Treatment of Guillain-Barre syndrome and CIDP
ABSTRACT Guillain-Barré syndrome (GBS) and chronic inflammatory demyelinating poly-(radiculo)neuropathy (CIDP) are immune-mediated disorders with a variable duration of progression and a range in severity of weakness. Infections can trigger GBS and exacerbate CIDP. Anti-ganglioside antibodies are important, but there is debate on the role of genetic factors in the pathogenesis of these disorders. Randomized controlled trials (RCT) have shown that intravenous immunoglobulin (IVIg) and plasma exchange (PE) are effective in both GBS and CIDP. Most CIDP patients also improve after steroid therapy. Despite current treatment options, many patients have residual deficits or need to be treated for a long period of time. Therefore, new treatment trials are highly indicated. This review focuses on the current and possible new treatment options that could be guided by recent results from laboratory experiments.
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ABSTRACT: Background: Guillain Barré Syndrome (GBS) has a variable clinical course. The influence of season on the rate of recovery has not been evaluated previously, despite documentation of seasonal variation in the occurrence of GBS. This study evaluated the influence of season on the rate of recovery from GBS. Materials and Methods: Records of 184 patients with GBS over a 10-year period were reviewed. Patients were divided into four groups depending on the date of admission: Q1 (March-May), Q2 (June-August), Q3 (September-November), and Q4 (December-February). Demographic characteristics and recovery characteristics (duration of mechanical ventilation, ICU and hospital stay, and time for recovery from the time of initiation of definitive therapy) were compared across the four quarters. Results: There was no significant difference in age, antecedent illnesses, treatment received, electrophysiological findings, and muscle power at admission across the four groups. Significant differences among various seasons were found with respect to duration of mechanical ventilation (23 ± 20, 36 ± 34, 27 ± 22, and 38 ± 28 days for Q1-Q4, respectively; P = 0.05), ICU stay (27 ± 22, 40 ± 37, 31 ± 23, and 43 ± 30 days for Q1-Q4, respectively; P = 0.05), hospital stay (42 ± 28, 55 ± 44, 47 ± 34, and 72 ± 54 days for Q1-Q4, respectively; P = 0.02), and time for recovery from treatment (15 ± 14, 29 ± 34, 18 ± 14, and 29 ± 20 days for Q1-Q4, respectively; P = 0.02). Conclusions: This study demonstrates a seasonal variation in the recovery of patients with GBS requiring mechanical ventilation. Patients admitted in Q1 have the fastest recovery and those in Q4 have the slowest recovery.Neurology India 09/2013; 61(4):349-54. DOI:10.4103/0028-3886.117582 · 1.08 Impact Factor
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ABSTRACT: Chapter 1 is the general introduction of this thesis, in which di.erent aspects of the Guillain-Barré syndrome (GBS) are described. GBS is an acute post-infectious polyneuropathy, in which the immune system plays an important role. Insight in pathogenesis, clinical and electrophysiological features, functional outcome, and residual complaints is increasing. To date, fatigue is considered one of the most disabling residual symptoms, seriously a.ecting quality of life. The considerable percentages of (long-lasting) morbidity and mortality, as well as the residual complaints of severe fatigue, were the most important reasons to initiate this study, entitled; ‘treatment of GBS and causes and treatment of residual fatigue’. Items discussed in the introduction range from diagnosis to pathogenesis and treatment of GBS. Attention is partially focused towards prognosis and outcome, in particular towards residual fatigue. Chapter 2.1 is a review about the peripheral neuropathies GBS, chronic in.ammatory demyelinating polyneuropathy (CIDP) and multifocal motor neuropathy (MMN), all potentially treatable immune-mediated disorders. It was postulated that the use of appropriate assessment scales to evaluate the e.ects of treatment is essential. Recent clinical trials and Cochrane reviews on the e.ect of various treatments in patients with GBS, CIDP and MMN are discussed. It is concluded that intravenous immunoglobulin (IVIg) remains the cornerstone of treatment for GBS, but that combinations of treatment may be even more e.ective. Also studies on prognostic factors related to improvement are discussed. Whether patients with Miller Fisher syndrome or those with mild GBS should also be treated remains a matter of debate. IVIg is the best studied therapy for CIDP patients. IVIg is e.ective and is one of the most important treatments in these patients. Whether steroids are able to eradicate the disease besides suppressing disease activity in CIDP remains to be established. Some GBS patients have secondary deterioration or .nally turn out to have CIDP; additional information in this group of patients may lead to more appropriate disease management. Most patients with CIDP and those with MMN need long-term treatment. New treatment strategies should also focus on the e.ect and the costs of treatment during long-term follow up. In chapter 2.2 a multi-center open label study on the additional e.ect of Mycophenolate Mofetil (MM) when added to the combination of IVIg and intravenous methylprednisolone (MP) is described. An important reason for this study was that, despite di.erent treatments, GBS remains associated with considerable co-morbidity, mortality, and long-lasting residual de. cits. The aims of this pilot study were to investigate the safety of this treatment combination and to study the potential additional e.ect of MM on the outcome in patients with GBS. Patients were treated for 6 consecutive weeks with MM in a dosage of 2g daily, and for 5 days with 0.4 g/kg of body weight IVIg and 500 mg MP daily. Because the IVIg-MP treatment group of the Dutch IVIg-MP trial was used as control group, identical outcome measures were used. The primary endpoint was the percentage of patients showing improvement with at least one grade on the GBS-disability scale, 4 weeks after inclusion. Twenty-six patients were included in analysis. Sixty- 189 two percent reached the primary endpoint in the group of patients treated with IVIg-MP-MM, as compared with 68% in the group treated with IVIg-MP (OR 1.3, 95% CI 0.6-3.2, p=0.54). None of the secondary endpoints showed any signi.cant di.erences either. Complications and adverse events were comparable in both groups. It was concluded that although side e.ects were generally mild, there appears to be no signi.cant improved outcome in the group of patients additionally treated with MM. It was concluded that new treatment studies are warranted. In chapter 3.1 a double-blind, placebo controlled, cross-over study is described, aiming to reduce fatigue with amantadine treatment, in 80 severely fatigued GBS patients. Fatigue was assessed using the Fatigue Severity Scale (FSS) and Fatigue Impact Scale (FIS). Patients could participate when they were severely fatigued (de.ned as FSS = 5.0), and other possible confounding factors causing fatigue were excluded. Amantadine was not superior to placebo. However, some reduction of fatigue was observed both during the pre-treatment period and during the consecutive visits, both in the placebo and amantadine treated group, suggesting that increased attention for fatigue provided by this study already had an ameliorating in.uence on fatigue. Also secondary outcome measures including impact of fatigue, anxiety and depression, handicap, and quality of life did not reveal any signi.cant di.erence. Fatigue scores obtained from the FSS (fatigue severity) as well as from the FIS (impact of fatigue on cognitive, physical, and social functioning) showed the same trends and changes during the consecutive follow-up visits. In chapter 3.2 the favourable results are discussed of a 12-week during bicycle exercise training in 20 severely fatigued patients. Sixteen patients were relatively good recovered from GBS and 4 had stable chronic in.ammatory demyelinating polyneuropathy (CIDP). Selfreported fatigue scores as measured with the FSS, as well as aerobic capacity and isokinetic muscle strength improved signi.cantly compared to baseline measurements. Daily physical activity, as measured with the Rotterdam activity monitor (RAM) showed that endurance related physical activities (e.g., duration of standing, walking, cycling and transitions from positions), did not show any signi.cant improvement. It was suggested that changing the level of daily physical activity is not an important adaptation strategy in these fatigued patients. Maybe the maintenance of normal activity patterns contributes to fatigue. Both GBS and CIDP patients showed comparable signi.cant improvements on the FSS and FIS. Patients reported that increased physical activity in the past often resulted in increased neurological complaints, resembling the initial phase of GBS or CIDP, and resulting in threatening and anxious feelings of getting a relapse. This medical supervised training showed the opposite, patients became con.dent, and a negative circle seemed to be broken. It was suggested that the motivational aspect of increased social contacts with fellow-patients, besides promoting exercise adherence, has also lead to better psychological performances. Quality of life improved, mainly on the physically focused domains of the SF-36, suggesting being a result of improved physical .tness and muscle strength. Most patients (80%) were motivated to continue with regular training activities. Marcel BW.indd 189 02-11-2005 16:23:25 In chapter 3.3 the long-term follow-up of fatigue, quality of life, physical .tness and muscle strength in GBS and CIDP patients is described, evaluated 2 years after training intervention. Mean reduction of self-reported fatigue compared to pre-training values was not di.erent from the fatigue reduction observed directly after the training intervention. Also maximal ‘power output’and muscle strength (except elbow .exion), remained signi.cantly improved compared to pre-training values. It seemed that lower fatigue scores and improved functional scores were not a result of natural reduction of fatigue after GBS nor could be explained by ongoing reconditioning activities. Patients became more convinced of their physical capabilities, and were less afraid and more capable to perform rather high level (short-lasting) maximal physical e.ort, as measured by the increased maximal power output and muscle strength measurements. Presumably, training has taught the patients that increased physical activity can be performed without getting new neurological complaints or ‘relapse’feelings, and thus helped them to take the barrier. Although some methodological remarks had to be made, these results favoured a long-term e.ect of a physical training intervention. The results of a cross-sectional study within a prospective nationwide study in the Netherlands, in which the presence of fatigue after GBS was investigated, is described in chapter 4.1. The occurrence of severe fatigue, and its relation with disease course, clinical characteristics, and antecedent infections was studied in 100 GBS patients. Severe fatigue, again expressed as a mean FSS-score of at least 5.0, was present in 60% of all patients; it was more frequently present in females and in patients over 50 years (pStroke 01/2005; · 6.02 Impact Factor