Apheresis therapy in Guillain-Barré syndrome

Based on previous randomized controlled-trials (RCTs), plasma exchange (PE) has been established as an effective treatment for Guillain-Barré syndrome (GBS). Double filtration plasmapheresis (DFPP) and immunoadsorption plasmapheresis (IAPP) may be effective for GBS, however their effectiveness have not been established because any RCTs have not been available so far. Intravenous immunoglobulin (IVIG) has also been established to be effective for GBS according to several RCTs, however complications have been more frequent in PE than IVIG. Therefore IVIg treatment should be considered as the first choice for GBS, and PE treatment remains as the second-line if IVIg treatment is contraindicated.     

Meeting the challenge of Guillain-Barré syndrome

Acute Guillain-Barré syndrome (GBS) is the most frequent cause of flaccid paralysis in otherwise healthy adults. Here, learn how the health care team can best meet the needs of patients with this challenging, mysterious condition.     

Pilot study of a new treatment concept for acute Guillain-Barré syndrome

The pathogenesis of acute Guillain-Barré syndrome (GBS) is unknown as yet. Numerous clinical and experimental data suggest an autoimmune disorder. Controlled multicenter trials have demonstrated the efficacy of plasma exchange and intravenous immunoglobulin (IgG). A combination of these treatments appears to be a promising concept. We assume that initial selective adsorption treatment eliminates the postulated circulating pathogenetic agent and thereby allows for a more effective immunomodulation by the subsequent administration of i.v. IgG. In a pilot study of 30 patients (11 plasma exchange, 7 selective adsorption, and 12 patients treated with selective adsorption and subsequent administration of i.v. IgG) we found no significant differences for the variables including disability on admission, maximal disability, change of disability during treatment, and disability on discharge. Moreover, we found no apparent differences with respect to tolerance of the procedures or procedural side-effects. We conclude from these preliminary findings that these treatment regimens are viable and safe procedures and result in similar clinical improvement. Statistical evaluation of the efficacy of this treatment concept can only be done after a multicenter study of a large patient group.     

Guillain-Barré syndrome

Guillain-Barré syndrome (GBS) is a group of autoimmune syndromes consisting of demyelinating and acute axonal degenerating forms of the disease. Nerve conduction study helps differentiate the heterogeneous subtypes of GBS. Patients exhibit a progressive paralysis that reaches a plateau phase. In most patients, resolution is complete or near complete. Mortality from GBS most often is associated with dysautonomia and mechanical ventilation. GBS usually is associated with an antecedent infection by one of several known pathogens. Cross-reactivity between the pathogen and the nerve tissue sets up the autoimmune response. Treatment consists of supportive care, ventilatory management (in about one third of patients), and specific therapy with intravenous immunoglobulin or plasmapheresis. Consultation with a neurologist is suggested.     

Treatment of the adult Guillain-Barré syndrome: indications for plasma exchange

The Guillain-Barré syndrome is the most common cause of acute flaccid paralysis. Currently, 5% of patients die and 10% are left with severe motor sequelae at one year. Multidisciplinary teams, trained to specific treatments, are required to manage these patients. Oral and intravenous steroid treatment of GBS has been disappointing. Two large randomized clinical trials comparing plasma exchange (PE) to standard supportive treatment have shown a short-term and a one-year benefit of PE. Appropriate number of exchanges and indications of PE are now more precisely known. Patients with mild forms of the disease (able to walk) should receive two PEs, while a further two exchanges should be done in case of deterioration or in advanced forms (loss of walking ability, mechanical ventilation). A greater number of exchanges does not appeared beneficial. More recently, two randomized trials produced some evidence that intravenous immune globulin (IVIg, 0.4 g/kg daily for five days) and PE had equivalent efficiency in advanced forms. The combination of PE with IVIg did not yield a significant advantage, but did increase cost and risk. In advanced forms, the choice between PE and IVIg depends on the contraindications of each treatment.     

The immunotherapy of Guillain-Barré syndrome

Introduction: Guillain-Barré syndrome is the most common cause of acute flaccid paralysis worldwide. Microorganisms such as Campylobacter jejuni, Cytomegalovirus, Epstein-Barr virus, Mycoplasma pneumoniae, Haemophilus influenzae and Zika virus have been linked to the disease. The most common clinical variants are acute inflammatory demyelinating polyneuropathy and acute motor axonal neuropathy. Plasma exchange and intravenous immunoglobulins are the standard therapy for the disease.

Areas covered: research to elucidate the pathophysiology of Guillain-Barré syndrome has led to the development of drugs directed towards new potential therapeutic targets. This review offers a comprehensive view of the current treatment based upon the physiopathology.

Expert opinion: patients with Guillain-Barré syndrome need a multidisciplinary approach, limitation to walk unaided and disability score are indicators for treatment as well as the presence of autonomic dysfunction and pain. Admission to intensive care units should be considered for those patients presenting with respiratory failure, bulbar involvement and progression of the disease. Research aimed to deciphering the pathophysiology of the disease, discovering new biomarkers and establishing algorithms of prediction of both the disease and its outcomes is warranted.     

Posterior reversible encephalopathy syndrome associated with Guillain-Barré syndrome: Case report and clinical management considerations

Around half of the patients with Guillain-Barré syndrome (GBS) present autonomic dysfunction requiring admission to intensive care unit in up to a quarter of patients. Treatment of GBS consists of plasma exchange (PE) and intravenous immunoglobulins (IVIG). Posterior reversible encephalopathy syndrome (PRES) consists in a reversible subcortical vasogenic brain edema caused by endothelial damage triggered by abrupt blood pressure changes. We report on a woman who presented with PRES in the course of GBS treated first with IVIG, and then with PE. The present report underlines the challenge that the clinicians face when these two rare syndromes concur. The literature is not helpful considering that both blood pressure fluctuations and IVIG are reported to be involved in the pathogenesis of PRES. In the present letter, both pathogenic mechanisms and clinical management considerations are discussed.     

Motor recovery after Guillain-Barré syndrome in childhood

Purpose. To determine the clinical factors that modify the recovery time for gait after Guillain-Barré syndrome (GBS) in childhood.

Method. Medical records of patients admitted to Instituto de Ortopedia Infantil Roosevelt (IOIR) between years 1991 and 2001, were reviewed. Age, sex, cranial nerve impairment, requirement of assisted ventilation, number of days of assisted ventilation, muscular strength at day 10 of the disease, presence of quadriplegia, intravenous infusion of human gamma globulins (IVIG), were taken as independent variables. The number of needed days to reach Hughes State III was taken as the major outcome. First, univariate analysis was performed and with the factors that showed a statistically significant association with recovery time, multiple linear regression analysis and Cox regression were also performed.

Results. Data of 332 children under 15 years old was collected. (Mean age: 7.1 years). A sample of 215 children was gathered for the study, all of them were regarded as functional states IV or V. Acute Motor Axonal Neuropathy (AMAN) was found in 30% of all cases. In the univariate analysis Cranial nerve impairment, requirement of assisted ventilation, presence of quadriplegia and presence of non-excitable motor nerves were associated with delayed motor recovery time. Patients who received IVIG reached Hughes state III faster than those who received only support treatment. This finding, that was more important in the presence of Acute Inflammatory Demyelinating Polyradiculoneuropathy (AIDP), lost its value in the Cox regression analysis. In the multivariate analysis, muscular strength, assessed at day 10 of the disease was the most important predictor to determine motor recovery. The presence of quadriplegia was strongly associated with a delayed recovery time. Relative risk: 3.3 (95% Confidence Interval 2.1 – 5.2).

Conclusions. Muscular strength at day 10 of the disease is the most useful clinical factor to determine prognosis of motor recovery in children who have suffered Guillain-Barré syndrome.     

An uncommon cause of lower limb weakness

We describe a case of a severely mentally disabled patient diagnosed as suffering from Guillain-Barré syndrome and treated with repeated plasma exchange. However, the abrupt onset of a cardiovascular collapse prompted a more in-depth diagnostic workup which demonstrated that the neurologic symptoms were likely to be ascribed to poisoning with heavy metals from a large number of ingested coins and other metallic items. Received: 12 October 1999 Accepted: 19 October 1999     

Prognosis of acute polyneuritis requiring artificial ventilation

We compared two selected groups of patients with acute Guillain-Barré syndrome requiring artificial ventilation. Both groups were treated with conventional therapy, group A (n=35) between 1970 and 1978, group B (n=21) between 1979 and 1983. Neither plasmapheresis nor any other immunosuppressive treatment was applied. Significant differences were found favouring group B: There was a decline of mortality from about 60% to less than 10% (p<0.001) and a smaller number of severe complications due to improvement of supportive care.     

What are the experiences of adults returning to work following recovery from Guillain-Barré syndrome? An interpretative phenomenological analysis

Purpose.?Guillain-Barré syndrome (GBS) is a transient inflammatory disorder affecting peripheral nerves, characterised by weakness and numbness in limbs, upper body and face. Residual problems affect a large minority, and complicate return to work. This qualitative study explored the experiences of people who returned to work following their diagnosis of GBS and recovery, to gain insight into factors that facilitated or inhibited this process.

Method.?Five people participated in in-depth interviews. Individual and common experiences were explored through interpretative phenomenological analysis.

Findings.?Three recurring themes are presented: the perceived value of work; losing and recovering a familiar identity at work; and dilemmas around using support and adaptations at work. Certain individual issues also emerged but are beyond the scope of this article. Participants tended to measure their recovery in terms of returning to work yet continued to experience certain physical and psychosocial difficulties at work related to GBS, which required active coping strategies. Limited public awareness of GBS was perceived as a hindrance when returning to work.

Conclusion.?This study provides a rich account of the experiences that people encounter returning to work following GBS. Rehabilitation specialists may offer more effective preparation for this process, drawing upon the issues identified.