general population has increased over the years. Future research is necessary to confirm this increase in prevalence and to identify new and potentially modifiable risk factors.
Guillain-Barré syndrome (GBS) is a potentially life-threatening postinfectious disease characterized by rapidly progressive, symmetrical weakness of the extremities. About 25% of patients develop ...respiratory insufficiency and many show signs of autonomic dysfunction. Diagnosis can usually be made on clinical grounds, but lumbar puncture and electrophysiological studies can help to substantiate the diagnosis and to differentiate demyelinating from axonal subtypes of GBS. Molecular mimicry of pathogen-borne antigens, leading to generation of crossreactive antibodies that also target gangliosides, is part of the pathogenesis of GBS; the subtype and severity of the syndrome are partly determined by the nature of the antecedent infection and specificity of such antibodies. Intravenous immunoglobulin and plasma exchange are proven effective treatments but many patients have considerable residual deficits. Discrimination of patients with treatment-related fluctuations from those with acute-onset chronic inflammatory demyelinating polyneuropathy is important, as these conditions may require different treatments. Novel prognostic models can accurately predict outcome and the need for artificial ventilation, which could aid the selection of patients with a poor prognosis for more-individualized care. This Review summarizes the clinical features of and diagnostic criteria for GBS, and discusses its pathogenesis, treatment and prognosis.
Treatment dilemmas in Guillain-Barré syndrome Verboon, Christine; van Doorn, Pieter A; Jacobs, Bart C
Journal of neurology, neurosurgery and psychiatry,
04/2017, Letnik:
88, Številka:
4
Journal Article
Recenzirano
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Guillain-Barré syndrome (GBS) is an acute polyradiculoneuropathy with a highly variable clinical course and outcome. Intravenous immunoglobulin (IVIg) and plasma exchange are proven effective ...treatments, but the efficacy has been demonstrated mainly on motor improvement in adults with a typical and severe form of GBS. In clinical practice, treatment dilemmas may occur in patients with a relatively mild presentation, variant forms of GBS, or when the onset of weakness was more than 2 weeks ago. Other therapeutic dilemmas may arise in patients who do not improve or even progress after initial treatment. We provide an overview of the current literature about therapeutic options in these situations, and additionally give our personal view that may serve as a basis for therapeutic decision-making.
The 2021 guideline of the European Academy of Neurology/Peripheral Nerve Society on chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) includes important revisions to the previous 2010 ...guideline. This article highlights the new criteria and recommendations for the differential diagnosis of CIDP. In the revised guideline, the CIDP spectrum has been modified to include typical CIDP and four well-characterized CIDP variants, namely distal, multifocal/focal, motor and sensory CIDP, replacing the term ‘atypical’ CIDP. To improve the diagnosis of CIDP, the revised guideline attempts to improve the specificity of the diagnostic criteria for typical CIDP and the four CIDP variants. Specific clinical and electrodiagnostic (including both motor and sensory conduction) criteria are provided for typical CIDP and each of the CIDP variants. The levels of diagnostic certainty have been changed to CIDP and possible CIDP, with the removal of probable CIDP (due to the lack of difference in the accuracy of the electrodiagnostic criteria for probable CIDP) and definite CIDP (due to the lack of a gold standard for diagnosis). If the clinical and electrodiagnostic criteria allow only for a diagnosis of possible CIDP, cerebrospinal fluid analysis, nerve ultrasound, nerve magnetic resonance imaging, objective treatment response, and nerve biopsy can be used as supportive criteria to upgrade the diagnosis to CIDP. Although the revised guideline needs to be validated and its strengths and weaknesses assessed, using the guideline will likely improve the accuracy of diagnosis of CIDP and variants of CIDP, and aid in distinguishing CIDP from conditions with similar features.
•2021 guidelines have made important revisions to the diagnostic criteria for CIDP.•CIDP is classified as typical or variant (distal, multifocal/focal, motor or sensory).•Each type of CIDP has specific clinical and electrodiagnostic criteria.•May upgrade diagnosis of possible CIDP to CIDP if ≥2 supportive criteria are present.•Guideline use is likely to aid in the differential diagnosis of CIDP and its variants.
Axonal damage in chronic inflammatory demyelinating polyneuropathy (CIDP) is the main predictor of poor outcome. We hypothesized that serum neurofilament light chain (sNfL) reflects disease activity ...by detecting ongoing neuro‐axonal damage in CIDP. Three prospective cohorts of CIDP patients were studied: (a) patients starting induction treatment (IT cohort, N = 29) measured at baseline and 6 months after starting treatment; (b) patients on maintenance treatment (MT) starting intravenous immunoglobuline (IVIg) withdrawal (MT cohort, N = 24) measured at baseline and 6 months after IVIg withdrawal or at time of relapse; and (c) patients in long‐term remission without treatment (N = 27). A single molecule array assay was used to measure sNfL. Age‐matched healthy controls (N = 30) and age‐specific reference values were used for comparison. At baseline, sNfL was higher in patients starting IT compared to healthy controls. Ten out of 29 IT (34%) patients have sNfL levels above the 95th percentile of age‐specific cut‐off values. In the MT and remission cohort, elevated sNfL levels were infrequent and not different from healthy controls. sNfL levels were correlated with electrophysiological markers of axonal damage. At follow‐up assessment, patients with active disease (non‐responders and patients who relapsed after IVIg withdrawal) had higher sNfL levels compared with patients with stable disease (responders and patients who were successfully withdrawn from IVIg treatment). sNfL levels were increased in a third of CIDP patients starting IT and reflected axonal damage. sNfL levels might be usable as biomarker of disease activity in a subset of CIDP patients.
OBJECTIVE:To determine the prevalence of chronic polyneuropathy in an unselected community-dwelling population of middle-aged and elderly people.
METHODS:The current study was embedded in the ...prospective, population-based Rotterdam Study. Between June 2013 and October 2015, 1,310 participants (mean age 70 years, 55% female) were screened for the presence of polyneuropathy. This screening consisted of a questionnaire, neurologic examination, and nerve conduction studies. Polyneuropathy was diagnosed by a consensus panel that categorized participants into no, possible, probable, or definite polyneuropathy, depending on the level of abnormality of the screening. Medical records were scrutinized to evaluate whether the disorder was diagnosed before and laboratory investigations were performed to determine the presence of associated risk factors.
RESULTS:Prevalence of definite polyneuropathy was 5.5% (95% confidence interval 4.4–6.9), age-standardized to the population of the Netherlands 4.0% (3.1–5.3). Prevalence was higher in male participants (6.7% compared to 4.5%) and increased with age. When combining probable and definite polyneuropathy, age-standardized prevalence was 9.4% (7.9–11.1). Almost half of the polyneuropathies (49%) were newly diagnosed. The majority of polyneuropathies were idiopathic (46%). Diabetes, present in 31% of participants with polyneuropathy, was the most commonly found risk factor.
CONCLUSIONS:Prevalence of polyneuropathy in the general middle-aged and elderly population is at least 4%, and increases with age. Almost half of the cases were newly diagnosed, indicating that the presence of polyneuropathy is underreported or underdiagnosed. Currently, almost half of the polyneuropathies are idiopathic. Future prospective cohort studies should focus on identifying new determinants of polyneuropathy.
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