Hughes RA, Newsom-Davis JM, Perkin GD, Pierce JM.
Controlled trial prednisolone in acute polyneuropathy.
Lancet. 1978 Oct 7;2(8093):750-3. doi: 10.1016/s0140-6736(78)92644-2.
Abstract/Text
In a multicentre, randomised trial of prednisolone in acute polyneuropathy of undetermined aetiology (Guillain-Barré syndrome), 21 patients were treated with prednisolone (60 mg daily for one week, 40 mg daily for four days, and then 30 mg daily for three days) and 19 did not have steroid treatment. Patients were graded on a six-point scale by one of two neurologists who had no knowledge of the treatment schedule. Reassessment at one, three, and twelve months consistently showed greater improvement in the control than the prednisolone group but the only statistically significant result was in the improvement at three months among patients entered to the trial within a week of onset of illness. The 6 control patients had improved by 2.5 +/- 0.43 grades by three months from entry to the trial whereas the 10 prednisolone patients had only improved by 0.9 +/- 0.46 grades (P less than 0.05). There was 1 death related to the polyneuropathy in each group, and 1 suicide in a control patient during convalescence. 6 prednisolone patients were left with considerable disability compared with 1 control patient. There were 3 relapses in the prednisolone group, but none in the control group. The results indicate that steroid treatment is not beneficial and can be detrimental in acute neuropathy of undetermined aetiology.
Walgaard C, Lingsma HF, Ruts L, van Doorn PA, Steyerberg EW, Jacobs BC.
Early recognition of poor prognosis in Guillain-Barre syndrome.
Neurology. 2011 Mar 15;76(11):968-75. doi: 10.1212/WNL.0b013e3182104407.
Abstract/Text
BACKGROUND: Guillain-Barré syndrome (GBS) has a highly diverse clinical course and outcome, yet patients are treated with a standard therapy. Patients with poor prognosis may benefit from additional treatment, provided they can be identified early, when nerve degeneration is potentially reversible and treatment is most effective. We developed a clinical prognostic model for early prediction of outcome in GBS, applicable for clinical practice and future therapeutic trials.
METHODS: Data collected prospectively from a derivation cohort of 397 patients with GBS were used to identify risk factors of being unable to walk at 4 weeks, 3 months, and 6 months. Potential predictors of poor outcome (unable to walk unaided) were considered in univariable and multivariable logistic regression models. The clinical model was based on the multivariable logistic regression coefficients of selected predictors and externally validated in an independent cohort of 158 patients with GBS.
RESULTS: High age, preceding diarrhea, and low Medical Research Council sumscore at hospital admission and at 1 week were independently associated with being unable to walk at 4 weeks, 3 months, and 6 months (all p 0.05-0.001). The model can be used at hospital admission and at day 7 of admission, the latter having a better predictive ability for the 3 endpoints; the area under the receiver operating characteristic curve (AUC) is 0.84-0.87 and at admission the AUC is 0.73-0.77. The model proved to be valid in the validation cohort.
CONCLUSIONS: A clinical prediction model applicable early in the course of disease accurately predicts the first 6 months outcome in GBS.
Luijten LWG, Doets AY, Arends S, Dimachkie MM, Gorson KC, Islam B, Kolb NA, Kusunoki S, Papri N, Waheed W, Walgaard C, Yamagishi Y, Lingsma H, Jacobs BC; IGOS Consortium.
Modified Erasmus GBS Respiratory Insufficiency Score: a simplified clinical tool to predict the risk of mechanical ventilation in Guillain-Barré syndrome.
J Neurol Neurosurg Psychiatry. 2023 Apr;94(4):300-308. doi: 10.1136/jnnp-2022-329937. Epub 2022 Nov 25.
Abstract/Text
BACKGROUND: This study aimed to determine the clinical and diagnostic factors associated with mechanical ventilation (MV) in Guillain-Barré syndrome (GBS) and to simplify the existing Erasmus GBS Respiratory Insufficiency Score (EGRIS) for predicting the risk of MV.
METHODS: Data from the first 1500 patients included in the prospective International GBS Outcome Study (IGOS) were used. Patients were included across five continents. Patients <6 years and patients from Bangladesh were excluded. Univariable logistic and multivariable Cox regression were used to determine which prespecified clinical and diagnostic characteristics were associated with MV and to predict the risk of MV at multiple time points during disease course.
RESULTS: 1133 (76%) patients met the study criteria. Independent predictors of MV were a shorter time from onset of weakness until admission, the presence of bulbar palsy and weakness of neck flexion and hip flexion. The modified EGRIS (mEGRIS) was based on these factors and accurately predicts the risk of MV with an area under the curve (AUC) of 0.84 (0.80-0.88). We internally validated the model within the full IGOS cohort and within separate regional subgroups, which showed AUC values of 0.83 (0.81-0.88) and 0.85 (0.72-0.98), respectively.
CONCLUSIONS: The mEGRIS is a simple and accurate tool for predicting the risk of MV in GBS. Compared with the original model, the mEGRIS requires less information for predictions with equal accuracy, can be used to predict MV at multiple time points and is also applicable in less severely affected patients and GBS variants. Model performance was consistent across different regions.
© Author(s) (or their employer(s)) 2023. No commercial re-use. See rights and permissions. Published by BMJ.
Yamagishi Y, Suzuki H, Sonoo M, Kuwabara S, Yokota T, Nomura K, Chiba A, Kaji R, Kanda T, Kaida K, Ikeda SI, Mutoh T, Yamasaki R, Takashima H, Matsui M, Nishiyama K, Sobue G, Kusunoki S.
Markers for Guillain-Barré syndrome with poor prognosis: a multi-center study.
J Peripher Nerv Syst. 2017 Dec;22(4):433-439. doi: 10.1111/jns.12234. Epub 2017 Sep 11.
Abstract/Text
Guillain-Barré syndrome (GBS) is an acute monophasic neuropathy. Prognostic tools include the modified Erasmus GBS outcome score (mEGOS), Erasmus GBS respiratory insufficiency score (EGRIS), and the increase in serum IgG levels (ΔIgG) 2 weeks after intravenous immunoglobulin (IVIg) treatment. Given that proportions of GBS subtypes differ between Western countries and Japan, the usefulness of these tools in Japan or other countries remains unknown. We enrolled 177 Japanese patients with GBS from 15 university hospitals and retrospectively obtained mEGOS and EGRIS for all and ΔIgG status for 79 of them. High mEGOS scores on admission or on day 7 were significantly associated with poorer outcomes (unable to walk independently at 6 months). High EGRIS scores (≥5 points) were associated with an increased risk for mechanical ventilation. Patients with ΔIgG <1,108 mg/dl had significantly poorer outcomes. We suggest that mEGOS, EGRIS, and ΔIgG in GBS are clinically relevant in Japan.
© 2017 Peripheral Nerve Society.
Rajabally YA, Uncini A.
Outcome and its predictors in Guillain-Barre syndrome.
J Neurol Neurosurg Psychiatry. 2012 Jul;83(7):711-8. doi: 10.1136/jnnp-2011-301882. Epub 2012 May 7.
Abstract/Text
Despite the use of plasma exchanges and intravenous immunoglobulins, Guillain-Barré syndrome (GBS) still carries non-negligible morbidity and mortality. Furthermore, the psychosocial consequences of GBS may persist longer than expected. Various aetiological, clinical, electrophysiological and immunological factors may carry prognostic predictive value. The objective of this article was to perform a summary of the current knowledge-base on outcome and its determinants in adequately-treated adult-onset GBS. Relevant prospective literature was reviewed through a Medline search of English-language articles published between 1966 and March 2012. GBS causes severe persistent disability in 14% of patients at 1 year. Loss of full strength, persistent pain and need for professional change occurs in about 40%. Mortality is of about 4% within the first year. Analysis of prognostic predictors consistently demonstrates the negative impact of higher age, preceding diarrhoea, greater disability/weaker muscles at admission, short interval between symptom-onset and admission, mechanical ventilation and absent/low amplitude compound muscle action potentials. Further outcome studies will soon be underway and may in future contribute to adequately integrate all potential factors in more reliable predictive models.
斎藤豊和,有村公良,納 光弘.ギラン・バレー症候群全国疫学調査:第二次アンケート調査の結果報告.厚生省特定疾患 免疫性神経疾患調査研究分科会 平成11年度研究報告書.2000; 83-84.
荻野美恵子,斎藤豊和,有村公良,納 光弘.Guillain-Barré症候群の全国調査―第3次調査結果を含めた最終報告―.厚生省特定疾患 免疫性神経疾患調査研究分科会 平成12年度研究報告書.2001; 59-101.
Mitsui Y, Kusunoki S, Arimura K, Kaji R, Kanda T, Kuwabara S, Sonoo M, Takada K; Japanese GBS Study Group.
A multicentre prospective study of Guillain-Barré syndrome in Japan: a focus on the incidence of subtypes.
J Neurol Neurosurg Psychiatry. 2015 Jan;86(1):110-4. doi: 10.1136/jnnp-2013-306509. Epub 2013 Nov 22.
Abstract/Text
OBJECTIVE: Guillain-Barré Syndrome (GBS) is classified into the two major subtypes; acute inflammatory demyelinating polyneuropathy (AIDP) and acute motor axonal neuropathy (AMAN). Previous studies have suggested that AIDP is predominant and AMAN is rare in Western countries, whereas AMAN is not always uncommon in East Asia. We aimed to clarify the incidence of the subtypes of GBS in Japan.
METHODS: We performed a prospective multicentre survey over 3 years (2007-2010). Clinical and electrophysiological findings were collected from 184 patients with GBS in 23 tertiary neurology institutes. Anti-ganglioside antibodies were measured by ELISA. We also surveyed the incidence of Fisher syndrome (FS).
RESULTS: By electrodiagnostic criteria of Ho et al, patients were classified as having AIDP (40%), or AMAN (22%), or unclassified (38%). Anti-GM1 IgG antibodies were found for 47% of AMAN patients, and 18% of AIDP patients (p<0.001). There were no specific regional trends of the electrodiagnosis and anti-GM1 positivity. During the same study period, 79 patients with FS were identified; the percentage of FS cases out of all cases (FS/(GBS+FS)) was 26%.
CONCLUSIONS: The frequency of GBS patients with the electrodiagnosis of AMAN by single nerve conduction studies is approximately 20% in Japan, and the AMAN pattern is closely associated with anti-GM1 antibodies. The incidence of FS appears to be much higher in Japan than in Western countries.
Published by the BMJ Publishing Group Limited. For permission to use (where not already granted under a licence) please go to http://group.bmj.com/group/rights-licensing/permissions.
Krauer F, Riesen M, Reveiz L, Oladapo OT, Martínez-Vega R, Porgo TV, Haefliger A, Broutet NJ, Low N; WHO Zika Causality Working Group.
Zika Virus Infection as a Cause of Congenital Brain Abnormalities and Guillain-Barré Syndrome: Systematic Review.
PLoS Med. 2017 Jan;14(1):e1002203. doi: 10.1371/journal.pmed.1002203. Epub 2017 Jan 3.
Abstract/Text
BACKGROUND: The World Health Organization (WHO) stated in March 2016 that there was scientific consensus that the mosquito-borne Zika virus was a cause of the neurological disorder Guillain-Barré syndrome (GBS) and of microcephaly and other congenital brain abnormalities based on rapid evidence assessments. Decisions about causality require systematic assessment to guide public health actions. The objectives of this study were to update and reassess the evidence for causality through a rapid and systematic review about links between Zika virus infection and (a) congenital brain abnormalities, including microcephaly, in the foetuses and offspring of pregnant women and (b) GBS in any population, and to describe the process and outcomes of an expert assessment of the evidence about causality.
METHODS AND FINDINGS: The study had three linked components. First, in February 2016, we developed a causality framework that defined questions about the relationship between Zika virus infection and each of the two clinical outcomes in ten dimensions: temporality, biological plausibility, strength of association, alternative explanations, cessation, dose-response relationship, animal experiments, analogy, specificity, and consistency. Second, we did a systematic review (protocol number CRD42016036693). We searched multiple online sources up to May 30, 2016 to find studies that directly addressed either outcome and any causality dimension, used methods to expedite study selection, data extraction, and quality assessment, and summarised evidence descriptively. Third, WHO convened a multidisciplinary panel of experts who assessed the review findings and reached consensus statements to update the WHO position on causality. We found 1,091 unique items up to May 30, 2016. For congenital brain abnormalities, including microcephaly, we included 72 items; for eight of ten causality dimensions (all except dose-response relationship and specificity), we found that more than half the relevant studies supported a causal association with Zika virus infection. For GBS, we included 36 items, of which more than half the relevant studies supported a causal association in seven of ten dimensions (all except dose-response relationship, specificity, and animal experimental evidence). Articles identified nonsystematically from May 30 to July 29, 2016 strengthened the review findings. The expert panel concluded that (a) the most likely explanation of available evidence from outbreaks of Zika virus infection and clusters of microcephaly is that Zika virus infection during pregnancy is a cause of congenital brain abnormalities including microcephaly, and (b) the most likely explanation of available evidence from outbreaks of Zika virus infection and GBS is that Zika virus infection is a trigger of GBS. The expert panel recognised that Zika virus alone may not be sufficient to cause either congenital brain abnormalities or GBS but agreed that the evidence was sufficient to recommend increased public health measures. Weaknesses are the limited assessment of the role of dengue virus and other possible cofactors, the small number of comparative epidemiological studies, and the difficulty in keeping the review up to date with the pace of publication of new research.
CONCLUSIONS: Rapid and systematic reviews with frequent updating and open dissemination are now needed both for appraisal of the evidence about Zika virus infection and for the next public health threats that will emerge. This systematic review found sufficient evidence to say that Zika virus is a cause of congenital abnormalities and is a trigger of GBS.
Palaiodimou L, Stefanou MI, Katsanos AH, Fragkou PC, Papadopoulou M, Moschovos C, Michopoulos I, Kokotis P, Bakirtzis C, Naska A, Vassilakopoulos TI, Chroni E, Tsiodras S, Tsivgoulis G.
Prevalence, clinical characteristics and outcomes of Guillain-Barré syndrome spectrum associated with COVID-19: A systematic review and meta-analysis.
Eur J Neurol. 2021 Oct;28(10):3517-3529. doi: 10.1111/ene.14860. Epub 2021 Apr 28.
Abstract/Text
BACKGROUND AND PURPOSE: Mounting evidence supports an association between Guillain-Barré syndrome spectrum (GBSs) and severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infection. However, GBSs in the setting of coronavirus disease 2019 (COVID-19) remains poorly characterized, whilst GBSs prevalence amongst COVID-19 patients has not been previously systematically evaluated using a meta-analytical approach.
METHODS: A systematic review and meta-analysis of observational cohort and case series studies reporting on the occurrence, clinical characteristics and outcomes of patients with COVID-19-associated GBSs was performed. A random-effects model was used to calculate pooled estimates and odds ratios (ORs) with corresponding 95% confidence intervals (CIs), compared to non-COVID-19, contemporary or historical GBSs patients.
RESULTS: Eighteen eligible studies (11 cohorts, seven case series) were identified including a total of 136,746 COVID-19 patients. Amongst COVID-19 patients, including hospitalized and non-hospitalized cases, the pooled GBSs prevalence was 0.15‰ (95% CI 0%-0.49‰; I2 = 96%). Compared with non-infected contemporary or historical controls, patients with SARS-CoV-2 infection had increased odds for demyelinating GBSs subtypes (OR 3.27, 95% CI 1.32%-8.09%; I2 = 0%). In SARS-CoV-2-infected patients, olfactory or concomitant cranial nerve involvement was noted in 41.4% (95% CI 3.5%-60.4%; I2 = 46%) and 42.8% (95% CI 32.8%-53%; I2 = 0%) of the patients, respectively. Clinical outcomes including in-hospital mortality were comparable between COVID-19 GBSs patients and non-infected contemporary or historical GBSs controls.
CONCLUSION: GBSs prevalence was estimated at 15 cases per 100,000 SARS-CoV-2 infections. COVID-19 appears to be associated with an increased likelihood of GBSs and with demyelinating GBSs variants in particular.
© 2021 European Academy of Neurology.
Keh RYS, Scanlon S, Datta-Nemdharry P, Donegan K, Cavanagh S, Foster M, Skelland D, Palmer J, Machado PM, Keddie S, Carr AS, Lunn MP; BPNS/ABN COVID-19 Vaccine GBS Study Group.
COVID-19 vaccination and Guillain-Barré syndrome: analyses using the National Immunoglobulin Database.
Brain. 2023 Feb 13;146(2):739-748. doi: 10.1093/brain/awac067.
Abstract/Text
Vaccination against viruses has rarely been associated with Guillain-Barré syndrome (GBS), and an association with the COVID-19 vaccine is unknown. We performed a population-based study of National Health Service data in England and a multicentre surveillance study from UK hospitals to investigate the relationship between COVID-19 vaccination and GBS. Firstly, case dates of GBS identified retrospectively in the National Immunoglobulin Database from 8 December 2021 to 8 July 2021 were linked to receipt dates of COVID-19 vaccines using data from the National Immunisation Management System in England. For the linked dataset, GBS cases temporally associated with vaccination within a 6-week risk window of any COVID-19 vaccine were identified. Secondly, we prospectively collected incident UK-wide (four nations) GBS cases from 1 January 2021 to 7 November 2021 in a separate UK multicentre surveillance database. For this multicentre UK-wide surveillance dataset, we explored phenotypes of reported GBS cases to identify features of COVID-19 vaccine-associated GBS. Nine hundred and ninety-six GBS cases were recorded in the National Immunoglobulin Database from January to October 2021. A spike of GBS cases above the 2016-2020 average occurred in March-April 2021. One hundred and ninety-eight GBS cases occurred within 6 weeks of the first-dose COVID-19 vaccination in England [0.618 cases per 100,000 vaccinations; 176 ChAdOx1 nCoV-19 (AstraZeneca), 21 tozinameran (Pfizer) and one mRNA-1273 (Moderna)]. The 6-week excess of GBS (compared to the baseline rate of GBS cases 6-12 weeks after vaccination) occurred with a peak at 24 days post-vaccination; first-doses of ChAdOx1 nCoV-19 accounted for the excess. No excess was seen for second-dose vaccination. The absolute number of excess GBS cases from January-July 2021 was between 98-140 cases for first-dose ChAdOx1 nCoV-19 vaccination. First-dose tozinameran and second-dose of any vaccination showed no excess GBS risk. Detailed clinical data from 121 GBS patients were reported in the separate multicentre surveillance dataset during this timeframe. No phenotypic or demographic differences identified between vaccine-associated and non-vaccinated GBS cases occurring in the same timeframe. Analysis of the linked NID/NIMS dataset suggested that first-dose ChAdOx1 nCoV-19 vaccination is associated with an excess GBS risk of 0.576 (95% confidence interval 0.481-0.691) cases per 100 000 doses. However, examination of a multicentre surveillance dataset suggested that no specific clinical features, including facial weakness, are associated with vaccination-related GBS compared to non-vaccinated cases. The pathogenic cause of the ChAdOx1 nCoV-19 specific first dose link warrants further study.
© The Author(s) 2022. Published by Oxford University Press on behalf of the Guarantors of Brain.
Kao JC, Liao B, Markovic SN, Klein CJ, Naddaf E, Staff NP, Liewluck T, Hammack JE, Sandroni P, Finnes H, Mauermann ML.
Neurological Complications Associated With Anti-Programmed Death 1 (PD-1) Antibodies.
JAMA Neurol. 2017 Oct 1;74(10):1216-1222. doi: 10.1001/jamaneurol.2017.1912.
Abstract/Text
IMPORTANCE: Neurological complications are an increasingly recognized consequence of the use of anti-programmed death 1 (PD-1) antibodies in the treatment of solid-organ tumors, with an estimated frequency of 4.2%. To date, the clinical spectrum and optimum treatment approach are not established.
OBJECTIVE: To investigate the frequency, clinical spectrum, and optimum treatment approach to neurological complications associated with anti-PD-1 therapy.
DESIGN, SETTING, AND PARTICIPANTS: This single-center, retrospective cohort study was conducted from either September or December 2014 (the approval dates of the study drugs by the US Food and Drug Administration) to May 19, 2016. All patients receiving anti-PD-1 monoclonal antibodies were identified using the Mayo Cancer Pharmacy Database. Patients with development of neurological symptoms within 12 months of anti-PD-1 therapy were included. Patients with neurological complications directly attributable to metastatic disease or other concurrent cancer-related treatments were excluded.
MAIN OUTCOMES AND MEASURES: Clinical and pathological characteristics, time to development of neurological symptoms, and modified Rankin Scale (mRS) score.
RESULTS: Among 347 patients treated with anti-PD1 monoclonal antibodies (pembrolizumab or nivolumab), 10 (2.9%) developed subacute onset of neurological complications. Seven patients were receiving pembrolizumab, and 3 patients were receiving nivolumab. The patients included 8 men and 2 women. Their median age was 71 years (age range, 31-78 years). Neurological complications occurred after a median of 5.5 (range, 1-20) cycles of anti-PD-1 inhibitors. Complications included myopathy (n = 2), varied neuropathies (n = 4), cerebellar ataxia (n = 1), autoimmune retinopathy (n = 1), bilateral internuclear ophthalmoplegia (n = 1), and headache (n = 1). Peripheral neuropathies included axonal and demyelinating polyradiculoneuropathies (n = 2), length-dependent neuropathies (n = 1), and asymmetric vasculitic neuropathy (n = 1). The time to maximum symptom severity varied from 1 day to more than 3 months. The median mRS score was 2.5 (range, 1-5), indicating mild to moderate disability. Five patients experienced other systemic immune-mediated complications, including hypothyroidism (n = 3), colitis (n = 2), and hepatitis (n = 1). Treatment with anti-PD-1 antibodies was discontinued in 7 patients. Treatment included corticosteroids (n = 7), intravenous immunoglobulin (n = 3), and plasma exchange (n = 1). Nine patients improved, with a median mRS score of 2 (range, 0-6). One patient with severe necrotizing myopathy died.
CONCLUSIONS AND RELEVANCE: Neurological adverse events associated with anti-PD-1 therapy have a diverse phenotype, with more frequent neuromuscular complications. Although rare, they will likely be encountered with increasing frequency as anti-PD-1 therapy expands to other cancers. The time of onset is unpredictable, and evolution may be rapid and life-threatening. Prompt recognition and discontinuation of anti-PD-1 therapy is recommended. In some cases, immune rescue treatment may be required.
Chakraborty T, Kramer CL, Wijdicks EFM, Rabinstein AA.
Dysautonomia in Guillain-Barré Syndrome: Prevalence, Clinical Spectrum, and Outcomes.
Neurocrit Care. 2020 Feb;32(1):113-120. doi: 10.1007/s12028-019-00781-w.
Abstract/Text
BACKGROUND: Guillain-Barré syndrome (GBS), when severe, involves the autonomic nervous system; our objective was to assess the spectrum and predictors of dysautonomia, and how it may impact functional outcomes.
METHODS: A retrospective review of patients admitted to the Mayo Clinic in Rochester, MN between January 1, 2000, and December 31, 2017, with GBS and dysautonomia was performed. Demographics, comorbidities, parameters of dysautonomia, clinical course, GBS disability score, and Erasmus GBS Outcome Score (EGOS) at discharge were recorded.
RESULTS: One hundred eighty seven patients were included with 71 (38%) noted to have at least one manifestation of dysautonomia. There are 72% of patients with a demyelinating form of GBS and 36% of patients with demyelination had dysautonomia. Ileus (42%), hypertension (39%), hypotension (37%), fever (29%), tachycardia or bradycardia (27%), and urinary retention (24%) were the most common features. Quadriparesis, bulbar and neck flexor weakness, and mechanical ventilation were associated with autonomic dysfunction. Patients with dysautonomia more commonly had cardiogenic complications, syndrome of inappropriate antidiuretic hormone, posterior reversible encephalopathy syndrome, and higher GBS disability score and EGOS. Mortality was 6% in patients with dysautonomia versus 2% in the entire cohort (P = 0.02).
CONCLUSIONS: Dysautonomia in GBS is a manifestation of more severe involvement of the peripheral nervous system. Accordingly, mortality and functional outcomes are worse. There is a need to investigate if more aggressive treatment is warranted in this category of GBS.
van Doorn PA, Van den Bergh PYK, Hadden RDM, Avau B, Vankrunkelsven P, Attarian S, Blomkwist-Markens PH, Cornblath DR, Goedee HS, Harbo T, Jacobs BC, Kusunoki S, Lehmann HC, Lewis RA, Lunn MP, Nobile-Orazio E, Querol L, Rajabally YA, Umapathi T, Topaloglu HA, Willison HJ.
European Academy of Neurology/Peripheral Nerve Society Guideline on diagnosis and treatment of Guillain-Barré syndrome.
Eur J Neurol. 2023 Dec;30(12):3646-3674. doi: 10.1111/ene.16073. Epub 2023 Oct 10.
Abstract/Text
Guillain-Barré syndrome (GBS) is an acute polyradiculoneuropathy. Symptoms may vary greatly in presentation and severity. Besides weakness and sensory disturbances, patients may have cranial nerve involvement, respiratory insufficiency, autonomic dysfunction and pain. To develop an evidence-based guideline for the diagnosis and treatment of GBS, using Grading of Recommendations, Assessment, Development and Evaluation (GRADE) methodology a Task Force (TF) of the European Academy of Neurology (EAN) and the Peripheral Nerve Society (PNS) constructed 14 Population/Intervention/Comparison/Outcome questions (PICOs) covering diagnosis, treatment and prognosis of GBS, which guided the literature search. Data were extracted and summarised in GRADE Summaries of Findings (for treatment PICOs) or Evidence Tables (for diagnostic and prognostic PICOs). Statements were prepared according to GRADE Evidence-to-Decision (EtD) frameworks. For the six intervention PICOs, evidence-based recommendations are made. For other PICOs, good practice points (GPPs) are formulated. For diagnosis, the principal GPPs are: GBS is more likely if there is a history of recent diarrhoea or respiratory infection; CSF examination is valuable, particularly when the diagnosis is less certain; electrodiagnostic testing is advised to support the diagnosis; testing for anti-ganglioside antibodies is of limited clinical value in most patients with typical motor-sensory GBS, but anti-GQ1b antibody testing should be considered when Miller Fisher syndrome (MFS) is suspected; nodal-paranodal antibodies should be tested when autoimmune nodopathy is suspected; MRI or ultrasound imaging should be considered in atypical cases; and changing the diagnosis to acute-onset chronic inflammatory demyelinating polyradiculoneuropathy (A-CIDP) should be considered if progression continues after 8 weeks from onset, which occurs in around 5% of patients initially diagnosed with GBS. For treatment, the TF recommends intravenous immunoglobulin (IVIg) 0.4 g/kg for 5 days, in patients within 2 weeks (GPP also within 2-4 weeks) after onset of weakness if unable to walk unaided, or a course of plasma exchange (PE) 12-15 L in four to five exchanges over 1-2 weeks, in patients within 4 weeks after onset of weakness if unable to walk unaided. The TF recommends against a second IVIg course in GBS patients with a poor prognosis; recommends against using oral corticosteroids, and weakly recommends against using IV corticosteroids; does not recommend PE followed immediately by IVIg; weakly recommends gabapentinoids, tricyclic antidepressants or carbamazepine for treatment of pain; does not recommend a specific treatment for fatigue. To estimate the prognosis of individual patients, the TF advises using the modified Erasmus GBS outcome score (mEGOS) to assess outcome, and the modified Erasmus GBS Respiratory Insufficiency Score (mEGRIS) to assess the risk of requiring artificial ventilation. Based on the PICOs, available literature and additional discussions, we provide flow charts to assist making clinical decisions on diagnosis, treatment and the need for intensive care unit admission.
© 2023 The Authors. European Journal of Neurology published by John Wiley & Sons Ltd on behalf of European Academy of Neurology.
Sejvar JJ, Kohl KS, Gidudu J, Amato A, Bakshi N, Baxter R, Burwen DR, Cornblath DR, Cleerbout J, Edwards KM, Heininger U, Hughes R, Khuri-Bulos N, Korinthenberg R, Law BJ, Munro U, Maltezou HC, Nell P, Oleske J, Sparks R, Velentgas P, Vermeer P, Wiznitzer M; Brighton Collaboration GBS Working Group.
Guillain-Barré syndrome and Fisher syndrome: case definitions and guidelines for collection, analysis, and presentation of immunization safety data.
Vaccine. 2011 Jan 10;29(3):599-612. doi: 10.1016/j.vaccine.2010.06.003. Epub 2010 Jun 18.
Abstract/Text
Fokke C, van den Berg B, Drenthen J, Walgaard C, van Doorn PA, Jacobs BC.
Diagnosis of Guillain-Barré syndrome and validation of Brighton criteria.
Brain. 2014 Jan;137(Pt 1):33-43. doi: 10.1093/brain/awt285. Epub 2013 Oct 26.
Abstract/Text
Guillain-Barré syndrome is an acute polyradiculoneuropathy with a variable clinical presentation. Accurate diagnostic criteria are essential for patient care and research, including clinical trials and vaccine safety studies. Several diagnostic criteria for Guillain-Barré syndrome have been proposed, including the recent set by the Brighton Collaboration. In the present study we describe in detail the key diagnostic features required to meet these Brighton criteria in a study population of 494 adult patients with Guillain-Barré syndrome, previously included in therapeutic and observational studies. The patients had a median age of 53 years (interquartile range 36-66 years) and males slightly predominated (56%). All patients developed bilateral limb weakness which generally involved both upper and lower extremities. The weakness remained restricted to the legs in 6% and to the arms in 1% of the patients. Decreased reflexes in paretic arms or legs were found initially in 91% of patients and in all patients during follow-up. Ten (2%) patients however showed persistence of normal reflexes in paretic arms. Disease nadir was reached within 2 weeks in 80%, within 4 weeks in 97% and within 6 weeks in all patients. A monophasic disease course occurred in 95% of patients, of whom 10% had a treatment-related fluctuation. A clinical deterioration after 8 weeks of onset of weakness occurred in 23 (5%) patients. Cerebrospinal fluid was examined in 474 (96%) patients. A mild pleocytosis (5 to 50 cells/μl) was found in 15%, and none had more than 50 cells/μl. An increased cerebrospinal fluid protein concentration was found only in 64% of patients, highly dependent on the timing of the lumbar puncture after onset of weakness (49% at the first day to 88% after 2 weeks). Nerve electrophysiology was compatible with the presence of a neuropathy in 99% of patients, but only 59% fulfilled the current criteria for a distinct subtype of Guillain-Barré syndrome. Patients with a complete data set (335) were classified according to the Brighton criteria, ranging from a high to a low level of diagnostic certainty, as level 1 in 61%, level 2 in 33%, level 3 in none, and level 4 in 6% of patients. Patients categorized in these levels did not differ with respect to proportion of patients with preceding events, initial clinical manifestations or outcome. The observed variability in the key diagnostic features of Guillain-Barré syndrome in the current cohort study, can be used to improve the sensitivity of the diagnostic criteria.
Kuitwaard K, de Gelder J, Tio-Gillen AP, Hop WC, van Gelder T, van Toorenenbergen AW, van Doorn PA, Jacobs BC.
Pharmacokinetics of intravenous immunoglobulin and outcome in Guillain-Barré syndrome.
Ann Neurol. 2009 Nov;66(5):597-603. doi: 10.1002/ana.21737.
Abstract/Text
OBJECTIVE: Intravenous immunoglobulin (IVIg) is the first choice treatment for Guillain-Barré syndrome (GBS). All patients initially receive the same arbitrary dose of 2g per kg body weight. Not all patients, however, show a good recovery after this standard dose. IVIg clearance may depend on disease severity and vary between individuals, implying that this dose is suboptimal for some patients. In this study, we determined whether the pharmacokinetics of IVIg is related to outcome in GBS.
METHODS: We included 174 GBS patients who had previously participated in 2 randomized clinical trials. At entry, all patients were unable to walk unaided and received a standard dose of IVIg. Total IgG levels in serum samples obtained immediately before and 2 weeks after the start of IVIg administration were determined by turbidimetry and related to clinical outcome at 6 months.
RESULTS: The increase in serum IgG (DeltaIgG) 2 weeks after IVIg treatment varied considerably between patients (mean, 7.8g/L; standard deviation, 5.6g/L). Patients with a low DeltaIgG recovered significantly more slowly, and fewer reached the ability to walk unaided at 6 months (log-rank p < 0.001). In multivariate analysis adjusted for other known prognostic factors, a low DeltaIgG was independently associated with poor outcome (p = 0.022).
INTERPRETATION: After a standard dose of IVIg treatment, GBS patients show a large variation in pharmacokinetics, which is related to clinical outcome. This may indicate that patients with a small increase in serum IgG level may benefit from a higher dosage or second course of IVIg.
Fokkink WR, Walgaard C, Kuitwaard K, Tio-Gillen AP, van Doorn PA, Jacobs BC.
Association of Albumin Levels With Outcome in Intravenous Immunoglobulin-Treated Guillain-Barré Syndrome.
JAMA Neurol. 2017 Feb 1;74(2):189-196. doi: 10.1001/jamaneurol.2016.4480.
Abstract/Text
IMPORTANCE: There is an urgent need for biomarkers to monitor treatment efficacy and anticipate outcome in patients with Guillain-Barré syndrome (GBS).
OBJECTIVE: To assess whether there is an association between serum albumin levels, a widely used and relatively easily measurable biomarker of health and inflammation, and the clinical course and outcome of GBS in patients treated with intravenous immunoglobulin (IVIG).
DESIGN, SETTING, AND PARTICIPANTS: We used serum samples derived from a cohort of patients with GBS admitted to hospitals across the Netherlands participating in national GBS studies from May 5, 1986, through August 2, 2000. Serum albumin was measured from January 13 to 20, 2011. Analysis was performed from February 25, 2013, to September 6, 2016. All patients fulfilled the criteria for GBS and had severe disease (defined as not being able to walk unaided >10 m). Patients misdiagnosed as having GBS were retrospectively excluded from the study. Serum samples were obtained before and after IVIG treatment at 4 standardized time points from 174 patients. Albumin levels were determined by routine diagnostic turbidimetry and related to demographics and clinical course during a follow-up of 6 months.
MAIN OUTCOMES AND MEASURES: Serum albumin concentration was determined before and after treatment with IVIG and related to clinical outcome: muscle weakness (measured by Medical Research Council sum score), respiratory failure (measured by requirement and duration of mechanical ventilation), and ability to walk (measured by GBS disability score).
RESULTS: Serum albumin levels were determined in 174 patients with GBS (mean [SD] age, 49.6 [20.1] years; 99 males [56.9%]). Before treatment, the median serum albumin level was 4.2 g/dL (interquartile range, 3.8-4.5 g/dL), with hypoalbuminemia (albumin, <3.5 g/dL) in 20 (12.8%) of 156 patients. Two weeks after commencing treatment with IVIG (2 g/kg), the median serum albumin level decreased to 3.7 g/dL (interquartile range, 3.2-4.1 g/dL) (P < .001), and the number with hypoalbuminemia increased to 60 (34.5%) of 174 (P < .001). Hypoalbuminemia was associated with an increased chance of respiratory failure before (16 [36.4%] of 44, P = .001) or after (29 [54.7%] of 53, P < .001) IVIG treatment, inability to walk unaided (21 [35.0%] of 60 vs 6 [5.3%] of 114, P < .001), and severe muscle weakness at 4 weeks (Medical Research Council sum score, 31.8 vs 52.9, P < .001) and 6 months (Medical Research Council sum score, 49.4 vs 58.4, P < .001).
CONCLUSIONS AND RELEVANCE: Patients with GBS may develop hypoalbuminemia after treatment with IVIG, which is related to a more severe clinical course and a poorer outcome. Further studies are required to confirm that serum albumin can be used as a biomarker to monitor disease activity and treatment response to IVIG in patients with GBS.
Oji S, et al. Pretreatment hypo albuminemia is associated with severity and short-term outcome of in patients with Guillain–Barré syndrome. Peripheral Nerve. 2019;30:90-96.
Walgaard C, Jacobs BC, Lingsma HF, Steyerberg EW, Cornblath DR, van Doorn PA; Dutch GBS Study Group.
Second IVIg course in Guillain-Barré syndrome patients with poor prognosis (SID-GBS trial): Protocol for a double-blind randomized, placebo-controlled clinical trial.
J Peripher Nerv Syst. 2018 Dec;23(4):210-215. doi: 10.1111/jns.12286. Epub 2018 Sep 24.
Abstract/Text
One course of intravenous immunoglobulins (IVIg) of 2 g/kg is standard treatment in Guillain-Barré syndrome (GBS) patients unable to walk independently. Despite treatment some patients recover poorly, in part related to rapid consumption of IVIg, indicating that they may benefit from a second course of IVIg. The aim of the study is to determine whether a second course of IVIg, administered 1 week after start of the first course in patients with GBS and predicted poor outcome improves functional outcome on the GBS disability scale after 4 weeks. Secondary outcome measures include adverse events (AEs), Medical Research Council sumscore and GBS disability score after 8, 12, and 26 weeks, length of hospital and ICU admission, mortality, and changes in serum IgG levels. GBS patients of 12 years and older with a poor prognosis, based on the modified Erasmus GBS outcome score (mEGOS) at 1 week after start of the first IVIg course are eligible for randomization in this double-blind, placebo-controlled (IVIg or albumin) clinical trial. This study will determine if a second course of IVIg administered in the acute phase of the disease is safe, feasible, and effective in patients with GBS and a poor prognosis. This Dutch trial is registered prospectively as NTR 2224 in the Netherlands National Trial Register (NTR) which is the Primary Registry in the WHO Registry Network for the Netherlands.
© 2018 Peripheral Nerve Society.
Hadden RD, Cornblath DR, Hughes RA, Zielasek J, Hartung HP, Toyka KV, Swan AV.
Electrophysiological classification of Guillain-Barré syndrome: clinical associations and outcome. Plasma Exchange/Sandoglobulin Guillain-Barré Syndrome Trial Group.
Ann Neurol. 1998 Nov;44(5):780-8. doi: 10.1002/ana.410440512.
Abstract/Text
We performed electrophysiological and serological testing within 15 days of symptom onset on 369 patients with Guillain-Barré Syndrome (GBS) enrolled in a trial comparing plasma exchange, intravenous immunoglobulin, and both treatments. Patients were classified into five groups by motor nerve conduction criteria; 69% were demyelinating, 3% axonal, 3% inexcitable, 2% normal, and 23% equivocal. Six of 10 (60%) patients with axonal neurophysiology had had a preceding diarrheal illness compared with 71 of 359 (20%) in other groups. Antiganglioside GM1 antibodies were present in a higher proportion of patients with axonal physiology or inexcitable nerves than other patients. The number dead or unable to walk unaided at 48 weeks was greater in the group with initially inexcitable nerves (6 of 12, 50%) compared with the rest (52 of 357, 15%), but was not significantly different between the axonal (1 of 10, 10%) and demyelinating (44 of 254, 17%) groups. Sensory action potentials and clinical sensory examination were both normal in 53 of 342 (16%) patients, and these "pure motor GBS" patients were more likely than other GBS patients to have IgG antiganglioside GM1 antibodies and to have had preceding diarrhea but had a similar outcome. The axonal group was more likely than other groups to have normal sensory action potentials. The outcomes in response to the three treatments did not differ in any subgroup (including patients with pure motor GBS or preceding diarrhea) or any neurophysiological category.
Ho TW, Mishu B, Li CY, Gao CY, Cornblath DR, Griffin JW, Asbury AK, Blaser MJ, McKhann GM.
Guillain-Barré syndrome in northern China. Relationship to Campylobacter jejuni infection and anti-glycolipid antibodies.
Brain. 1995 Jun;118 ( Pt 3):597-605. doi: 10.1093/brain/118.3.597.
Abstract/Text
Guillain-Barré syndrome has been considered to be primarily an acute inflammatory demyelinating polyneuropathy (AIDP). Our experience with Guillain-Barré syndrome in northern China differs from the traditional concept. Electrophysiologically and pathologically, most of our patients have motor axonal degeneration with minimal cellular inflammation, which we have termed 'acute motor axonal neuropathy' (AMAN). The current studies were undertaken to characterize prospectively the clinical, electrophysiological, and serological features of Guillain-Barré syndrome, defined clinically, in northern China. In 1991 and 1992, we characterized by electrodiagnostic criteria 129 Chinese patients with Guillain-Barré syndrome. The AMAN form was present in 65% of patients, the AIDP form in 24% and 11% were unclassifiable. For the 38 patients who presented from January to October, 1992, we performed serological assays for antibodies to Campylobacter jejuni and to glycolipids. Of these 38 patients, 55% had AMAN, 32% had AIDP and 13% were unclassifiable. Sixty-six percent of the 38 had serological evidence of recent C. jejuni infection as compared with 16% of village controls (P = 0.001). Seventy-six percent of AMAN patients and 42% of AIDP patients were seropositive. IgG anti-GM1 antibodies were more frequent in Guillain-Barré syndrome patients compared with village controls (42% versus 6%; P < 0.01). However, no statistically significant correlations were found between the pattern of disease, AMAN or AIDP, anti-glycolipid antibodies, or C. jejuni antibodies. Based on electrophysiological criteria, Guillain-Barré syndrome in northern China can be divided into two predominant forms: AIDP and AMAN. The AMAN form is more common and predominates in the yearly summer outbreaks of Guillain-Barré syndrome.(ABSTRACT TRUNCATED AT 250 WORDS)
Patwa HS, Chaudhry V, Katzberg H, Rae-Grant AD, So YT.
Evidence-based guideline: intravenous immunoglobulin in the treatment of neuromuscular disorders: report of the Therapeutics and Technology Assessment Subcommittee of the American Academy of Neurology.
Neurology. 2012 Mar 27;78(13):1009-15. doi: 10.1212/WNL.0b013e31824de293.
Abstract/Text
OBJECTIVE: To assess the evidence for the efficacy of IV immunoglobulin (IVIg) to treat neuromuscular disorders.
METHODS: The MEDLINE, Web of Science, and EMBASE databases were searched (1966-2009). Selected articles were rated according to the American Academy of Neurology's therapeutic classification of evidence scheme; recommendations were based on the evidence level.
RESULTS AND RECOMMENDATIONS: IVIg is as efficacious as plasmapheresis and should be offered for treating Guillain-Barré syndrome (GBS) in adults (Level A). IVIg is effective and should be offered in the long-term treatment of chronic inflammatory demyelinating polyneuropathy (Level A). IVIg is probably effective and should be considered for treating moderate to severe myasthenia gravis and multifocal motor neuropathy (Level B). IVIg is possibly effective and may be considered for treating nonresponsive dermatomyositis in adults and Lambert-Eaton myasthenic syndrome (Level C). Evidence is insufficient to support or refute use of IVIg in the treatment of immunoglobulin M paraprotein-associated neuropathy, inclusion body myositis, polymyositis, diabetic radiculoplexoneuropathy, or Miller Fisher syndrome, or in the routine treatment of postpolio syndrome or in children with GBS (Level U). IVIg combined with plasmapheresis should not be considered for treating GBS (Level B). More data are needed regarding IVIg efficacy as compared with other treatments/treatment combinations. Most studies concluded IVIg-related serious adverse effects were rare. Given the variable nature of these diseases, individualized treatments depending on patient need and physician judgment are important.
Elovaara I, Apostolski S, van Doorn P, Gilhus NE, Hietaharju A, Honkaniemi J, van Schaik IN, Scolding N, Soelberg Sørensen P, Udd B; EFNS.
EFNS guidelines for the use of intravenous immunoglobulin in treatment of neurological diseases: EFNS task force on the use of intravenous immunoglobulin in treatment of neurological diseases.
Eur J Neurol. 2008 Sep;15(9):893-908. doi: 10.1111/j.1468-1331.2008.02246.x.
Abstract/Text
Despite high-dose intravenous immunoglobulin (IVIG) is widely used in treatment of a number of immune-mediated neurological diseases, the consensus on its optimal use is insufficient. To define the evidence-based optimal use of IVIG in neurology, the recent papers of high relevance were reviewed and consensus recommendations are given according to EFNS guidance regulations. The efficacy of IVIG has been proven in Guillain-Barré syndrome (level A), chronic inflammatory demyelinating polyradiculoneuropathy (level A), multifocal mononeuropathy (level A), acute exacerbations of myasthenia gravis (MG) and short-term treatment of severe MG (level A recommendation), and some paraneoplastic neuropathies (level B). IVIG is recommended as a second-line treatment in combination with prednisone in dermatomyositis (level B) and treatment option in polymyositis (level C). IVIG should be considered as a second or third-line therapy in relapsing-remitting multiple sclerosis, if conventional immunomodulatory therapies are not tolerated (level B), and in relapses during pregnancy or post-partum period (good clinical practice point). IVIG seems to have a favourable effect also in paraneoplastic neurological diseases (good practice point) [corrected],stiff-person syndrome (level A), some acute-demyelinating diseases and childhood refractory epilepsy (good practice point).
van der Meché FG, Schmitz PI.
A randomized trial comparing intravenous immune globulin and plasma exchange in Guillain-Barré syndrome. Dutch Guillain-Barré Study Group.
N Engl J Med. 1992 Apr 23;326(17):1123-9. doi: 10.1056/NEJM199204233261705.
Abstract/Text
BACKGROUND: The subacute demyelinating polyneuropathy known as Guillain-Barré syndrome improves more rapidly with plasma exchange than with supportive care alone. We conducted a multicenter trial to determine whether intravenous immune globulin is as effective as the more complicated treatment with plasma exchange.
METHODS: To enter the study, patients had to have had Guillain-Barré syndrome for less than two weeks and had to be unable to walk independently. They were randomly assigned to receive either five plasma exchanges (each of 200 to 250 ml per kilogram of body weight) or five doses of a preparation of intravenous immune globulin (0.4 g per kilogram per day). The predefined outcome measure was improvement at four weeks by at least one grade on a seven-point scale of motor function.
RESULTS: After 150 patients had been treated, strength had improved by one grade or more in 34 percent of those treated with plasma exchange, as compared with 53 percent of those treated with immune globulin (difference, 19 percent; 95 percent confidence interval, 3 percent to 34 percent; P = 0.024). The median time to improvement by one grade was 41 days with plasma exchange and 27 days with immune globulin therapy (P = 0.05). The immune globulin group had significantly fewer complications and less need for artificial ventilation.
CONCLUSIONS: In the acute Guillain-Barré syndrome, treatment with intravenous immune globulin is at least as effective as plasma exchange and may be superior.
Bril V, Ilse WK, Pearce R, Dhanani A, Sutton D, Kong K.
Pilot trial of immunoglobulin versus plasma exchange in patients with Guillain-Barré syndrome.
Neurology. 1996 Jan;46(1):100-3. doi: 10.1212/wnl.46.1.100.
Abstract/Text
We compared intravenous immunoglobulin (IVIG) and plasma exchange (PLEX) in the treatment of 50 patients with Guillain-Barré syndrome (GBS). Standard outcome measures did not differ for the two groups. Sixty-one percent of the PLEX-treated group and 69% of the IVIG-treated group improved by one disability grade at 1 month. The complication rate was higher in the PLEX-treated group. We conclude that the efficacy of IVIG in the treatment of GBS is comparable with that of PLEX and that it can be used safely, although we had a small number of patients. We did not observe a higher relapse rate with IVIG. The usefulness of combination therapy is unknown at this time.
.
Randomised trial of plasma exchange, intravenous immunoglobulin, and combined treatments in Guillain-Barré syndrome. Plasma Exchange/Sandoglobulin Guillain-Barré Syndrome Trial Group.
Lancet. 1997 Jan 25;349(9047):225-30.
Abstract/Text
BACKGROUND: The relative efficacy of plasma exchange (PE) and intravenous immunoglobulin (IVIg) for the treatment of Guillain-Barré syndrome has not been established. We compared PE with IVIg, and with a combined regimen of PE followed by IVIg, in an international, multicentre, randomised trial of 383 adult patients with Guillain-Barré syndrome.
METHODS: The patients were randomly assigned PE (five 50 mL/kg exchanges over 8-13 days), IVIg (Sandoglobulin, 0.4 g/kg daily for 5 days), or the PE course immediately followed by the IVIg course. The inclusion criteria were severe disease (aid needed for walking) and onset of neuropathic symptoms within the previous 14 days. Patients were followed up for 48 weeks.
FINDINGS: Four patients were excluded because they did not meet the randomisation criteria. All the remaining 379 patients were assessed for the major outcome criterion-change on a seven-point disability grade scale-by an observer unaware of treatment assignment, 4 weeks after randomisation. At that time, the mean improvement was 0.9 (SD 1.3) in the 121 PE-group patients, 0.8 (1.3) in the 130 IVIg-group patients, and 1.1 (1.4) in the 128 patients who received both treatments (intention-to-treat analysis). None of the differences between the groups for this major outcome criterion was significant. The difference between PE alone and IVIg alone was so small that a 0.5 grade difference was excluded at the 95% level of confidence. There was no significant difference between any of the treatment groups in the secondary outcome measures: time to recovery of unaided walking, time to discontinuation of ventilation, and trend describing the recovery from disability up to 48 weeks. There was a non-significant trend towards a more favourable outcome on some outcome measures with combined treatment.
INTERPRETATION: In treatment of severe Guillain-Barré syndrome during the first 2 weeks after onset of neuropathic symptoms, PE and IVIg had equivalent efficacy. The combination of PE with IVIg did not confer a significant advantage.
野村恭一ほか:Guillain-Barre症候群に対する免疫グロブリン療法と血漿交換療法とのランダム割り付け比較試験. 神経治療 2001; 18: 69-81.
Färkkilä M, Kinnunen E, Haapanen E, Iivanainen M.
Guillain-Barré syndrome: quantitative measurement of plasma exchange therapy.
Neurology. 1987 May;37(5):837-40. doi: 10.1212/wnl.37.5.837.
Abstract/Text
A prospective, controlled study with quantitative measurement of hand muscle-force for plasma exchange treatment in acute Guillain-Barré polyradiculitis was done. Of the 29 patients with severe symptoms, every second patient was selected to the plasma exchange group and all others to the control group. The muscle forces increased and CSF protein decreased significantly more in the plasma exchange group than in the control group, but there were no differences in hospitalization or recovery periods.
.
Plasmapheresis and acute Guillain-Barré syndrome. The Guillain-Barré syndrome Study Group.
Neurology. 1985 Aug;35(8):1096-104.
Abstract/Text
We compared plasmapheresis with conventional therapy in 245 patients with the Guillain-Barré syndrome of recent onset. Statistically significant differences, favoring the plasmapheresis group, were found in terms of improvement at 4 weeks, time to improve one clinical grade, time to independent walking, and outcome at 6 months. Plasmapheresis was not effective for all patients, but was particularly effective for patients who received this treatment within 7 days of onset and for patients who required mechanical ventilation after entry into the study. Plasmapheresis appears to be of benefit in patients with Guillain-Barré syndrome of recent onset.
.
Efficiency of plasma exchange in Guillain-Barré syndrome: role of replacement fluids. French Cooperative Group on Plasma Exchange in Guillain-Barré syndrome.
Ann Neurol. 1987 Dec;22(6):753-61. doi: 10.1002/ana.410220612.
Abstract/Text
The goals of this multicenter controlled trial were: (1) to study the short-term effect of plasma exchange in the Guillain-Barré syndrome when applied alone within 17 days of onset of the disease; and (2) to compare two replacement fluids, diluted albumin and fresh frozen plasma (FFP) with regard to efficacy and morbidity. Two hundred twenty patients were included, 111 in the control group, 109 in the plasma exchange group, 57 of whom were assigned to receive albumin and 52 to receive fresh frozen plasma. Treatment consisted of four plasma exchanges of two plasma volumes each, initiated on the day of randomization and repeated on alternate days. Significant short-term benefits appeared in the group that received plasma exchange as demonstrated by the reduction in the proportion of patients who required assisted ventilation after randomization, and the decrease in time before beginning weaning from ventilator, time to onset of motor recovery, and time to walk with and without assistance. No statistically significant difference was found between the group that received albumin and the group that received fresh frozen plasma. Incidents during exchanges and complications related to the plasma exchange were more frequent in patients who received fresh frozen plasma. Plasma exchange is beneficial in Guillain-Barré syndrome when it is carried out early in the course of the disease. Given its risks and the lack of its clear superiority over albumin, however, we recommend that fresh frozen plasma be abandoned in the treatment of Guillain-Barré syndrome.
.
Appropriate number of plasma exchanges in Guillain-Barré syndrome. The French Cooperative Group on Plasma Exchange in Guillain-Barré Syndrome.
Ann Neurol. 1997 Mar;41(3):298-306. doi: 10.1002/ana.410410304.
Abstract/Text
Plasma exchange (PE) is the standard treatment in Guillain-Barré syndrome (GBS) patients who have lost the ability to walk. The effect of exchanges before this stage and the optimal number of exchanges for the other patients are still unknown. We randomized 556 GBS patients according to severity and number of exchanges as follows: Zero versus 2 PEs for patients who could walk-with or without aid-but not run, or who could stand up unaided (mild group); 2 versus 4 PEs for patients who could not stand up unaided (moderate group); and 4 versus 6 PEs for mechanically ventilated patients (severe group). In the mild group, 2 PEs were more effective than none for time to onset of motor recovery (median, 4 vs 8 days, respectively). In the moderate group, 4 PEs were more beneficial than 2 for time to walk with assistance (median, 20 vs 24 days) and for 1-year full muscle-strength recovery rate (64% vs 46%). Six PEs were no more beneficial than 4 in the severe cases. Patients with mild GBS on admission should receive 2 PEs. Patients with moderate and severe forms should benefit from 2 further exchanges.
Diener HC, Haupt WF, Kloss TM, Rosenow F, Philipp T, Koeppen S, Vietorisz A; Study Group.
A preliminary, randomized, multicenter study comparing intravenous immunoglobulin, plasma exchange, and immune adsorption in Guillain-Barré syndrome.
Eur Neurol. 2001;46(2):107-9. doi: 10.1159/000050777.
Abstract/Text
Haupt WF, Birkmann C, van der Ven C, Pawlik G.
Apheresis and selective adsorption plus immunoglobulin treatment in Guillain-Barré syndrome.
Ther Apher. 2000 Jun;4(3):198-200. doi: 10.1046/j.1526-0968.2000.00182.x.
Abstract/Text
Plasma exchange (PE) and administration of intravenous immunoglobulin (i.v. IgG) are established treatments for Guillain-Barré syndrome (GBS). Combination treatment with elimination of postulated pathogenetic factors by PE or selective adsorption (SA) treatment and subsequent immunomodulation by intravenous IgG may provide a more effective treatment. In a single-center randomized study, 45 patients with acute GBS were prospectively examined using a clinical score. We treated 11 patients by PE, 13 with SA using a tryptophan-linked polyvinyl alcohol gel adsorbent, and 21 with SA followed by intravenous IgG. The patients treated sequentially by selective adsorption and intravenous IgG improved significantly better than the patients who received plasma treatment only. The results suggest that combination treatment of GBS may be superior to plasma treatment alone.
Kuwabara S, Nakajima M, Moroo I, Hirayama K.
[Double filtration and immunoadsorption plasmapheresis in Guillain-Barré syndrome].
Rinsho Shinkeigaku. 1996 Feb;36(2):289-92.
Abstract/Text
We studied the effect of double filtration and immunoadsorption plasmapheresis in Guillain-Barré syndrome retrospectively in 70 patients. Thirty three patients had plasmapheresis, of whom 23 had double filtration and 10 immunoadsorption. The rest of 37 patients consisted of a control group. Clinical disability was evaluated using the functional grading scale by Hughes. In patients having a maximum disability of grade 4 and 5, the double filtration plasmapheresis group showed statistically significant improvement at 3 months after the treatment. There was no significant difference in prognoses between the immunoadsorption plasmapheresis and control groups. In mildly-illed patients having grade 2 and 3, the prognoses were not different between the three groups. In Guillain-Barré syndrome, double filtration plasmapheresis, as well as well-established plasma exchange, had benefit for severely-illed patients.
Araki T, Nakata H, Kusunoki S, Arai Y, Katayama Y.
[Immunoadsorption therapy with TR-350 (tryptophan column) for Guillain-Barré syndrome: investigation including serum antiganglioside antibody assay].
Rinsho Shinkeigaku. 2000 Oct;40(10):979-85.
Abstract/Text
Immunoadsorption therapy (IAT) using TR-350 was performed for 14 patients with Guillain-Barré syndrome (GBS). Presence of serum antiganglioside antibodies (AGA) was investigated in all the patients in the acute phase. In 14 patients studied, 6 men and 8 women, ages from 24 to 74 years(mean, 42.5 years), 7 patients had suffered from common cold and 3 from diarrhea before neurological onset. Ophthalmoplegia was seen in 6 patients, facial palsy in 6, bulbar palsy in 3 and cerebellar sign in 2. Functional grade scores (FGS) by Hughes et al. of the patients were from 5 to 1 (mean, 3.3). In 4 patients, whose FGS were 1 or 2, IAT were performed, because of worsting of bulbar palsy, bilateral facial palsy and limb weakness. Relapse occurred in one patient. IAT was started from 2 to 18 days (mean, 8.4) after neurological onset and performed 3 to 14 times (mean, 7.5) for each patient. Mean FGS improved from 3.3 to 2.1 after IAT. The mean time to improve 1 grade was 10.3 days and mean time to improve 2 grades was 39.0 days. The mean FGS after 1 month was 1.4 and that after 3 months was 0.4. Some of 14 patients had elevated titers of AGA in sera in the acute phase. Four patients had anti-GQ1b IgG antibody and showed external ophthalmoplegia. One patient with anti-GD1b IgG antibody had cerebellar signs as well as peripheral neuropathy. Those AGAs decreased after IAT in parallel with improvement. IAT is an effective treatment in acute phase of GBS.
Hughes RA, Swan AV, van Doorn PA.
Corticosteroids for Guillain-Barré syndrome.
Cochrane Database Syst Rev. 2010 Feb 17;(2):CD001446. doi: 10.1002/14651858.CD001446.pub3. Epub 2010 Feb 17.
Abstract/Text
BACKGROUND: Guillain-Barré syndrome is caused by inflammation of the peripheral nerves, which corticosteroids should benefit.
OBJECTIVES: To examine the efficacy of corticosteroids.
SEARCH STRATEGY: We searched The Cochrane Neuromuscular Disease Group Trials Specialized Register (June 2009), MEDLINE (January 1966 to June 2009) and EMBASE from (January 1980 to June 2009).
SELECTION CRITERIA: We included quasi-randomised or randomised controlled trials of any form of corticosteroid or adrenocorticotrophic hormone. Our primary outcome was change in disability grade on a seven-point scale after four weeks. Secondary outcomes included time from randomisation until recovery of unaided walking, time from randomisation until discontinuation of ventilation (for those ventilated), death, death or disability (inability to walk without aid) after 12 months, relapse, and adverse events.
DATA COLLECTION AND ANALYSIS: Two authors extracted the data.
MAIN RESULTS: No new trials were discovered in the new search in June 2009. Six trials with 587 participants provided data for the primary outcome. According to moderate quality evidence, the disability grade change after four weeks in the corticosteroid groups was not significantly different from that in the control groups, weighted mean difference (WMD) 0.36 less improvement (95% confidence intervals (CI) 0.16 more to 0.88 less improvement). In four trials of oral corticosteroids with 120 participants in total, there was significantly less improvement after four weeks with corticosteroids than without corticosteroids, WMD 0.82 disability grades less improvement, 95% CI 0.17 to 1.47). In two trials with a combined total of 467 participants, there was no significant difference, WMD 0.17 (95% CI -0.06 to 0.39) of a disability grade more improvement after four weeks with intravenous corticosteroids. According to moderate to high quality evidence, there were no significant differences between the corticosteroid-treated and the control groups in any of the secondary efficacy outcomes. Diabetes was significantly more common and hypertension significantly much less common in the corticosteroid-treated participants.
AUTHORS' CONCLUSIONS: According to moderate quality evidence, corticosteroids given alone do not significantly hasten recovery from GBS or affect the long-term outcome. According to low quality evidence oral corticosteroids delay recovery. Diabetes requiring insulin was significantly more and hypertension less common with corticosteroids.
Singh NK, Gupta A.
Do corticosteroids influence the disease course or mortality in Guillain-Barre' syndrome?
J Assoc Physicians India. 1996 Jan;44(1):22-4.
Abstract/Text
Forty-six patients of Guillain-Barre' Syndrome were randomized to receive either prednisolone (40 mg daily for 2 weeks and then tapered off) or placebo. The patients were followed up for 6 months and were assessed on an objective scale of disability. The improvement in mean disability grade was significantly better at 2 weeks and 4 weeks in the placebo group as compared to those who received corticosteroids. The difference persisted at 24 weeks, but was statistically insignificant. A greater proportion of patients in the placebo group had improved by at least 1 disability grade at all points of time. The group of patients treated with steroids took twice as long to improve by 1 disability grade as compared to those in the placebo group. At 6 months, 41.7% of the patients in the steroid group had recovered almost completely (good outcome) as compared to 54.5% of the patients is the placebo group. Corticosteroids, therefore, do not appear to benefit GBS patients, and may in fact, delay the recovery from acute illness.
.
Double-blind trial of intravenous methylprednisolone in Guillain-Barré syndrome. Guillain-Barré Syndrome Steroid Trial Group.
Lancet. 1993 Mar 6;341(8845):586-90.
Abstract/Text
Steroids have been beneficial in the treatment of demyelinating diseases with features similar to those of Guillain-Barré syndrome (GBS). However, steroid treatment of GBS has been disappointing; in an earlier trial oral prednisolone was ineffective, although the dose was low and the sample small. We assessed the benefit of a high-dose steroid regimen in a large sample of patients with GBS in a multicentre, randomised, double-blind trial. 242 adult patients were randomised to receive intravenous methylprednisolone (IVMP) 500 mg (124 patients) or a placebo (118) daily for 5 days. Patients were diagnosed by standard clinical criteria and entered the trial within 15 days of onset of neurological symptoms. All patients were too weak to run. Some patients received plasma exchange depending on the practice of their centre. Disability was graded on a scale from 0 (healthy) to 6 (dead) at intervals for 48 weeks. There was no significant difference in any outcome variable between patients treated with IVMP and those given placebo. The most important outcome was the difference between the groups in disability grade 4 weeks after randomisation, which was only a 0.06 grade (95% Cl -0.23 to 0.36) greater improvement in the IVMP than the placebo group. The 39 patients in the IVMP group who required ventilation did so for a median of 18 days, 9 days fewer than the 44 patients who had a placebo and required ventilation (95% Cl -9.6 to 27.6). Median time to walk unaided was 38 days in the IVMP patients and 50 days in the placebo patients (difference 12 days, (95% Cl -21.3 to 45.3). A short course of high-dose IVMP given early in GBS is ineffective.
van Koningsveld R, Schmitz PI, Meché FG, Visser LH, Meulstee J, van Doorn PA; Dutch GBS study group.
Effect of methylprednisolone when added to standard treatment with intravenous immunoglobulin for Guillain-Barré syndrome: randomised trial.
Lancet. 2004 Jan 17;363(9404):192-6. doi: 10.1016/s0140-6736(03)15324-x.
Abstract/Text
BACKGROUND: Despite available treatment with intravenous immunoglobulin (IVIg), morbidity and mortality are considerable in patients with Guillain-Barré syndrome (GBS). Our aim was to assess whether methylprednisolone, when taken with IVIg, improves outcome when compared with IVIg alone.
METHODS: We did a double-blind, placebo-controlled, multicentre, randomised study, to which we enrolled patients who were unable to walk independently and who had been treated within 14 days after onset of weakness with IVIg (0.4 g/kg bodyweight per day) for 5 days. We assigned 233 individuals to receive either intravenous methylprednisolone (500 mg per day; n=116) or placebo (n=117) for 5 days within 48 h of administration of first dose of IVIg. Because age is an important prognostic factor, we split treatment groups into two age-groups-ie, younger than age 50 years, or 50 years and older. Our primary outcome was an improvement from baseline in GBS disability score of one or more grades 4 weeks after randomisation. Analysis was by intention to treat.
FINDINGS: We analysed 225 patients. GBS disability scores increased by one grade or more in 68% (76 of 112) of patients in the methylprednisolone group and in 56% (63 of 113) of controls (odds ratio [OR] 1.68, 95% CI 0.97-2.88; p=0.06). After adjustment for age and degree of disability at entry, treatment OR was 1.89 (95% CI 1.07-3.35; p=0.03). Side-effects did not differ greatly between groups.
INTERPRETATION: We noted no significant difference between treatment with methylprednisolone and IVIg and IVIg alone. Because of the relevance of prognostic factors and the limited side-effects of methylprednisolone, the potential importance of combination treatment with the drug and IVIg, however, warrants further investigation.
Pandey CK, Bose N, Garg G, Singh N, Baronia A, Agarwal A, Singh PK, Singh U.
Gabapentin for the treatment of pain in guillain-barré syndrome: a double-blinded, placebo-controlled, crossover study.
Anesth Analg. 2002 Dec;95(6):1719-23, table of contents. doi: 10.1097/00000539-200212000-00046.
Abstract/Text
UNLABELLED: Pain syndromes of Guillain-Barré are neuropathic as well as nociceptive in origin. We aimed to evaluate the therapeutic efficacy of gabapentin in relieving the bimodal nature of pain in Guillain-Barré syndrome in a randomized, double-blinded, placebo-controlled, crossover study in 18 patients admitted to the intensive care unit for ventilatory support. Patients were assigned to receive either gabapentin (15 mg. kg(-1). d(-1) in 3 divided doses) or matching placebo as initial medication for 7 days. After a 2-day washout period, those who previously received gabapentin received placebo, and those previously receiving placebo received gabapentin as in the initial phase. Fentanyl 2 micro g/kg was used as a rescue analgesic on patient demand or when the pain score was >5 on a numeric rating scale of 0-10. The numeric rating score, sedation score, consumption of fentanyl, and adverse effects were noted, and these observed variables were compared. The numeric pain score decreased from 7.22 +/- 0.83 to 2.33 +/- 1.67 on the second day after initiation of gabapentin therapy and remained low during the period of gabapentin therapy (2.06 +/- 0.63) (P < 0.001). There was a significant decrease in the need for fentanyl from Day 1 to Day 7 during the gabapentin therapy period (211.11 +/- 21.39 to 65.53 +/- 16.17 [ micro g]) in comparison to the placebo therapy period (319.44 +/- 25.08 to 316.67 +/- 24.25 [ micro g]) (P < 0.001).
IMPLICATIONS: Gabapentin, an antiepileptic drug, has been used effectively for different types of pain management. This study demonstrates that gabapentin has minimal side effects and is an alternative to opioids and nonsteroidal antiinflammatory drugs for management of the bimodal nature of pain of Guillain-Barré Syndrome patients.
Tripathi M, Kaushik S.
Carbamezapine for pain management in Guillain-Barré syndrome patients in the intensive care unit.
Crit Care Med. 2000 Mar;28(3):655-8. doi: 10.1097/00003246-200003000-00009.
Abstract/Text
OBJECTIVE: To evaluate carbamezapine (CBZ) for neuritic pain relief in Guillain-Barré syndrome (GBS) patients in the intensive care unit (ICU).
DESIGN: Prospective, double-blind, randomly allocated cross-over study days.
SETTING: ICU in a tertiary care university hospital.
PARTICIPANTS: Twelve consecutive, conscious adult (22-54 yrs) patients with GBS during recovery from the muscular weakness and receiving pressure-support ventilation in the ICU. All patients complained of severe backache and/or leg cramps and tenderness in muscles, and they required opioids for pain relief.
INTERVENTIONS: CBZ (100 mg every 8 hrs) or equivalent placebo was given to nursing staff in coded powder form. Medication was given to patients through a nasogastric feeding tube. The same coded medicine was given for 3 days, and after a 1-day omission, a second set of coded powder was given for the next 3 days in a randomized, double-blind, crossover fashion. Pethidine (1 mg x kg(-1)) was given intravenously in between, if the pain score was >2. Group 1 (n = 6) patients were given a placebo on the first 3 days, followed by CBZ. Group 2 (n = 6) patients were given CBZ on the first 3 days, followed by a placebo.
MEASUREMENTS AND MAIN RESULTS: In these two study periods of different medications, we observed and scored pain (1, no pain; 5, severe pain), sedation (1, alert; 6, asleep, does not respond to verbal command), and total pethidine requirement per day. In group 1 patients, a significant (p < .001) improvement in the sedation score and a low requirement for pethidine was observed 3 days later, when CBZ was started. However, in group 2 patients, a gradual increase in the pethidine requirement and a high sedation score were noteworthy in the later days of placebo medication. Observations were also analyzed for CBZ days vs. placebo days. Overall, the pain score (1.7 +/- 0.8) during the CBZ period of both regimens was significantly (p < .001) lower than during the placebo days (3.1 +/- 0.9). Significantly higher doses of pethidine (3.7 +/- 0.9 mg/kg/day) were used on the placebo days than on the CBZ days (1.7 +/- 1.0 mg/kg/day).
CONCLUSION: The pain in GBS has a dual origin, and we recommend CBZ as an adjuvant to treat pain in GBS patients, during the recovery phase in the ICU, to reduce the narcotic requirement.
Pandey CK, Raza M, Tripathi M, Navkar DV, Kumar A, Singh UK.
The comparative evaluation of gabapentin and carbamazepine for pain management in Guillain-Barré syndrome patients in the intensive care unit.
Anesth Analg. 2005 Jul;101(1):220-5, table of contents. doi: 10.1213/01.ANE.0000152186.89020.36.
Abstract/Text
We evaluated the effects of gabapentin and carbamazepine for pain relief in 36 Guillain-Barré syndrome patients. Patients were randomly assigned to receive gabapentin 300 mg, carbamazepine 100 mg, or matching placebo 3 times a day for 7 days. Fentanyl 2 microg/kg was used as a supplementary analgesic on patient demand. The pain score was recorded by using a numeric pain rating scale of 0-10, and sedation was recorded with a Ramsay sedation scale of 1-6 before medications were given and then at 6-h intervals throughout the study period. Total daily fentanyl consumption was recorded each day for each patient. The results of the study demonstrated that patients in the gabapentin group had significantly lower (P < 0.05) median numeric pain rating scale scores (3.5, 2.5, 2.0, 2.0, 2.0, 2.0, and 2.0) compared with patients in the placebo group (6.0, 6.0, 6.0, 6.0, 6.0, 6.0, and 6.0) and the carbamazepine group (6.0, 6.0, 5.0, 4.0, 4.0, 3.5, and 3.0). There was no significant difference in fentanyl consumption between the gabapentin and carbamazepine groups on Day 1 (340.1 +/- 34.3 microg and 347.5 +/- 38.0 microg, respectively), but consumption was significantly less in these 2 groups compared with the placebo group (590.4 +/- 35.0 microg) (P < 0.05). For the rest of the study period, there was a significant difference in fentanyl consumption among all treatment groups, and it was minimal in the gabapentin group (P < 0.05). We conclude that gabapentin is more effective than carbamazepine for decreasing pain and fentanyl consumption.
Attal N, Cruccu G, Baron R, Haanpää M, Hansson P, Jensen TS, Nurmikko T.
EFNS guidelines on the pharmacological treatment of neuropathic pain: 2010 revision.
Eur J Neurol. 2010 Sep;17(9):1113-e88. doi: 10.1111/j.1468-1331.2010.02999.x. Epub 2010 Apr 9.
Abstract/Text
BACKGROUND AND OBJECTIVES: This second European Federation of Neurological Societies Task Force aimed at updating the existing evidence about the pharmacological treatment of neuropathic pain since 2005.
METHODS: Studies were identified using the Cochrane Database and Medline. Trials were classified according to the aetiological condition. All class I and II randomized controlled trials (RCTs) were assessed; lower class studies were considered only in conditions that had no top-level studies. Treatments administered using repeated or single administrations were considered, provided they are feasible in an outpatient setting.
RESULTS: Most large RCTs included patients with diabetic polyneuropathies and post-herpetic neuralgia, while an increasing number of smaller studies explored other conditions. Drugs generally have similar efficacy in various conditions, except in trigeminal neuralgia, chronic radiculopathy and HIV neuropathy, with level A evidence in support of tricyclic antidepressants (TCA), pregabalin, gabapentin, tramadol and opioids (in various conditions), duloxetine, venlafaxine, topical lidocaine and capsaicin patches (in restricted conditions). Combination therapy appears useful for TCA-gabapentin and gabapentin-opioids (level A).
CONCLUSIONS: There are still too few large-scale comparative studies. For future trials, we recommend to assess comorbidities, quality of life, symptoms and signs with standardized tools and attempt to better define responder profiles to specific drug treatments.
Inokuchi H, Yasunaga H, Nakahara Y, Horiguchi H, Ogata N, Fujitani J, Matsuda S, Fushimi K, Haga N.
Effect of rehabilitation on mortality of patients with Guillain-Barre Syndrome: a propensity-matched analysis using nationwide database.
Eur J Phys Rehabil Med. 2014 Aug;50(4):439-46. Epub 2014 Mar 20.
Abstract/Text
BACKGROUND: Rehabilitation for patients with Guillain-Barre Syndrome (GBS) is recommended as it improves the outcome of neurological deficits. Few studies focused on the effect of rehabilitation on mortality of the patients.
AIM: To investigate the effect of rehabilitation on hospital mortality of patients with GBS using the Japanese Diagnosis Procedure Combination (DPC) nationwide administrative claims database.
DESIGN: A retrospective observational cohort study.
SETTING: Hospitals adopting the Japanese DPC system.
POPULATION: Patients hospitalized with a diagnosis of GBS between July 2007 and October 2011.
METHODS: Data analyzed included sex, age, Barthel index at admission, use of ventilation, immune therapy, and rehabilitation during hospitalization, comorbidity, hospital volume, type of hospital, and in-hospital death. One-to-one propensity score-matching was used to compare hospital mortality rates within 30- and 90-days after admission in rehabilitation and non-rehabilitation groups. The adjusted odds ratios of rehabilitation to hospital mortality were also estimated.
RESULTS: A total of 3835 patients were identified and analyzed. Patients with advancing age, lower Barthel index at admission, comorbidities, ventilation, or immune therapy were more likely to receive rehabilitation during hospitalization. Propensity-matched analysis of 926 pairs showed that the rehabilitation group had lower hospital mortality rates within both 30- and 90-days than the non-rehabilitation group. The adjusted odds ratios of rehabilitation to hospital mortality within 30- and 90-days were 0.14 and 0.23, respectively.
CONCLUSION: After matching patients' background, rehabilitation was associated with lower hospital mortality of patients with GBS.
CLINICAL REHABILITATION IMPACT: Rehabilitation treatment is essential for patients with GBS to improve their survival.
Khan F.
Rehabilitation in Guillian Barre syndrome.
Aust Fam Physician. 2004 Dec;33(12):1013-7.
Abstract/Text
BACKGROUND: Guillian Barre syndrome (GBS) is the most common form of neuromuscular paralysis. It mostly affects young people and can cause long-term residual disability.
OBJECTIVE: This article outlines the rehabilitation treatment for patients recovering from GBS.
DISCUSSION: Recovery from GBS can be prolonged. Early rehabilitation intervention ensures medical stability, appropriate treatment and preventive measures to minimise long term complications. Specific problems include deep venous thrombosis prevention, complications of immobility, dysautonomia, de-afferent pain syndromes, muscle pain and fatigue. Longer-term issues include psychosocial adjustment, return to work and driving, and resumption of the role within the family and community. Effective communication between the GP and rehabilitation physicians is imperative for improved functional outcomes and successful social reintegration.
Demir SO, Köseoğlu F.
Factors associated with health-related quality of life in patients with severe Guillain-Barré syndrome.
Disabil Rehabil. 2008;30(8):593-9. doi: 10.1080/09638280701352626.
Abstract/Text
PURPOSE: To compare the health-related quality of life (HRQOL) in control subjects and patients with severe Guillain-Barré syndrome (GBS) 6 months after rehabilitation. To determine the relationship of several sociodemographic and medical factors with the HRQOL of the GBS survivors.
METHODS: Thirty-one patients with severe GBS and 31 control subjects were included in the study. Demographic and medical variables were recorded. The functional outcome was measured using the Functional Independence Measure (FIM), both at admission and discharge and also at the 6-month follow-up examination. The HRQOLs were assessed by the Nottingham Health Profile (NHP) at the 6-month follow-up examination.
RESULTS: There were significant improvements in functional status as measured by the FIM at discharge and also at 6 months. The scores of all of the NHP dimensions of the GBS patients were significantly higher than in the control subjects. Functional disability scores were highly related to the energy level, physical mobility and emotional reactions of the NHP domains. Education, gender, employment, mechanical ventilation and tendency to depression were the factors most related to the NHP domains. Age and marital status showed no significant correlation with the NHP scores.
CONCLUSION: The HRQOL of the GBS patients remains lower than that of the control subjects. In addition to functional scores, several sociodemographic and medical variables, such as education, psychological factors, gender, mechanical ventilation and employment may play a crucial role in determining the quality of life in persons with GBS.
Meythaler JM, DeVivo MJ, Braswell WC.
Rehabilitation outcomes of patients who have developed Guillain-Barré syndrome.
Am J Phys Med Rehabil. 1997 Sep-Oct;76(5):411-9. doi: 10.1097/00002060-199709000-00012.
Abstract/Text
The objective of this study was to determine associations between early variables (requirement for ventilator support, anemia, indicators of abnormal peripheral nerve function (proprioception, vibratory, fine touch/pinprick, deep-tendon reflexes, cranial nerve involvement, dysautonomia, electrodiagnostic findings), plasmapheresis, age, and gender) and outcome variables (length of acute hospitalization, length of inpatient rehabilitation, Functional Independence Measure (FIM) Rasch converted scores, and acute and rehabilitation charges) in Guillain Barré Syndrome (GBS). The design of the study was a retrospective case review of 39 GBS admissions (as defined by National Institute of Neurologic Disorders and Stroke clinical criteria) to an inpatient rehabilitation unit at a university tertiary care rehabilitation center during a three-year period. The average length of stay for 39 patients requiring transfer to the inpatient rehabilitation unit (40% of all acute care GBS admissions) was 34 days in acute care and 26 days in rehabilitation. The average adjusted charges for inpatient rehabilitation (1993 dollars) was $31,636.28. Those who required ventilator support before rehabilitation v those who did not had an admission mean FIM Rasch converted motor score of 26.6 v a score of 38.3 (P = 0.0469), gained only 10.3 points on their FIM Rasch converted motor score v 27.7 points (P = 0.0001), and had a mean acute length of stay of 66.2 days v 19.3 days (P = 0.0029). Patients requiring ventilator support were more likely to have dysautonomia (P = 0.0009). Thirty-one of 39 patients with GBS (79%) had anemia. No correlation was found between hematocrit or hemoglobin and motor function recovery as assessed via the Rasch transformed FIM motor scores. There was an association between autonomic dysfunction and an increased acute care length of stay (P = 0.0325) and total length of hospital stay (P = 0.0203). Cranial nerve dysfunction resulted in an increase in the acute care length of stay (P = 0.0266), the total length of hospital stay (P = 0.0123), and adjusted hospital charges while undergoing inpatient rehabilitation (P = 0.0235). For patients with GBS necessitating admission to inpatient rehabilitation, the requirement of prior ventilator support most strongly predicts an extended length of stay for inpatient rehabilitation.
Nicholas R, Playford ED, Thompson AJ.
A retrospective analysis of outcome in severe Guillain-Barre syndrome following combined neurological and rehabilitation management.
Disabil Rehabil. 2000 Jul 10;22(10):451-5. doi: 10.1080/09638280050045929.
Abstract/Text
PURPOSE: To determine the outcome in severe Guillain-Barre syndrome following combined neurological and rehabilitation management using standardized disability and handicap measures and to identify the factors which affect this outcome.
METHODS: A retrospective study was performed of 24 patients with Guillain-Barre Syndrome admitted over a 3 year period to the neurological rehabilitation unit of the National Hospital for Neurology and Neurosurgery. Queen Square, London. Disability and handicap on admission and discharge were measured using the modified Barthel Index (BI), Functional Independence Measure (FIM). Environmental Status Scale (ESS) and Handicap Assessment Scale (HAS). Data was collected to identify any factors affecting outcome; age at onset, time to nadir, duration of ventilation, total in-patient stay, duration of stay at the neurological rehabilitation unit, symptoms, signs and electrophysiological findings.
RESULTS: The majority of patients had multiple problems. Outcome was related to the duration of rehabilitation. The mean modified BI score and the FIM score increased whereas ESS scores and HAS scores decreased, these changes were compatible with a reduction in disability and handicap.
CONCLUSIONS: Significant improvement in function occurred during rehabilitation. These changes can be demonstrated using standardized outcome measures.