Guillain-Barré Syndrome Disability Scale Research Articles

Outcome of Guillain-Barré syndrome with bulbar palsy

BackgroundElective intubation is advocated in Guillain-Barré syndrome (GBS) with bulbar palsy to prevent aspiration pneumonia and lung collapse. We evaluate the outcome of GBS patients with bulbar palsy, and also compare the risks and benefits of intubation and MV in them. Methods187 GBS patients with bulbar palsy from a cohort of 547 GBS registry were analyzed. Detailed clinical records and peak disability on a 0-6 GBS Disability Scale (GBSDS) were noted. The patients were intubated if arterial blood gas (ABG) analysis revealed hypoxia, hypercarbia or acidosis. The patients with normal ABG parameters were fed by nasogastric tube, and nursed in lateral position. Occurrence of pneumonia, in-hospital death and outcomes at 6-months were classified as complete (GBSDS <2), partial (GBSDS 2-3) and poor (GBSDS >3). Results76/187(40.6%) patients required MV, and they had a shorter duration of illness (p = 0.007), higher peak disability (p < 0.001), autonomic dysfunction (p < 0.001) and more frequently received IVIg (p = 0.02). Pneumonia (63% vs 10.8%; p < 0.001) and in-hospital deaths (7.9% vs 1.8%; p = 0.06) were more frequent in MV group compared to nasogastric fed group. At 6-months,104 (55.6%) patients recovered completely. On multivariate analysis, the independent predictors of poor outcome were peak disability [Adjusted Odds Ratio (AOR) 9.84, 95% Confidence Interval (CI) 3.15-30.74, p < 0.0001], day of hospitalization from disease onset (AOR 1.09, 95% Cl 1.01-1.01; p=0.009) and requirement of MV (AOR 0.10; 95% 0.02-0.50; p = 0.005). ConclusionGBS patients with bulbar palsy may be managed by nasogastric feeding and nursing in lateral position without increasing the risk of pneumonia. Mechanical ventilation based on ABG does not worsen outcomes of GBS with bulbar palsy.

Predictors of long-term health-related quality of life in Guillain-Barré syndrome: A hospital-based study

ObjectiveThis study aimed to determine the impact of patients’ baseline clinical, neurophysiological data, and management plan of Guillain-Barré syndrome (GBS) on long-term quality of life (QoL) and to identify its potential predictors. MethodsSeventy-nine GBS patients were recruited. On admission, participants were evaluated using the Medical Research Council (MRC) sumscore, GBS disability scale (GDS), and Erasmus GBS Respiratory Insufficiency Score (EGRIS). Neurophysiological data were collected, and a management plan was devised. MRC sumscore was repeated at nadir. MRC, GDS and Short Form Survey (SF-36) were assessed at first-year follow-up. ResultsThe mean age was 37.84 ± 17.26 years, with 43 male patients (54.4%). QoL at one year correlated significantly with baseline clinical variables (age, number of days between weakness and admission, MRC sumscore at onset and nadir, high GDS, and EGRIS scores). Antecedent events, especially diarrhoea, neck muscle weakness, autonomic dysfunction, cranial nerve involvement, and mechanical ventilation (MV), associated with worse QoL. Axonal GBS patients had lower QoL than AIDP patients, and PE patients exhibited lower QoL than IVIG patients. Multiple regression analysis showed that older age, diarrhoea, number of days between weakness and admission, neck muscle weakness, cranial nerve involvement, autonomic dysfunction, early MV, and MRC at onset and nadir and high GDS could predict poor QoL. ConclusionOlder age, more days between weakness and admission, neck muscle weakness, cranial nerve involvement, autonomic dysfunction, early MV, diarrhoea, low MRC at onset and nadir, high GDS at onset, axonal type, and PE treatment were potential predictors of poor QoL in GBS.

Clinical and Electrophysiological Characteristics of Very Early Guillain-Barré Syndrome.

This study compared the clinical and electrodiagnostic (EDX) features and long-term outcomes of patients with very early Guillain-Barré syndrome (VEGBS, duration of illness ≤4 days) and those with early/late (>4 days)-presenting GBS. One hundred patients with GBS were clinically evaluated and categorized into VEGBS and early/late GBS groups. Electrodiagnostic studies were performed on the bilateral median, ulnar, and fibular motor nerves and the bilateral median, ulnar, and sural sensory nerves. Admission and peak disability were assessed using the 0 to 6 Guillain-Barré Syndrome Disability Scale (GBSDS). The primary outcome was disability at 6 months, which was categorized as complete (GBSDS ≤1) or poor (GBSDS ≥2). The secondary outcomes were frequencies of abnormal electrodiagnostic findings, in-hospital progression, and mechanical ventilation (MV). Patients with VEGBS had higher peak disability (median 5 vs. 4; P = 0.02), frequent in-hospital disease progression (42.9% vs. 19.0%, P < 0.01), needed MV (50% vs. 22.4%; P < 0.01), and less frequent albuminocytologic dissociation (52.4% vs. 74.1%; P = 0.02) than those with early/late GBS. Thirteen patients were lost to follow-up at 6 months (nine patients with VEGBS and four patients with early/late GBS). The proportion of patients with complete recovery at 6 months was comparable (60.6% vs. 77.8%; P = ns ). Reduced d-CMAP was the most common abnormality, noted in 64.7% and 71.6% of patients with VEGBS and early/late GBS, respectively ( P = ns). Prolonged distal motor latency (≥130%) was more common in early/late GBS than in VEGBS (36.2% vs. 25.4%; P = 0.02), whereas absent F-waves were more frequent in VEGBS (37.7% vs. 28.7%; P = 0.03). Patients with VEGBS were more disabled at admission than those with early/late GBS. However, 6 month's outcomes were similar between the groups. F-wave abnormalities were frequent in VEGBS, and distal motor latency prolongation was common in early/late GBS.

A Retrospective Single-center Study of Presentation and Prognosis of Guillain-Barré Syndrome in Pediatric Patients

Background: Guillain-Barré syndrome (GBS) is a post-infectious immune-mediated peripheral neuropathy, progressing bilaterally and often symmetrically and affecting sensory and motor function. Most cases completely recover, but around 20% of cases may lead to complications, incomplete recovery, or even death. Objectives: This study aims to assess the prognosis of GBS in pediatric patients and possible associated conditions regarding recovery or prognosis. Methods: We investigated 71 cases of GBS admitted to Mofid Pediatric Hospital from March 2014 to March 2017. Demographic, clinical, and laboratory data were retrospectively recorded and analyzed. Two follow-up visits were performed after 1 to 3 and 5 to 8 years from onset, according to the GBS Disability Scale, and recovery of motor function was assessed during patients’ visits to the clinic. Results: We found 35 male and 36 female subjects with an average age of 6.17 ± 3.82 (range 0.9 up to 15 years old); cases were mostly presented with myalgia and weakness (78.9%) followed by headache, found in 5 patients (7%). Around 84.5% of patients had an upper respiratory infection as their antecedent infection. Fifteen cases of autonomic dysfunction were observed, and 15 patients had cranial nerve involvement. Most cases had the acute inflammatory demyelinating polyradiculoneuropathy (AIDP) form of GBS on electrophysiologic tests. Analysis showed only axonal involvement was significantly correlated with poor prognosis (P-value

Childhood Guillain–Barre syndrome in the SARS‐CoV‐2 era: Is there any causative relation?

We reported an association between SARS-CoV-2 infection and Guillain-Barre syndrome (GBS). From 37 patients with GBS, previous SARS-CoV-2 clinical clues, including fever, cough, and diarrhea, were recorded in 18 patients. Among them, SARS-CoV-2 IgG was detected in seven patients, considered confirmed as cases. SARS-CoV-2 PCR was positive in just one patient. Although we found no increase in patient recruitment during the pandemic compared to previous years, our study indicated that SARS-CoV-2 is associated with poorer outcomes regarding GBS disability scale and hospital stay.

A SUCCESSIVE CLINICAL STORY ON GUILLAIN-BARRE SYNDROME UNDER THE LIGHT OF AYURVEDA: A CASE STUDY

Guillain-Barre syndrome (GBS) is a group of autoimmune syndromes comprising the disease's demyelinating and acute axonal degenerating forms. It is also an acute-onset, monophasic, immune-mediated polyneuropathy that often follows an antecedent infection. Here is an interesting case study where a 16-year-old girl with her parents reported to the outpatient department with the chief complaint of inability to walk without support, weakness in bilateral calf muscles, weakness in bilateral upper limbs and lower limbs and tingling sensation, pulling type of pain in the right ankle joint and also the heaviness of bilateral lower limbs, more in the morning. By examination, her sensory and motor systems showed impairment; one was hypotonic, proprioception was affected, and her gait was a high steppage gait. Her assessment was done by using Hughes GBS Disability Scale- 3/6. The patient’s nerve conduction study revealed severe motor and sensory axonal neuropathy in the upper and lower limbs. By seeing the nature of the disease and the patient's symptoms, we correlated with sarvanga vata according to our classics. Treatments were koshta shodhana (gut cleansing), abhyanga (massage of the whole body with medicated oil), Shastikashali panda sweda (Rubbing of medicated rice poultice over the body), basti (transrectal administration of medicines) and oral medicaments. Panchakarma treatments were for 30 days, followed by oral medications for the next 140 days. The intervention period of 154 days showed complete recovery of all the motor and sensory deficits; however, follow-up of the patient was maintained for 269 days, looking into the sustainability of the outcomes.

Outcome of Guillain-Barré syndrome following intravenous immunoglobulin compared to natural course.

Intravenous immunoglobulin (IVIg) is recommended in Guillain-Barré syndrome (GBS), but its efficacy may vary in different subtypes. We report the outcomes of patients with GBS following IVIg treatment compared to the natural course (NC). We also compare the effect of IVIg treatment in different subtypes of GBS. From a cohort of 528 GBS subjects, we have extracted 189 patients who received IVIg and compared their outcomes with 199 age- and peak disability-matched patients who did not receive IVIg, plasmapheresis, or corticosteroid. Disability was assessed using the 0-6 Guillain-Barré Syndrome Disability Scale (GBSDS). Clinical and neurophysiological subtypes were recorded. The primary outcome was functional disability at 6 months, which was categorized as complete (GBSDS ≤ 1), partial (GBSDS 2-3), or poor (GBSDS &gt; 3). The secondary outcomes were in-hospital death, duration of hospitalization, and mechanical ventilation. In-hospital death (2.6% vs. 2%, p=0.74) and 3-month poor recovery (20.7% vs. 18%) were similar in the IVIg and NC groups. At 6 months, however, a lesser proportion of patients in the IVIg group had poor recovery (2.2% vs. 8.3%, p=0.026). The outcomes of IVIg and NC were compared in 72 acute motor axonal neuropathy (AMAN) and 256 acute inflammatory demyelinating polyradiculoneuropathy (AIDP) patients. IVIg therapy did not alter the outcome in AMAN but resulted in a lesser proportion of poor recovery at 6 months in AIDP (0.8% vs. 6.6%, p=0.03). IVIg is beneficial in AIDP variants of GBS but not in the AMAN subtype. A customized treatment may be cost-effective until a randomized controlled trial is conducted in AMAN.

Responsiveness of 2 Different Ability Outcome Measures in Guillain-Barré Syndrome.

The most frequently used ability outcome measure in Guillain-Barré syndrome (GBS) is the GBS disability scale (GDS). Recently developed inflammatory Rasch-built overall disability (I-RODS) scale has been suggested to be used in inflammatory polyneuropathies. In the present study, we wanted to assess the comparative responsiveness of I-RODS and GDS in subjects who were diagnosed with GBS during a follow-up period of 6 months. Our prospective, multicentric study included 72 subjects. Patients were tested, using GDS and I-RODS, on day 14, day 28, month 3, and month 6 from the start of the symptoms. We defined improvement as a reduction for 1 or more points on GDS or improvement on I-RODS as defined by Draak (2014). Between days 14 and 28 there was an improvement in 28% of patients as measured with GDS and only in 10% patients as measured with I-RODS. At month 3 compared with day 14, we noticed an improvement in GDS score in 90% of GBS patients and I-RODS score in 65%. At month 6 improvements were noticed in 94% of patients measured by GDS and 78% according to I-RODS. Our findings support the use of GDS in an acute phase of GBS. I-RODS have their role mostly during a longer follow-up period when the majority of patients are ambulant and their other abilities besides walking are also of great importance.

P-OT007. Is it GBS treatment related fluctuation or inadequate treatment of GBS? A case report

Introduction . Guillain-Barre Syndrome (GBS) is an autoimmune disease with acute ascending paralysis of the limb. Intravenous Immunoglobulin (IVIG) and plasmapharesis are still the effective treatment for GBS. In some cases, recurrence and treatment related fluctuation (TRF) of GBS can occur. TRF is defined as improvement in the GBS disability scale of at least one grade after completion of therapy followed by a worsening of the disability scale of at least one grade within the first 2 months after disease onset. Meanwhile, recurrence of GBS is defined as two or more episodes that fulfilled the criteria for GBS, with either a minimum interval greater than 4 months between episodes if the patient did not recover completely or greater than 2 months when there was a complete or near complete recovery after the previous episode. Results . A 47-year-old male with acute ascending paralysis accompanied with paresthesia in his leg. EMG result showed moderate axonal demyelinating motor sensory polyneuropathy (AMSAN) and diagnosed with GBS AMSAN type. He had plasmapharesis 2 times and his condition was improved (GBS Disability scale from 4 before plasmapharesis and became 3 after treatment). One month after onset, he complained his condition became worse again (GBS Disability scale 4). We assessed this condition as TRF. We conducted plasmapharesis again but only 2 times, because of limitation in national health insurance claim. Patient was discharged from hospital with no improvement. He routinely underwent physiotherapy and not on any pharmacotherapy. His condition improved in six months. Conclusion . We assumed that TRF in this patient was caused by inadequacy of plasmapharesis. This problem arises because of high cost of plasmapharesis while the national health insurance does not cover all serial plasmapharesis.

Phase IIB Randomized Trial on the Use of 4-Aminopyridine in Guillain-Barré Syndrome

ObjectiveTo determine the safety and efficacy of orally delivered 4-aminopyridine (4-AP) in persons with Guillain-Barré Syndrome (GBS) >6 months from initial diagnosis. DesignA randomized, double-blind, placebo-controlled, crossover study. SettingTertiary care clinical outpatient program. ParticipantsNineteen participants enrolled (14 male, 5 female; N=19), neurologic impairment secondary to GBS and functional loss on the FIM motor score (stable for ≥12mo) and >3.0 but <5.0 on the American Spinal Injury motor scale. Twelve participants (mean age, 59y; range, 23-77y) completed the study. InterventionsA 4-AP dose-escalation study with 8 weeks in each period with a 3-week washout period, followed by 3 months open-label follow-up. Main Outcome MeasuresFIM motor score was the primary outcome measure; also evaluated were the American Spinal Injury motor strength score (all limbs), handheld dynamometer, 6-minute walk test, Medical Outcomes Study 12-Item Short Form, Center for Epidemiological Studies Depression scale, Positive and Negative Affect Schedule, pain, GBS disability scale, Jepsen-Taylor Hand Function Test, Minnesota Manual Dexterity Test and Minnesota Rate of Manipulation Test, Get Up and Go Test, McGill Pain Inventory, Craig Handicap Assessment and Reporting Technique, and participant self-evaluation. ResultsSeven participants discontinued the study prematurely: 3 because of adverse events, 3 because of travel difficulties or relocation, and 1 because of pretreatment laboratory abnormalities. After removing 3 participants with maximum FIM scores, 4-AP arm trended superior to placebo (P=.065). Patients subjectively could always tell when they were on the active agent usually by tingling sensations or a sense of wellness. No statistically significant differences were found for other outcome measures although there were strong trends. ConclusionsThis study demonstrates the safety of 4-AP in the patient population with GBS as the predominate goal of the study. A trend toward improved function after treatment was noted with most patients electing to stay on the medication after the trial.

Altered serum levels of IL-36 cytokines (IL-36α, IL-36β, IL-36γ, and IL-36Ra) and their potential roles in Guillain-Barré syndrome.

Guillain-Barré syndrome (GBS) is an acute autoimmune neurological disorder mainly involving the peripheral nerves. Currently, various cytokines have been shown to be involved in the pathogenesis of GBS. Because of their similar biological structures, interleukin (IL)-36α, IL-36β, IL-36γ, and IL-36 receptor antagonist (Ra) were all renamed and collectively called IL-36 cytokines. The roles of IL-36 cytokines in GBS currently remain unclear.Forty-two patients with GBS and 32 healthy volunteers were included in our study. Serum IL-36α, β, γ, and interleukin-36 receptor antagonist (IL-36Ra) levels of patients with GBS in the acute and remission phases and healthy volunteers were measured by enzyme-linked immunosorbent assay (ELISA). In addition, we examined the serum levels of other inflammatory factors that have been shown to be involved in GBS pathogenesis, represented by IL-17 and tumor necrosis factor-α (TNF-α). Furthermore, the correlations between the serum levels of IL-36 cytokines and different clinical data or the serum levels of other inflammatory factors in GBS patients were analyzed.Significantly higher serum IL-36α and IL-36γ levels were measured in the acute phase than in the remission phase and in healthy control (HC) subjects (P < .05), while lower serum IL-36Ra levels were measured in the acute phase than in the remission phase and in HC subjects (P < .05). Serum IL-36α and IL-36γ levels were positively correlated with GBS disability scale scores (GDSs), while serum IL-36Ra levels were negatively correlated with GDSs. Correlation analyses among inflammatory factors showed that serum IL-36α and IL-36γ levels in GBS patients were positively correlated with serum IL-17 and TNF-α levels, while serum IL-36Ra levels were negatively correlated with the levels of these 2 inflammatory factors. Similar results were observed in cerebrospinal fluid (CSF), IL-36α and IL-36γ levels in CSF were positively correlated with GDSs, while IL-36Ra levels in CSF were negatively correlated with GDSs. Additionally, the serum and CSF levels of IL-36α and IL-36γ in the axonal subtype of GBS patients were higher than those in the demyelination subtype.Based on our findings, IL-36 cytokines may be involved in the pathogenesis of GBS and some of these cytokines may help predict the disease severity and other clinical characteristics of GBS.

Relationship of Glycemic Status with Disease Severity in Guillain-Barré Syndrome

Background: Guillain-Barré syndrome (GBS) is an acute autoimmune polyneuroradiculopathy characterized by flaccid paralysis which may lead to respiratory failure requiring intensive care.&#x0D; Objective: The purpose of the present study was to explore the relationship between the fasting plasma glucose (FPG), hemoglobin A1c (HbA1c) and disease severity of GBS patients who are not known to have DM.&#x0D; Methodology: This cross-sectional study included adult GBS patients without having DM [age 35 (22-48) years, median (interqurtile range, IQR); 39 male 22 female] who were admitted to Neurology department, National Institute of Neurosciences and Hospital, Dhaka, Bangladesh from July 2018 to June 2019. Demographics, clinical data were noted and FPG, HbA1c were measured. Disease severity were assessed by the GBS disability scale ranging from 0 to 6 with increasing score reflecting increased disability.&#x0D; Results: Patients with more severe GBS (disability score ≥4, unable to walk) had higher frequency of elevated FPG &gt;5.5 mmol/L (61.2%; 30/49) in comparison to those with less severe GBS (disability score ≤3, able to walk; FPG &gt;5.5 mmol/L in 16.7%, 2/12; p=0.006). But distribution of HbA1c category was not different across the groups (disability score ≥4 vs. ≤3: HbA1c &lt;5.7: 40% vs. 58%; 5.7-6.4: 50% vs. 25%; &gt;6.4: 10% vs. 17%; p=0.296). Participants with elevated FPG were elder [elevated vs. normal FPG: 40 (28-54) vs. 25 (19-43) years; median (IQR), p=0.012] and had higher CSF glucose (p=0.002) than those with normal FPG, but there was no difference in respct of gender, MRC sum score, requirement of assisted ventilation, CSF protein, GBS subtypes and duration of hospital stay (p=not significant for all).&#x0D; Conclusions: Patients with severe GBS have higher frequency of elevated FPG but not HbA1c. An acute change in glucose metabolism may occur in GBS which needs further study.&#x0D; Journal of National Institute of Neurosciences Bangladesh, 2020;6(2): 96-100

Nerve conduction studies in Guillain-Barré syndrome: Influence of timing and value of repeated measurements

ObjectiveNerve conduction studies (NCS) are essential to differentiate between demyelinating and axonal subtypes in Guillain-Barré syndrome (GBS). However, it is debated to which extent the delay of NCS after symptom onset and repeated measurements during the disease course influence the diagnostic accuracy. MethodsWe evaluated NCS in 93 patients with a classical GBS applying two widely used criteria (Hadden's and Rajabally's). The initial measurements after symptom onset were compared to follow-up studies where available (n = 43). We analyzed the influence of NCS timing after symptom onset and clinical severity on fulfilling the electrophysiological criteria for axonal or demyelinating subtypes and evaluated the impact of repeated measurements. We further evaluated the presence of reversible conduction failure. ResultsA higher GBS disability scale at nadir correlated with a successful subclassification whereas the delay of the first NCS after symptom onset did not influence the diagnostic yield (75% for Hadden's and 68% for Rajabally's criteria for the first assessment). A second measurement allowed the additional successful classification in 19% and 14% of patients, respectively. On the other hand, a repeated measurement in patients with an initial successful classification resulted in a different subtype in 5% and 7%, respectively. Reversible conduction failure was found in 7% of patients. ConclusionClinical severity but not timing of NCS influenced the fulfilment of electrophysiological criteria for either the axonal or demyelinating subtype. Repeated electrophysiological measurements led to a further specification or a change in subtype classification in a relevant proportion of patients.

Self-reported autonomic dysfunction in a recovery phase of Guillain-Barré syndrome

ObjectiveAutonomic dysfunction occurs in approximately two-thirds of Guillain-Barré syndrome (GBS) patients in the acute phase of the disease. Although improving over time, subclinical autonomic involvement may be present for 3–8 years after the GBS episode. The aim of this study was to determine the frequency of self-reported autonomic disorders in GBS patients three and six months after disease onset compared to healthy controls (HCs). MethodsOur study included adult patients diagnosed with GBS from May 2017 until May 2018 in seven healthcare centers (67.6 % with demyelinating and 13.6 % with axonal syubtype). Functional disability was assessed by the Guillain-Barré syndrome disability scale (GDS). Each subject filled in the Serbian version of the SCOPA-Aut questionnaire. Using GDS and SCOPA-Aut, patients were tested at month 3 (M3) (n = 71) and month 6 (M6) (n = 70) from symptom onset. ResultsDysautonomia was more common in patients with GBS compared to HCs at M3 (p < 0.01), while there was no difference at M6 (p > 0.05). Among autonomic disorders, constipation, complications to pass stool, and orthostatic hypotension were the most frequently reported. Patients with axonal variants had worse total SCOPA-Aut scores at M3 in comparison to AIDP patients (11.7 ± 10.1 vs. 6.1 ± 5.1, p < 0.05). GDS score correlated with the total SCOPA-Aut score. ConclusionAutonomic symptoms are common in GBS patients during the recovery phase. They are more pronounced in patients with axonal forms of GBS and those with a higher degree of functional disability.

Longitudinal study of neuropathic pain in patients with Guillain-Barré syndrome.

The aim of this study was to analyze neuropathic pain (NeP) and its therapy in patients with Guillain-Barré syndrome (GBS) during a 6-month follow-up period. This longitudinal multicenter study included 69 newly diagnosed adult GBS patients. NeP diagnosis was based on the criteria of Finnerup and confirmed by the PainDETECT Questionnaire (PD-Q). Severity of GBS was assessed by GBS disability scale (GDS). Patients were assessed: on day 14 (D14), day 28 (D28), month 3 (M3), and month 6 (M6) from the disease onset. At D14, pain was present in 85.5% of patients, while 26.4% had NeP. At M6, 72.5% of patients had pain, 20.0% of them NeP. In acute GBS, pain intensity was higher in patients with NeP compared to those with non-NeP (p < 0.01). Pain intensity in patients with NeP did not change during time, but it decreased in patients with non-NeP at M6 (p < 0.05). Around 20% of GBS patients were on specific NeP medication throughout the observed period. One quarter of patients with NeP were not on specific NeP drug in the acute phase. Up to one third of patients with NeP were on NeP medication but still had significant NeP. Pooled PD-Q score was in correlation with pooled GDS score (rho = + 0.43, p < 0.01). NeP is a common and potentially severe symptom in GBS that may persist for months. It is important to recognize NeP, start specific treatment on time, in adequate doses, and for prolonged period of time.

Rate of progression of Guillain-Barré syndrome is not associated with the short-term outcome of the disease.

There are no many data on association between progression rate of Guillain-Barré syndrome (GBS) and disease outcome. The aim of our study was to analyze short-term outcome of GBS in relation to the rate of disease progression. Our retrospective study included patients diagnosed with GBS in seven tertiary healthcare centers from 2009 to 2014. According to the rate of disease progression from onset of symptoms to the nadir, patients were divided in three groups: rapid-onset GBS (nadir reached in maximum 48h), gradual-onset (nadir reached in three to 14days), and slow-onset (nadir in 15 to 28days). GBS disability scale (GDS) was used to assess functional disability at nadir and on discharge. Among 380 patients included in the study, 24 (6.3%) patients had rapid-onset, 274 (72.1%) gradual-onset, and 82 (21.6%) slow-onset GBS. Time from the onset of the disease to the hospital admission was much shorter in faster-onset forms (3.0 ± 4.1days in rapid-onset vs. 6.8 ± 9.5days in gradual-onset and 21.0 ± 9.6days in slow-onset GBS, p < 0.01). Preceding events were less commonly identified in slow-onset forms. Patients with rapid-onset GBS were more likely to have axonal variants (p < 0.05). All three groups of patients were treated in a similar way, and there were no differences in GDS score at nadir (p > 0.05) and on discharge (p > 0.05) and no differences in the duration of hospital stay. Faster progression of GBS does not imply a poorer short-term functional outcome of the disease.

Six-month prospective study of quality of life in Guillain-Barre syndrome.

Guillain-Barre syndrome (GBS) is an acute disease of the peripheral nerves and their roots. Quality of life (QoL) in the first year after acute episode of GBS is still underresearched area. The aim of our study was to investigate QoL in GBS patients during a 6-month follow-up period. Multicentric, prospective study included 74 adult patients with GBS (54% males). GBS disability scale (GDS) was used to assess functional disability (severe disability GDS>2), and Individualized Neuromuscular Quality of Life Questionnaire (INQoL) to asses QoL. Patients were tested on day 14, day 28, month 3, and month 6 from symptom onset. Disability as measured by GDS improved during time (P<.01). INQoL scores also improved during time (P<.01) but were not able to differentiate between day 14 and day 28, and some scores also did not make difference between month 3 and 6 (pain, social relations, emotions and total INQoL score; P>.05). Pooled GDS scores correlated with pooled INQoL scores, especially with independence, activities, and weakness subscores (P<.01). Multiple linear regression analysis showed that GDS at day 14 (β=.52, P<.01) and fatigue score at day 14 (β=.41, P<.01) were independent predictors of the worse GDS at month 6 (adjusted R2 =.34, P<.01 for overall model). During a 6-month follow-up period of GBS patients, we observed a gradual recovery of patients' disability and QoL. Our study confirms the importance of patient-reported outcomes and their ability to capture some important issues that are omitted by classic ability measures such as GDS.

Original research: Second IVIg course in Guillain-Barré syndrome with poor prognosis: the non-randomised ISID study

ObjectiveTo compare disease course in patients with Guillain-Barré syndrome (GBS) with a poor prognosis who were treated with one or with two intravenous immunoglobulin (IVIg) courses.MethodsFrom the International GBS Outcome...

A unicenter, prospective study of Guillain-Barré syndrome in Spain.

We report a prospective study analysing clinical characteristics, subtyping and prognosis in Guillain-Barré syndrome (GBS). The study was based on consecutive GBS patients admitted between 2009 and 2017. Disability was serially assessed using the GBS disability scale. Fifty-six GBS patients were identified with an average age of 55years (range, 5-86years) and a male/female ratio of 2.1. The interval to nadir was <7days in 59% of cases, and 7 to 28days in the remainder; at nadir, 35.5% of patients were able to walk unaided, and 64.5% did not. Mechanical ventilation was needed in 20% of cases. There were two fatal cases. Clinical variants included paraparetic GBS seven cases, Miller Fisher syndrome one case, and acute sensory ataxic neuropathy (ASAN) one case. Serial electrophysiology showed a demyelinating pattern in 62.5% of cases, axonal in 28.5%, inexcitable in 1.8%, equivocal in 1.8%, and normal in 5.4%. Very early (1 to 4days after onset) electrophysiology was done in 18 patients; equivocal or normal features in six of them evolved into an axonal pattern in four. Reversible conduction failure of sensitive nerves occurred in ASAN. Antiganglioside antibodies were only detected in axonal GBS. At 24-month follow-up, functional outcome did not differ between demyelinating and axonal GBS. Clinico-pathological correlation in an early fatal case is reported. This GBS study demonstrates comparable clinical features to previous investigations from well-defined populations. There was a relatively high prevalence of axonal GBS. We provide new pathophysiological insights on nerve conduction alterations.

Efficient design and analysis of randomized controlled trials in rare neurological diseases: An example in Guillain-Barré syndrome.

BackgroundRandomized controlled trials (RCTs) pose specific challenges in rare and heterogeneous neurological diseases due to the small numbers of patients and heterogeneity in disease course. Two analytical approaches have been proposed to optimally handle these issues in RCTs: covariate adjustment and ordinal analysis. We investigated the potential gain in efficiency of these approaches in rare and heterogeneous neurological diseases, using Guillain-Barré syndrome (GBS) as an example.MethodsWe analyzed two published GBS trials with primary outcome ‘at least one grade improvement’ on the GBS disability scale. We estimated the treatment effect using logistic regression models with and without adjustment for prognostic factors. The difference between the unadjusted and adjusted estimates was disentangled in imbalance (random differences in baseline covariates between treatment arms) and stratification (change of the estimate due to covariate adjustment). Second, we applied proportional odds regression, which exploits the ordinal nature of the GBS disability score. The standard error of the estimated treatment effect indicated the statistical efficiency.ResultsBoth trials were slightly imbalanced with respect to baseline characteristics, which was corrected in the adjusted analysis. Covariate adjustment increased the estimated treatment effect in the two trials by 8% and 18% respectively. Proportional odds analysis resulted in lower standard errors indicating more statistical power.ConclusionCovariate adjustment and proportional odds analysis most efficiently use the available data and ensure balance between the treatment arms to obtain reliable and valid treatment effect estimates. These approaches merit application in future trials in rare and heterogeneous neurological diseases like GBS.