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Pieter A Van Doorn - One of the best experts on this subject based on the ideXlab platform.
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diagnosis and management of Guillain Barre Syndrome in ten steps
Nature Reviews Neurology, 2019Co-Authors: Sonja E Leonhard, R A C Hughes, Pieter A Van Doorn, David R Cornblath, Kathleen Bateman, Maria Lucia Brito Ferreira, Melissa R Mandarakas, Francisco De Assis Aquino Gondim, Mario Emilio Dourado, Badrul IslamAbstract:Guillain-Barre Syndrome (GBS) is a rare, but potentially fatal, immune-mediated disease of the peripheral nerves and nerve roots that is usually triggered by infections. The incidence of GBS can therefore increase during outbreaks of infectious diseases, as was seen during the Zika virus epidemics in 2013 in French Polynesia and 2015 in Latin America. Diagnosis and management of GBS can be complicated as its clinical presentation and disease course are heterogeneous, and no international clinical guidelines are currently available. To support clinicians, especially in the context of an outbreak, we have developed a globally applicable guideline for the diagnosis and management of GBS. The guideline is based on current literature and expert consensus, and has a ten-step structure to facilitate its use in clinical practice. We first provide an introduction to the diagnostic criteria, clinical variants and differential diagnoses of GBS. The ten steps then cover early recognition and diagnosis of GBS, admission to the intensive care unit, treatment indication and selection, monitoring and treatment of disease progression, prediction of clinical course and outcome, and management of complications and sequelae.
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Guillain Barre Syndrome pathogenesis diagnosis treatment and prognosis
Nature Reviews Neurology, 2014Co-Authors: Bianca Van Den Berg, Bart C Jacobs, Christiaan Fokke, Judith Drenthen, Christa Walgaard, Pieter A Van DoornAbstract:Guillain-Barre Syndrome (GBS) is a potentially life-threatening postinfectious disease characterized by rapidly progressive, symmetrical weakness of the extremities. About 25% of patients develop respiratory insufficiency and many show signs of autonomic dysfunction. Diagnosis can usually be made on clinical grounds, but lumbar puncture and electrophysiological studies can help to substantiate the diagnosis and to differentiate demyelinating from axonal subtypes of GBS. Molecular mimicry of pathogen-borne antigens, leading to generation of crossreactive antibodies that also target gangliosides, is part of the pathogenesis of GBS; the subtype and severity of the Syndrome are partly determined by the nature of the antecedent infection and specificity of such antibodies. Intravenous immunoglobulin and plasma exchange are proven effective treatments but many patients have considerable residual deficits. Discrimination of patients with treatment-related fluctuations from those with acute-onset chronic inflammatory demyelinating polyneuropathy is important, as these conditions may require different treatments. Novel prognostic models can accurately predict outcome and the need for artificial ventilation, which could aid the selection of patients with a poor prognosis for more-individualized care. This Review summarizes the clinical features of and diagnostic criteria for GBS, and discusses its pathogenesis, treatment and prognosis.
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diagnosis of Guillain Barre Syndrome and validation of brighton criteria
Brain, 2014Co-Authors: Christiaan Fokke, Pieter A Van Doorn, Bianca Van Den Berg, Judith Drenthen, Christa Walgaard, Bart C JacobsAbstract:Guillain-Barre 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-Barre 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-Barre 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-Barre 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-Barre Syndrome in the current cohort study, can be used to improve the sensitivity of the diagnostic criteria.
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mortality in Guillain Barre Syndrome
Neurology, 2013Co-Authors: Bianca Van Den Berg, Pieter A Van Doorn, Carina Bunschoten, Bart C JacobsAbstract:Objective: To determine the frequency, timing, causes, and risk factors of death in Guillain-Barre Syndrome (GBS). Methods: Prospectively collected data were reviewed from a cohort of 527 patients with GBS previously included in 1 observational and 3 therapeutic studies. Risk factors were identified by comparing deceased and surviving patients with GBS. Results: Fifteen (2.8%) of 527 patients with GBS died within 6 months of follow-up at highly variable time points during the disease course, with a median time from onset of weakness to death of 76 days (interquartile range 23–152 days). In 356 patients with an extended follow-up of 12 months, the mortality rate was 3.9%. Only 3 patients (20%) died during the acute progressive phase and 2 patients (13%) died during the plateau phase. Ten patients (67%) died during the recovery phase after neurologic improvement, most frequently from respiratory or cardiovascular complications. Eleven patients (73%) were admitted to an intensive care unit during the course of disease, but only 7 patients (47%) died in the intensive care unit. Risk factors for death were age ( p p = 0.02), mechanical ventilation ( p p = 0.035), and time to peak disability ( p = 0.039). Conclusions: Death after GBS predominantly occurs in the elderly and severely affected patients, especially during the recovery phase. Future research is required to determine whether mortality of GBS can be reduced by intensified monitoring in patients with an increased risk profile.
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distinguishing acute onset cidp from fluctuating Guillain Barre Syndrome a prospective study
Neurology, 2010Co-Authors: Liselotte Ruts, Bart C Jacobs, Judith Drenthen, Pieter A Van DoornAbstract:Objective: The aim of the study was to provide criteria that can help to distinguish between GBS-TRF and A-CIDP in the early phase of disease. Background: The distinction between Guillain-Barre Syndrome (GBS) with fluctuations shortly after start of treatment (treatment-related fluctuations, or GBS-TRF) and chronic inflammatory demyelinating polyneuropathy with acute onset (A-CIDP) is difficult but important because prognosis and treatment strategy largely differ. Methods: Patients with GBS (n = 170) were included in a prospective longitudinal study. Patients with GBS-TRF (n = 16) and patients with A-CIDP (n = 8) were analyzed and compared. Extended clinical data, biologic material, and electrophysiologic data were collected during 1 year follow-up. Results: The first TRF in the GBS-TRF group always occurred within 8 weeks (median 18 days; range 10–54 days) from onset of weakness. In the GBS-TRF group, 5 (31%) patients had a second TRF and none had more TRFs. At all timepoints, patients in the A-CIDP group were less severely affected than patients with GBS-TRF, did not need artificial ventilation, rarely had cranial nerve dysfunction, and tended to have more CIDP-like electrophysiologic abnormalities. More GBS-TRF patients were severely affected and more patients had sensory disturbances when compared to the GBS group without fluctuations. Conclusions: The diagnosis of acute-onset chronic inflammatory demyelinating polyneuropathy (CIDP) should be considered when a patient thought to have Guillain-Barre Syndrome deteriorates again after 8 weeks from onset or when deterioration occurs 3 times or more. Especially when the patient remains able to walk independently and has no cranial nerve dysfunction or electrophysiologic features likely to be compatible with CIDP, maintenance treatment for CIDP should be considered.
R A C Hughes - One of the best experts on this subject based on the ideXlab platform.
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diagnosis and management of Guillain Barre Syndrome in ten steps
Nature Reviews Neurology, 2019Co-Authors: Sonja E Leonhard, R A C Hughes, Pieter A Van Doorn, David R Cornblath, Kathleen Bateman, Maria Lucia Brito Ferreira, Melissa R Mandarakas, Francisco De Assis Aquino Gondim, Mario Emilio Dourado, Badrul IslamAbstract:Guillain-Barre Syndrome (GBS) is a rare, but potentially fatal, immune-mediated disease of the peripheral nerves and nerve roots that is usually triggered by infections. The incidence of GBS can therefore increase during outbreaks of infectious diseases, as was seen during the Zika virus epidemics in 2013 in French Polynesia and 2015 in Latin America. Diagnosis and management of GBS can be complicated as its clinical presentation and disease course are heterogeneous, and no international clinical guidelines are currently available. To support clinicians, especially in the context of an outbreak, we have developed a globally applicable guideline for the diagnosis and management of GBS. The guideline is based on current literature and expert consensus, and has a ten-step structure to facilitate its use in clinical practice. We first provide an introduction to the diagnostic criteria, clinical variants and differential diagnoses of GBS. The ten steps then cover early recognition and diagnosis of GBS, admission to the intensive care unit, treatment indication and selection, monitoring and treatment of disease progression, prediction of clinical course and outcome, and management of complications and sequelae.
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plasma exchange for Guillain Barre Syndrome
Cochrane Database of Systematic Reviews, 2017Co-Authors: Jean-claude Raphaël, Sylvie Chevret, R A C Hughes, Djillali AnnaneAbstract:Background Guillain-Barre Syndrome is an acute symmetric, usually ascending and usually paralysing illness, due to inflammation of peripheral nerves. It is thought to be caused by autoimmune factors, such as antibodies. Plasma exchange removes antibodies and other potentially injurious factors from the blood stream. It involves connecting the patient's blood circulation to a machine which exchanges the plasma for a substitute solution, usually albumin. Several studies have evaluated plasma exchange for Guillain-Barre Syndrome. Objectives To systematically review the evidence concerning the efficacy of plasma exchange for treating Guillain-Barre Syndrome. Search strategy Search of the Cochrane Neuromuscular Disease Trial Register for randomised trials concerning plasma exchange in Guillain-Barre Syndrome, search of the bibliographies of identified papers and enquiry from the authors of the papers. Selection criteria Randomised and quasi-randomised trials of plasma exchange versus sham exchange or supportive treatment. Data collection and analysis Potentially relevant papers were scrutinised by two reviewers and the selection of eligible studies was agreed by them and a third reviewer. Data were extracted by one reviewer and checked by a second reviewer. Some missing data were obtained from the authors of studies. Main results Six eligible trials concerning 649 patients were identified, all comparing plasma exchange versus supportive treatment alone. Primary outcome measures ~bullet~Time to recover walking with aid In the only two trials for which this measure was reported, the median time to recover this ability was faster in the plasma exchange than the control group. ~bullet~Time to onset of motor recovery in mildly affected patients In the one trial for which this measure was available, the time was significantly shortened in the plasma exchange group. Secondary outcome measures ~bullet~Improvement in disability grade at four weeks In five trials, there were significantly more patients who had improved by one disability grade or more in the plasma exchange group as compared to the control group. Patients treated with plasma exchange fared significantly better in the following secondary outcome measures: time to recover walking without aid, percentage of patients requiring artificial ventilation, duration of ventilation, full muscle strength recovery after one year, and severe sequelae after one year. There were less patients with infectious events and cardiac arrhythmias in the plasma exchange than the control group. Subgroup analyses Plasma exchange was beneficial in patients with mild, moderate and severe (needing ventilation) Guillain-Barre Syndrome. It was beneficial in patients with a disease duration of seven or less days and also in those with disease lasting more than seven days. However, in the only trial that enrolled patients up to 30 days from disease onset, the benefit of plasma exchange in patients treated after seven days was less apparent. Type of treatment Single studies showed that two plasma exchanges were significantly superior to none for mild Guillain-Barre Syndrome and four to two for moderate Guillain-Barre Syndrome, but that six were not superior to four for severe Guillain-Barre Syndrome requiring ventilation. One study suggested that continuous flow plasma exchange was significantly superior to intermittent flow. Another study found no significant difference between the two techniques. The same study found a significantly higher rate of adverse events with fresh frozen plasma as the replacement fluid than albumin. Plasma exchange compared with cerebrospinal fluid filtration A single trial comparing these two treatments did not show any difference in outcomes but was too small to demonstrate equivalence. Reviewer's conclusions Plasma exchange is the first and only treatment that has been proven to be superior to supportive treatment alone in Guillain-Barre Syndrome. Consequently, plasma exchange should be regarded as the treatment against which new treatments, such as intravenous immunoglobulin, should be judged. In mild Guillain-Barre Syndrome two sessions of plasma exchange are superior to none. In moderate Guillain-Barre Syndrome four sessions are superior to two. In severe Guillain-Barre Syndrome six sessions are no better than four. Continuous flow plasma exchange machines may be superior to intermittent flow machines and albumin to fresh frozen plasma as the exchange fluid. Plasma exchange is more beneficial when started within seven days after disease onset rather than later, but was still beneficial in patients treated up to 30 days after disease onset. The value of plasma exchange in children less than 12 years old is not known. There is insufficient evidence to determine whether cerebrospinal fluid filtration is equivalent to plasma exchange.
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Guillain Barre Syndrome after exposure to influenza virus
Lancet Infectious Diseases, 2010Co-Authors: Helmar C Lehmann, Hanspeter Hartung, Bernd C Kieseier, R A C HughesAbstract:Guillain-Barre Syndrome (GBS) is an acute, acquired, monophasic autoimmune disorder of peripheral nerves that develops in susceptible individuals after infection and, in rare cases, after immunisation. Exposure to influenza via infection or vaccination has been associated with GBS. We review the relation between GBS and these routes of exposure. Epidemiological studies have shown that, except for the 1976 US national immunisation programme against swine-origin influenza A H1N1 subtype A/NJ/76, influenza vaccine has probably not caused GBS or, if it has, rates have been extremely low (less than one case per million vaccine recipients). By contrast, influenza-like illnesses seem to be relevant triggering events for GBS. The concerns about the risk of inducing GBS in mass immunisation programmes against H1N1 2009 do not, therefore, seem justified by the available epidemiological data. However, the experiences from the 1976 swine flu vaccination programme emphasise the importance for active and passive surveillance to monitor vaccine safety.
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supportive care for patients with Guillain Barre Syndrome
JAMA Neurology, 2005Co-Authors: John T Sladky, R A C Hughes, David R Cornblath, Eelco F M Wijdicks, Estelle Benson, Angelika F Hahn, Jay M Meythaler, Richard J Barohn, James C StevensAbstract:A multidisciplinary consensus group searched MEDLINE from 1966 to May 2003, extracted relevant references, and prepared recommendations on supportive care for Guillain-Barre Syndrome. In the absence of randomized controlled trials, we agreed on recommendations by consensus based on observational studies and expert opinion. In the acute phase in bed-bound adult patients, the group recommended the use of heparin and graduated pressure stockings to prevent deep vein thrombosis, monitoring for blood pressure, pulse, autonomic disturbances, and respiratory failure, and the timely institution of artificial ventilation and tracheostomy. Pain management is difficult, but carbamazepine or gabapentin may help. The cautious use of narcotic analgesics may be needed. Disabled patients should be treated by a multidisciplinary rehabilitation team and should receive an assistive exercise program. Persistent fatigue following Guillain-Barre Syndrome is common and may be helped by an exercise program. Because of a very small and possibly only theoretical increase in the risk of recurrence following immunization, the need for immunization should be reviewed on an individual basis. More research is needed to identify optimal methods for all aspects of supportive care.
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epidemiological study of Guillain Barre Syndrome in south east england
Journal of Neurology Neurosurgery and Psychiatry, 1998Co-Authors: J H Rees, R D Thompson, Nigel Smeeton, R A C HughesAbstract:Objectives—To determine the incidence, treatment,and outcome of Guillain-Barre Syndrome in south east England. Methods—Patients presenting with confirmed Guillain-Barre Syndrome between 1 July 1993 and 30 June 1994 were recruited via a voluntary reporting scheme coordinated by the British Neurological Surveillance Unit, hospital activity data collected from acute admitting hospitals within the South East and South West Thames Regional Health Authorities, death certificates, and a contemporary research study of Guillain-Barre Syndrome and Campylobacter jejuni infection. All patients were followed up for one year to determine outcome. Results—Seventy nine patients were recruited, 35 (44%) male, 44 (56%) female, including three children (two boys, one girl). The crude (95% confidence interval (95% CI)) annual incidence was 1.2 (0.9‐ 1.4) cases/100 000 population and 1.5 (1.3‐ 1.8)/100 000 when adjusted for undetected cases. Twenty (25%) patients required ventilation for an average (SD) of 42 (64) days. Thirty six (46%) patients received intravenous human immunoglobulin, five (6%) received plasma exchange, 11 (14%) both treatments, three (4%) steroids, and 25 (32%) no immunomodulatory treatment.One year later,six patients (8%) had died, all of whom were older than 60, three (4%) remained bedbound or ventilator dependent,seven (9%) were unable to walk unaided, 14 (17%) were unable to run, and 49 (62%) had made a complete or almost complete recovery. Increasing age was significantly associated with a poorer outcome at one year. Conclusions—Despite the frequent use of modern immunomodulatory treatments Guillain-Barre Syndrome still carries considerable morbidity and mortality. (J Neurol Neurosurg Psychiatry 1998;64:74‐77)
Bart C Jacobs - One of the best experts on this subject based on the ideXlab platform.
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Guillain Barre Syndrome pathogenesis diagnosis treatment and prognosis
Nature Reviews Neurology, 2014Co-Authors: Bianca Van Den Berg, Bart C Jacobs, Christiaan Fokke, Judith Drenthen, Christa Walgaard, Pieter A Van DoornAbstract:Guillain-Barre Syndrome (GBS) is a potentially life-threatening postinfectious disease characterized by rapidly progressive, symmetrical weakness of the extremities. About 25% of patients develop respiratory insufficiency and many show signs of autonomic dysfunction. Diagnosis can usually be made on clinical grounds, but lumbar puncture and electrophysiological studies can help to substantiate the diagnosis and to differentiate demyelinating from axonal subtypes of GBS. Molecular mimicry of pathogen-borne antigens, leading to generation of crossreactive antibodies that also target gangliosides, is part of the pathogenesis of GBS; the subtype and severity of the Syndrome are partly determined by the nature of the antecedent infection and specificity of such antibodies. Intravenous immunoglobulin and plasma exchange are proven effective treatments but many patients have considerable residual deficits. Discrimination of patients with treatment-related fluctuations from those with acute-onset chronic inflammatory demyelinating polyneuropathy is important, as these conditions may require different treatments. Novel prognostic models can accurately predict outcome and the need for artificial ventilation, which could aid the selection of patients with a poor prognosis for more-individualized care. This Review summarizes the clinical features of and diagnostic criteria for GBS, and discusses its pathogenesis, treatment and prognosis.
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diagnosis of Guillain Barre Syndrome and validation of brighton criteria
Brain, 2014Co-Authors: Christiaan Fokke, Pieter A Van Doorn, Bianca Van Den Berg, Judith Drenthen, Christa Walgaard, Bart C JacobsAbstract:Guillain-Barre 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-Barre 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-Barre 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-Barre 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-Barre Syndrome in the current cohort study, can be used to improve the sensitivity of the diagnostic criteria.
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mortality in Guillain Barre Syndrome
Neurology, 2013Co-Authors: Bianca Van Den Berg, Pieter A Van Doorn, Carina Bunschoten, Bart C JacobsAbstract:Objective: To determine the frequency, timing, causes, and risk factors of death in Guillain-Barre Syndrome (GBS). Methods: Prospectively collected data were reviewed from a cohort of 527 patients with GBS previously included in 1 observational and 3 therapeutic studies. Risk factors were identified by comparing deceased and surviving patients with GBS. Results: Fifteen (2.8%) of 527 patients with GBS died within 6 months of follow-up at highly variable time points during the disease course, with a median time from onset of weakness to death of 76 days (interquartile range 23–152 days). In 356 patients with an extended follow-up of 12 months, the mortality rate was 3.9%. Only 3 patients (20%) died during the acute progressive phase and 2 patients (13%) died during the plateau phase. Ten patients (67%) died during the recovery phase after neurologic improvement, most frequently from respiratory or cardiovascular complications. Eleven patients (73%) were admitted to an intensive care unit during the course of disease, but only 7 patients (47%) died in the intensive care unit. Risk factors for death were age ( p p = 0.02), mechanical ventilation ( p p = 0.035), and time to peak disability ( p = 0.039). Conclusions: Death after GBS predominantly occurs in the elderly and severely affected patients, especially during the recovery phase. Future research is required to determine whether mortality of GBS can be reduced by intensified monitoring in patients with an increased risk profile.
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recognizing Guillain Barre Syndrome in preschool children
Neurology, 2011Co-Authors: Joyce Roodbol, Christa Walgaard, M C Y De Wit, M De Hoog, Coriene E Catsmanberrevoets, Bart C JacobsAbstract:Objective: To determine whether recognition of Guillain-Barre Syndrome (GBS) is delayed in preschool children, what causes this delay, and if the clinical presentation differs from older children. Methods: In a retrospective cohort study, standardized data from all children with GBS seen at the Erasmus MC Sophia Children9s University Hospital in Rotterdam from 1987 to 2009 were collected regarding clinical presentation, patient9s delay, initial diagnosis, and doctor9s delay to the diagnosis. We compared preschool children ( Results: GBS was diagnosed in 23 preschool children and in 32 older children. Fifteen (68%) of the preschool children were initially misdiagnosed compared to 6 (21%) of the older children ( p = 0.001). Median patient delay to consult a pediatrician in both age groups was the same (5.0 days). The median doctor9s delay to diagnose possible GBS in preschool children was significantly longer than in older children (3 days vs 0 days). In one-quarter of preschool children, this doctor9s delay was more than 1 week, up to 22 days. In preschool children, refusal to walk and pain in the legs were the most frequent presenting symptoms (65%), while older children presented with more classic symptoms of weakness and paresthesias. The preschool children were initially misdiagnosed with myopathy, tonsillitis, meningitis, rheumatoid disorders, coxitis, or discitis. Conclusion: The diagnosis of GBS in preschool children is delayed compared to older children. This delay is partly explained by the nonspecific clinical presentation, challenging neurologic examination, and alternative diagnoses in preschool children.
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distinguishing acute onset cidp from fluctuating Guillain Barre Syndrome a prospective study
Neurology, 2010Co-Authors: Liselotte Ruts, Bart C Jacobs, Judith Drenthen, Pieter A Van DoornAbstract:Objective: The aim of the study was to provide criteria that can help to distinguish between GBS-TRF and A-CIDP in the early phase of disease. Background: The distinction between Guillain-Barre Syndrome (GBS) with fluctuations shortly after start of treatment (treatment-related fluctuations, or GBS-TRF) and chronic inflammatory demyelinating polyneuropathy with acute onset (A-CIDP) is difficult but important because prognosis and treatment strategy largely differ. Methods: Patients with GBS (n = 170) were included in a prospective longitudinal study. Patients with GBS-TRF (n = 16) and patients with A-CIDP (n = 8) were analyzed and compared. Extended clinical data, biologic material, and electrophysiologic data were collected during 1 year follow-up. Results: The first TRF in the GBS-TRF group always occurred within 8 weeks (median 18 days; range 10–54 days) from onset of weakness. In the GBS-TRF group, 5 (31%) patients had a second TRF and none had more TRFs. At all timepoints, patients in the A-CIDP group were less severely affected than patients with GBS-TRF, did not need artificial ventilation, rarely had cranial nerve dysfunction, and tended to have more CIDP-like electrophysiologic abnormalities. More GBS-TRF patients were severely affected and more patients had sensory disturbances when compared to the GBS group without fluctuations. Conclusions: The diagnosis of acute-onset chronic inflammatory demyelinating polyneuropathy (CIDP) should be considered when a patient thought to have Guillain-Barre Syndrome deteriorates again after 8 weeks from onset or when deterioration occurs 3 times or more. Especially when the patient remains able to walk independently and has no cranial nerve dysfunction or electrophysiologic features likely to be compatible with CIDP, maintenance treatment for CIDP should be considered.
Frédéric Ghawché - One of the best experts on this subject based on the ideXlab platform.
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increase in cases of Guillain Barre Syndrome during a chikungunya outbreak french polynesia 2014 to 2015
Eurosurveillance, 2015Co-Authors: Erwan Oehler, P Larre, S Lastère, Isabelle Leparcgoffart, Emmanuel Fournier, Stephanie Cubizolle, Chantal Sookhareea, Frédéric GhawchéAbstract:During the recent chikungunya fever outbreak in French Polynesia in October 2014 to March 2015, we observed an abnormally high number of patients with neurological deficit. Clinical presentation and complementary exams were suggestive of Guillain-Barre Syndrome (GBS) for nine patients. All nine had a recent dengue-like Syndrome and tested positive for chikungunya virus (CHIKV) in serology or RT-PCR. GBS incidence was increased four- to nine-fold during this period, suggesting a link to CHIKV infection.
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zika virus infection complicated by Guillain Barre Syndrome case report french polynesia december 2013
Eurosurveillance, 2014Co-Authors: Emily Oehler, P Larre, S Lastère, H P Mallet, Léo Baudouin, Isabelle Leparcgoffart, Laurent Watrin, Florent Valour, Didier Musso, Frédéric GhawchéAbstract:Zika fever, considered as an emerging disease of arboviral origin, because of its expanding geographic area, is known as a benign infection usually presenting as an influenza-like illness with cutaneous rash. So far, Zika virus infection has never led to hospitalisation. We describe the first case of Guillain-Barre Syndrome (GBS) occurring immediately after a Zika virus infection, during the current Zika and type 1 and 3 dengue fever co-epidemics in French Polynesia. .
Guy M Mckhann - One of the best experts on this subject based on the ideXlab platform.
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Guillain Barre Syndrome in northern china relationship to campylobacter jejuni infection and anti glycolipid antibodies
Brain, 1995Co-Authors: Ban Mishu, David R Cornblath, Arthur K Asbury, C Y Gao, John W Griffin, Martin J Blaser, Guy M MckhannAbstract:Guillain-Barre Syndrome has been considered to be primarily an acute inflammatory demyelinating polyneuropathy (AIDP). Our experience with Guillain-Barre 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-Barre Syndrome, defined clinically, in northern China. In 1991 and 1992, we characterized by electrodiagnostic criteria 129 Chinese patients with Guillain-Barre 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-Barre 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-Barre 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-Barre Syndrome.(ABSTRACT TRUNCATED AT 250 WORDS)
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Guillain Barre Syndrome in northern china the spectrum of neuropathological changes in clinically defined cases
Brain, 1995Co-Authors: John W Griffin, David R Cornblath, Ban Mishu, C Y Gao, Guy M Mckhann, P Xue, C Macko, C Yang, M TianAbstract:The pathology of the Guillain-Barre Syndrome remains controversial, and autopsied cases available for study by contemporary techniques are uncommon. Large numbers of cases clinically diagnosed as Guillain-Barre Syndrome occur in northern China. In this study we examined the neuro-pathological changes in 12 autopsied cases from Hebei Province, China. Eleven died early in the course of their disease. In all cases tissue was specially handled and fixed for electron microscopy and for immunocytochemistry. Three of these 12 cases had typical acute inflammatory demyelinating polyneuropathy (AIDP) with lymphocytic infiltration and macrophage-mediated demyelination, reproducing the pathological picture most often reported in Guillain-Barre Syndrome in North America, Europe, and Australia. Six cases had predominantly axonal involvement, characterized by Wallerian-like degeneration of nerve fibres, with only minimal demyelination and with minimal inflammation in five. Three cases, even though paralysed at the time of death, had only very mild changes in the spinal roots and sciatic nerves. Within the group of six predominantly axonal cases, there were important differences both in the severity of the abnormalities and in the classes of fibres involved. Three cases had extensive Wallerian-like degeneration of sensory as well as motor fibres acute motor-sensory atonal neuropathy (AMSAN), while in the other three cases the fibre degeneration affected the motor nerve fibres almost exclusively. These latter cases establish a structural basis for the clinical and electrophysiological picture termed the acute motor atonal neuropathy (AMAN) pattern. In both the AMAN and the AMSAN patterns, a prominent feature was the presence of macrophages within the periaxonal space, surrounding or displacing the axon, and surrounded by an intact myelin sheath. These studies show that the early pathological changes in cases clinically diagnosed as the Guillain-Barre Syndrome are diverse and not restricted to the well-known pattern of AIDP, and that the predominant pathological patterns may differ in different parts of the world. The differences in pathological findings between acute inflammatory demyelinating polyneuropathy and the axonal patterns are likely to reflect differences in the pathogenetic mechanisms. The periaxonal macrophages in the axonal patterns suggest that an important epitope may be localized to the axolemma or periaxonal space. The mild cases indicate that severe paralysis can occur early in Guillain-Barre Syndrome without prominent structural changes along the nerve, suggesting that physiological block or nerve terminal changes may be implicated.