Acute Guillain-Barré Syndrome Research Articles

High-Speed Video Blink Analysis Improves Detection of Facial Palsy in Early Guillain-Barré Syndrome.

Electrophysiological investigations in early Guillain-Barré Syndrome (GBS) can be nondiagnostic. Improved testing for facial weakness in the early phase of GBS may improve diagnostic processes, as such weakness is found in approximately 50% of patients with GBS. This work pilots the utility of high-speed video analysis to complement blink reflex testing in early GBS. This work prospectively evaluated consecutive patients admitted to a metropolitan teaching hospital in Melbourne, Australia, with suspected acute GBS within the first 14 days of neurological symptoms and compared them to a cohort of healthy controls. Blink reflex testing, mechanically-activated masseter reflexes, and analysis of high-speed video recordings of the evoked blinks were performed at admission (day 0), day 7, and day 21 (±2 days). 19 suspected GBS patients (12 GBS and 7 mimics) were compared to 22 healthy controls. At the first test, 83% of GBS patients and 29% of mimics demonstrated blink reflex abnormalities. 50% of GBS manifested video abnormalities (14% mimics), but abnormalities preceded electrophysiological changes in two GBS patients. The calculated reference values for peak lid velocity and lid excursion by video analysis were ~160 mm/s and 7-8 mm, respectively, with slightly different values for ipsilateral versus contralateral responses. Combining high-speed video analysis and blink reflex testing improves the detection of facial involvement in early GBS and helps discriminate from mimic disorders. Further work in a larger cohort is required to validate the sensitivity and specificity of this technique.

Early Electrophysiology in Suspected Acute Guillain-Barré Syndrome: A Prospective Study of Comprehensive Testing.

Electrophysiologic changes in early Guillain-Barré Syndrome (GBS) can be nondiagnostic. Improved testing paradigms may improve hyperacute treatment. This work prospectively evaluated consecutive patients admitted to a metropolitan teaching hospital in Melbourne, Australia, with suspected acute GBS. We performed extensive neurophysiology at three different time points. Novel tests, including cutaneous silent periods, long latency reflexes, and contraction-induced H reflexes, were assessed. Twenty-three participants were studied, including 13 cases of acute GBS. In total, 69% of acute cases of GBS were accurately diagnosed on the first nerve conduction study using published neurophysiologic criteria, with serial studies rarely altering the GBS subtype classification. Antidromic and orthodromic upper limb sensory studies were diagnostically equivalent. A sural sparing pattern was seen in 77% of cases of GBS at the first test. Long latency reflexes and contraction-induced H reflexes testing were abnormal in most participants but were limited by muscle weakness in some. Cutaneous silent periods testing was unobtainable in approximately 50% of cases because of weakness and did not discriminate from mimic disorders. Abnormalities of long latency reflexes and contraction-induced H reflexes may be helpful where initial electrophysiology is nondiagnostic but are nonspecific. Cutaneous silent periods testing seems of limited value. Comprehensive testing provides diagnostic certainty in most cases of GBS from the very first study.

Acute Guillain-Barré Syndrome After Sacroiliac Joint Fusion

BACKGROUND: Guillain-Barré syndrome (GBS), also called acute inflammatory demyelinating polyradiculopathy (AIDP), is one of the most common causes of acute, acquired weaknesses. GBS is an acute immune-mediated polyneuropathy that presents with progressive weakness of the arms or legs. GBS presents after infection; however, there are few reports that describe acute GBS after chronic pain procedures. CASE REPORT: A 70-year-old man with a past medical history of spinal stenosis status post anterior cervical discectomy and fusion, sacroiliac joint (SIJ) dysfunction status post SIJ fusion presented with imbalance, weakness, and difficulty walking. The patient underwent SIJ fusion one week prior; his weakness progressively worsened, requiring a walker. He underwent a series of labs and diagnostic tests, which were consistent with AIDP/GBS. He was placed on respiratory and cardiac monitoring and started on intravenous immunoglobulin treatment. He started developing bilateral facial palsies and started on plasmapheresis. His symptoms have improved and he was discharged from our inpatient rehab facility on after 28 days. CONCLUSIONS: This case report aims to highlight a rare, but potentially dangerous, complication of AIDP/GBS following an SIJ fusion. KEY WORDS: Sacroiliac joint fusion, sacroiliac joint pain, Guillain-Barré syndrome, case report

Early electrophysiological study variants and their relationship with clinical presentation and outcomes of patients with Guillain-Barré syndrome

This study compared the clinical outcomes of the two main neurophysiological types of Guillain-Barré Syndrome (GBS). Sixty-two GBS patients were examined clinically at onset using Medical Research Council (MRC), Hughes disability scales (HDS), and nerve conduction studies were evaluated in four limbs. The Modified Erasmus GBS outcome score (MEGOS) was assessed 2 weeks after onset. Outcomes were measured after 3 months using MRC and HDS scores. According to electrophysiological data two main groups identified acute inflammatory demyelinating polyneuropathy (AIDP = 31 cases) or acute axonal GBS including inexcitable forms (26 cases). The number of days between onset of weakness and admission was significantly shorter, and gastrointestinal symptoms were significantly higher among the axonal type than AIDP. MRC sum scores at onset and at nadir were significantly worse in the axonal type than in AIDP. Neck muscle weakness, impaired cough reflex, the need for mechanical ventilation, hypoalbuminemia, and hypernatremia were more common in the axonal type. At outcome, 74% of the AIDP were healthy/minor symptoms versus 38.46% of the axonal type. There was a high prevalence of the axonal variant (41.9%) compared with European and North American populations. The axonal type had a significantly worse outcome than AIDP type.

Current status of Guillain-Barré syndrome (GBS) in China: a 10-year comprehensive overview.

Guillain-Barré syndrome (GBS) is an acute inflammatory polyradiculoneuropathy; a disease involving the peripheral nervous system which is the most common cause of acute flaccid paralysis worldwide. So far, it is still lack of a comprehensive overview and understanding of the national epidemiological, clinical characteristics, and the risk factors of GBS in China, as well as differences between China and other countries and regions in these respects. With the global outbreak of the coronavirus disease 2019 (COVID-19), an epidemiological or phenotypic association between severe acute respiratory syndrome coronavirus-2 (SARS-CoV-2) infection and GBS has attracted great attention. In this review, we outlined the current clinical data of GBS in China by retrieving literature, extracting and synthesizing the data of GBS in China from 2010 to 2021. Besides, we compared the characteristics of epidemiology, preceding events and clinical profiles of GBS between China and other countries and regions. Furthermore, in addition to conventional intravenous immunoglobulin (IVIG) and plasma exchange (PE) therapy, the potential therapeutic effects with novel medications in GBS, such as complement inhibitors, etc., have become the research focus in treatments. We found that epidemiological and clinical findings of GBS inChina are approximately consistent with those in the International GBS Outcome Study (IGOS) cohort. We provided an overall picture of the present clinical status of GBS in China and summarized the global research progress of GBS, aiming to further understand the characteristics of GBS and improve the future work of GBS worldwide, especially in countries with the middle and low incomes.

Study of total duration of distal compound muscle action potential in demyelinating and axonal Guillain-Barre’ syndrome

ABSTRACT Objectives Electrophysiology plays a crucial role in Guillain-Barré syndrome (GBS) diagnosis and subtype classification. The aim of our study was to assess the potential role of distal compound muscle action potential (dCMAP) for early differentiation between acute inflammatory demyelinating polyneuropathy (AIDP) and axonal GBS. Methods We retrospectively reviewed the medical records of 24 subjects with AIDP and 18 subjects with axonal GBS. We built up receiver operating characteristic curves for total dCMAP duration and negative phase of dCMAP duration, in order to derive cut-off values able to differentiate between AIDP and axonal GBS. Results The total duration of dCMAP was significantly prolonged in AIDP compared to axonal GBS. AUCs, odds ratio and positive predictive values were higher for total duration than for negative peak duration. Nerve conduction parameters in the lower limbs were more sensitive than those in the upper limbs in distinguishing AIDP from axonal GBS. Discussion Total duration of dCMAP dispersion may capture an adjunctive component of distal demyelination, not measured by the more traditional parameters and may thus represent a useful tool for early differentiation between AIDP and axonal GBS.

A case series of parainfectious Guillain-Barré syndrome linked to influenza A (H1N1) virus infection

Guillain–Barré syndrome (GBS) is an immune-mediated peripheral neuropathy characterized by a typical post-infectious profile. Some post-Zika virus and post-severe acute respiratory syndrome-related coronavirus-2 GBS cases have been reported to occur with very short intervals between the infection and GBS onset. Evaluating 161 GBS patients consecutively admitted to two Italian Regional Hospitals between 2003 and 2019, we found that the only three with an antecedent influenza A (H1N1) virus infection developed GBS within an interval of less than 10 days from the influenza illness. The two of them with a demyelinating subtype promptly recovered without therapy. Overall, the parainfectious cases add heterogeneity to the GBS category, warranting pathogenetic insights.

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.

Pharmacological treatment other than corticosteroids, intravenous immunoglobulin and plasma exchange for Guillain-Barré syndrome.

Plasma exchange and intravenous immunoglobulin, but not corticosteroids, are beneficial in Guillain-Barré syndrome (GBS). The efficacy of other pharmacological agents is unknown. This review was first published in 2011 and previously updated in 2013, and 2016. To assess the effects of pharmacological agents other than plasma exchange, intravenous immunoglobulin and corticosteroids for GBS. On 28 October 2019, we searched the Cochrane Neuromuscular Specialised Register, CENTRAL, MEDLINE, and Embase for treatments for GBS. We also searched clinical trials registries. We included all randomised controlled trials (RCTs) or quasi-RCTs of acute GBS (within four weeks from onset) of all types and degrees of severity, and in individuals of all ages. We discarded trials that investigated only corticosteroids, intravenous immunoglobulin or plasma exchange. We included other pharmacological treatments or combinations of treatments compared with no treatment, placebo or another treatment. We followed standard Cochrane methodology. We found six trials of five different interventions eligible for inclusion in this review. The trials were conducted in hospitals in Canada, China, Germany, Japan and the UK, and included 151 participants in total. All trials randomised participants aged 16 years and older (mean or median age in the trials ranged from 36 to 57 years in the intervention groups and 34 to 60 years in the control groups) with severe GBS, defined by the inability to walk unaided. One trial also randomised patients with mild GBS who were still able to walk unaided. We identified two new trials at this update.The primary outcome measure for this review was improvement in disability grade four weeks after randomisation. Four of six trials had a high risk of bias in at least one respect. We assessed all evidence for the outcome mean improvement in disability grade as very low certainty, which means that we were unable to draw any conclusions from the data. One RCT with 19 participants compared interferon beta-1a (IFNb-1a) and placebo. It is uncertain whether IFNb-1a improves disability after four weeks (mean difference (MD) -0.1; 95% CI -1.58 to 1.38; very low-certainty evidence). A trial with 10 participants compared brain-derived neurotrophic factor (BNDF) and placebo. It is uncertain whether BDNF improves disability after four weeks (MD 0.75; 95% CI -1.14 to 2.64; very low-certainty evidence). A trial with 37 participants compared cerebrospinal fluid (CSF) filtration and plasma exchange. It is uncertain whether CSF filtration improves disability after four weeks (MD 0.02; 95% CI -0.62 to 0.66; very low-certainty evidence). One trial that compared the Chinese herbal medicine tripterygium polyglycoside with corticosteroids with 43 participants did not report the risk ratio (RR) for an improvement by one or more disability grade after four weeks, but did report improvement after eight weeks. It is uncertain whether tripterygium polyglycoside improves disability after eight weeks (RR 1.47; 95% CI 1.02 to 2.11; very low-certainty evidence). We performed a meta-analysis of two trials comparing eculizumab and placebo with 41 participants. It is uncertain whether eculizumab improves disability after four weeks (MD -0.23; 95% CI -1.79 to 1.34; very low-certainty evidence). Serious adverse events were uncommon in each of the trials and evidence was graded as either low or very low. It is uncertain whether serious adverse events were more common with IFNb-1a versus placebo (RR 0.92, 95% CI 0.23 to 3.72; 19 participants), BNDF versus placebo (RR 1.00, 95% CI 0.28 to 3.54; 10 participants) or CSF filtration versus plasma exchange (RR 0.13, 95% CI 0.01 to 2.25; 37 participants). The trial of tripterygium polyglycoside did not report serious adverse events. There may be no clear difference in the number of serious adverse events after eculizumab compared to placebo (RR 1.90, 0.34 to 10.50; 41 participants). We found no clinically important differences in any of the outcome measures selected for this review in any of the six trials. However, sample sizes were small and therefore clinically important benefit or harm cannot be excluded. All six RCTs were too small to exclude clinically important benefit or harm from the assessed interventions. The certainty of the evidence was low or very low for all interventions and outcomes.

Long-term outcomes of Guillain-Barré syndrome possibly associated with Zika virus infection.

BackgroundThis prospective cohort investigation analyzed the long-term functional and neurologic outcomes of patients with Zika virus-associated Guillain-Barré syndrome (GBS) in Barranquilla, Colombia.MethodsThirty-four Zika virus-associated GBS cases were assessed a median of 17 months following acute GBS illness. We assessed demographics, results of Overall Disability Sum Scores (ODSS), Hughes Disability Score (HDS), Zung Depression Scale (ZDS), and Health Related Quality of Life (HRQL) questionnaires; and compared outcomes indices with a normative sample of neighborhood-selected control subjects in Barranquilla without GBS.ResultsMedian age at time of acute neurologic onset was 49 years (range, 10–80); 17 (50%) were male. No deaths occurred. At long-term follow-up, 25 (73%) patients had a HDS 0–1, indicating complete / near complete recovery. Among the group, HDS (mean 1.4, range 0–4), ODSS (mean 1.9, range 0–9) and ZDS score (mean 34.4, range 20–56) indicated mild / moderate ongoing disability. Adjusting for age and sex, Zika virus-associated GBS cases were similar to a population comparison group (n = 368) in Barranquilla without GBS in terms of prevalence of physical or mental health complaints, though GBS patients were more likely to have an ODSS of ≥ 1 (OR 8.8, 95% CI 3.2–24.5) and to suffer from moderate / moderate-severe depression (OR 3.89, 95% CI 1.23–11.17) than the comparison group.ConclusionsLong-term outcomes of Zika virus-associated GBS are consistent with those associated with other antecedent antigenic stimuli in terms of mortality and ongoing long-term morbidity, as published in the literature. Persons with Zika virus-associated GBS more frequently reported disability and depression after approximately one year compared with those without GBS.

Pharmacological treatment other than corticosteroids, intravenous immunoglobulin and plasma exchange for Guillain-Barré syndrome.

Plasma exchange and intravenous immunoglobulin, but not corticosteroids, are beneficial in Guillain-Barré syndrome (GBS). The efficacy of other pharmacological agents is unknown. This review was first published in 2011 and updated in 2013 and 2016. To assess the effects of pharmacological agents other than plasma exchange, intravenous immunoglobulin and corticosteroids for GBS. On 18 January 2016, we searched the Cochrane Neuromuscular Specialised Register, Cochrane Central Register of Controlled Trials, MEDLINE, and Embase for treatments for GBS. We also searched clinical trials registries. We included all randomised controlled trials (RCTs) or quasi-RCTs of acute GBS (within four weeks from onset) of all types and degrees of severity, and in individuals of all ages. We discarded trials that investigated only corticosteroids, intravenous immunoglobulin or plasma exchange. We included other pharmacological treatments or combinations of treatments compared with no treatment, placebo or another treatment. We also identified a number of non-randomised studies during the search, the results of which we considered in the Discussion. We followed standard Cochrane methodology. We identified no new trials during this update of the review. In previous versions of this review we identified only very low quality evidence for four different interventions published in four studies. Each study had a high risk of bias in at least one respect. One RCT with 19 participants comparing interferon beta-1a and placebo showed no clinically important difference in any outcome between groups. Another with 10 participants comparing brain-derived neurotrophic factor and placebo showed no clinically important difference in any outcome between groups. A third with 37 participants comparing cerebrospinal fluid filtration and plasma exchange also showed no clinically important difference in any outcome between groups. In a fourth with 43 participants, the risk ratio for an improvement by one or more disability grade after eight weeks was greater with the Chinese herbal medicine tripterygium polyglycoside than with corticosteroids (risk ratio 1.47; 95% confidence interval 1.02 to 2.11); other outcomes in this trial showed no difference. Serious adverse events were uncommon with each of these treatments and in the control groups. The quality of the evidence was very low. Three small RCTs, comparing interferon beta-1a or brain-derived neurotrophic factor with placebo, and cerebrospinal fluid filtration with plasma exchange, showed no significant benefit or harm for any of the interventions. A fourth small trial showed that the Chinese herbal medicine, tripterygium polyglycoside, hastened recovery in people with GBS to a greater extent than corticosteroids, but this result needs confirmation. We were unable to draw any useful conclusions from the few observational studies we identified.

C1q-targeted inhibition of the classical complement pathway prevents injury in a novel mouse model of acute motor axonal neuropathy

IntroductionGuillain-Barré syndrome (GBS) is an autoimmune disease that results in acute paralysis through inflammatory attack on peripheral nerves, and currently has limited, non-specific treatment options. The pathogenesis of the acute motor axonal neuropathy (AMAN) variant is mediated by complement-fixing anti-ganglioside antibodies that directly bind and injure the axon at sites of vulnerability such as nodes of Ranvier and nerve terminals. Consequently, the complement cascade is an attractive target to reduce disease severity. Recently, C5 complement component inhibitors that block the formation of the membrane attack complex and subsequent downstream injury have been shown to be efficacious in an in vivo anti-GQ1b antibody-mediated mouse model of the GBS variant Miller Fisher syndrome (MFS). However, since gangliosides are widely expressed in neurons and glial cells, injury in this model was not targeted exclusively to the axon and there are currently no pure mouse models for AMAN. Additionally, C5 inhibition does not prevent the production of early complement fragments such as C3a and C3b that can be deleterious via their known role in immune cell and macrophage recruitment to sites of neuronal damage.Results and ConclusionsIn this study, we first developed a new in vivo transgenic mouse model of AMAN using mice that express complex gangliosides exclusively in neurons, thereby enabling specific targeting of axons with anti-ganglioside antibodies. Secondly, we have evaluated the efficacy of a novel anti-C1q antibody (M1) that blocks initiation of the classical complement cascade, in both the newly developed anti-GM1 antibody-mediated AMAN model and our established MFS model in vivo. Anti-C1q monoclonal antibody treatment attenuated complement cascade activation and deposition, reduced immune cell recruitment and axonal injury, in both mouse models of GBS, along with improvement in respiratory function. These results demonstrate that neutralising C1q function attenuates injury with a consequent neuroprotective effect in acute GBS models and promises to be a useful new target for human therapy.

Pharmacological treatment for pain in Guillain-Barré syndrome.

Pain in Guillain-Barré syndrome (GBS) is common, yet it is often under recognised and poorly managed. In recent years, a variety of pharmacological treatment options have been investigated in clinical trials for people with GBS-associated pain. This is an updated version of the original Cochrane review published in Issue 10, 2013. To assess the efficacy and safety of pharmacological treatments for various pain symptoms associated with GBS, during both the acute and convalescent (three months or more after onset) phases of GBS. On 3 November 2014, we searched the Cochrane Neuromuscular Disease Group Specialized Register, CENTRAL, MEDLINE and EMBASE. In addition, we searched ClinicalTrials.gov and the World Health Organization (WHO) International Clinical Trials Registry Platform. We included randomised controlled trials (RCTs) and quasi-RCTs in participants with confirmed GBS, with pain assessment as either the primary or secondary outcome. For cross-over trials, an adequate washout period between phases was required for inclusion. Two review authors independently screened the titles and abstracts of identified records, selected studies for inclusion, extracted eligible data, cross-checked the data for accuracy and assessed the risk of bias of each study. Three short-term RCTs, which enrolled 277 randomised participants with acute phase GBS, were included. Risk of bias in the included studies was generally unclear due to insufficient information. None of the included studies reported the primary outcome selected for this review, which was number of patients with self reported pain relief of 50% or greater. One small study investigated seven-day regimens of gabapentin versus placebo. Pain was rated on a scale from 0 (no pain) to 10 (maximum pain). Amongst the 18 participants, significantly lower mean pain scores were found at the endpoint (day 7) in the gabapentin phase compared to the endpoint of the placebo phase (mean difference -3.61, 95% CI -4.12 to -3.10) (very low quality evidence). For adverse events, no significant differences were found in the incidence of nausea (risk ratio (RR) 0.50, 95% CI 0.05 to 5.04) or constipation (RR 0.14, 95% CI 0.01 to 2.54). A second study enrolling 36 participants compared gabapentin, carbamazepine and placebo, all administered over seven days. Participants in the gabapentin group had significantly lower median pain scores on all treatment days in comparison to the placebo and carbamazepine groups (P < 0.05). There were no statistically significant differences in the median pain scores between the carbamazepine and placebo groups from day 1 to day 3, but from day 4 until the end of the study significantly lower median pain scores were noted in the carbamazepine group (P < 0.05) (very low quality evidence). There were no adverse effects of gabapentin or carbamazepine reported, other than sedation. One large RCT (223 participants, all also treated with intravenous immunoglobulin), compared a five-day course of methylprednisolone with placebo and found no statistically significant differences in number of participants developing pain (RR 0.89, 95% CI 0.68 to 1.16), number of participants with decreased pain (RR 0.95, 95% CI 0.63 to 1.42) or number of participants with increased pain (RR 0.85, 95% CI 0.52 to 1.41) (low quality evidence). The study did not report whether there were any adverse events. Since the last version of this review we found no new studies. While management of pain in GBS is essential and pharmacotherapy is widely accepted as being an important component of treatment, this review does not provide sufficient evidence to support the use of any pharmacological intervention in people with pain in GBS. Although reductions in pain severity were found when comparing gabapentin and carbamazepine with placebo, the evidence was limited and its quality very low. Larger, well-designed RCTs are required to further investigate the efficacy and safety of potential interventions for patients with pain in GBS. Additionally, interventions for pain in the convalescent phase of GBS should be investigated.

First case of anti-ganglioside GM1-positive Guillain–Barré syndrome due to hepatitis E virus infection

A 51-year-old previously healthy woman presented with Guillain-Barré syndrome (GBS) and elevated liver enzymes. Further diagnostic investigations showed the presence of an acute hepatitis E infection associated with anti-ganglioside GM1 antibodies. After treatment with intravenous immunoglobulins, the patient made a rapid recovery. Here, we report the first case of GBS due to acute hepatitis E virus (HEV) infection associated with the presence of anti-ganglioside GM1 antibodies. We also review available literature on the association between acute HEV infection and GBS.

Reduced circulating CD4+CD25+ cell populations in Guillain–Barré syndrome

Guillain–Barré syndrome (GBS) is a monophasic inflammatory disease considered to be due to autoimmunity. In order to test the hypothesis that the disease is associated with a perturbation of the circulating lymphoid cell population, we tested the mononuclear cells from the venous blood of 21 patients with Guillain–Barré syndrome (GBS) and 20 healthy controls by flow cytometry. The proportions and numbers of B and T lymphocytes, and CD4, CD8, double negative and γδ T cell subsets and numbers of monocytes were not significantly different in the patients compared with the controls. However, the number and proportion of CD4+CD25+ cells were reduced in acute GBS (mean number 61.7 cells/μl, 95% CI 42.9–80.4 and mean percentage 4.6%, 95% CI 3.8–5.4) compared with controls (mean number 99.8 cells/μl, 95% CI 74.7–124.9, p = 0.02, and mean percentage 6.0%, 95% CI 4.9–7.1%, p = 0.037). In addition, in GBS patients, the number and proportion of CD4+ T cells expressing CD25+ and HLA-DP, DQ, DR (mean number 11.9 cells/μl, 95% CI 7.6–16.1 and mean percentage 0.8%, 95% CI 0.5–1.1%) was lower than in healthy controls (23.5 cells/μl, 95% CI 16.4–30.6, p = 0.01, and mean percentage 1.4%, 95% CI 1.1–1.8%, p = 0.005. Since CD4+CD25+ cells include cells with special immunoregulatory functions, further investigation of this phenomenon and its relation to possible loss of regulatory T cell function in GBS is warranted.

Anaerobic muscle performance of children after long-term recovery from Guillain-Barré syndrome.

The aim of this study was to evaluate anaerobic muscle performance in children after long-term recovery from Guillain-Barré syndrome (GBS). Participants were recruited from all children and adolescents with GBS admitted to The Hospital for Sick Children, University of Toronto, Canada, between 1988 and 1995. We assessed 17 children (nine males, eight females; mean age at GBS diagnosis 7 years 11 months, SD 4 years 2 months; mean age at testing 14 years 1 month) more than two years after acute GBS. Participants completed the Wingate anaerobic test to evaluate mean and peak muscle power. For mean arm muscle power (mean 47.5% [SD 17.4%] of normal), only two participants were in the normal range. For mean leg muscle power (mean 83% [SD 28.3%] of normal), 15 participants were in the normal range. For arm peak power (mean 92.6% [SD 20.7%] of normal), 12 participants were in the normal range. For leg peak power (mean 116.3% of normal, SD 15.8%), all participants were in the normal range. Detailed manual muscle testing with the modified Medical Research Council scale was done on 34 muscle groups. Data were pooled to give a mean arm and leg muscle strength score (9.7, SD 0.30 and 9.4, SD 0.50 respectively). Pearson's correlations of muscle endurance with arm and leg strength were not significant. Children with GBS have excellent long-term recovery of peak muscle power. Muscle endurance, measured by mean muscle power, was normal in the legs but markedly low in the arms. Pediatric rehabilitation programs after GBS should specifically target endurance of arm muscles.

Long-term outcome in children with Guillain-Barré syndrome

Objective To determine the long-term sequelae and early predictors of sequelae for children with Guillain-Barré syndrome (GBS) after the introduction of intravenous gammaglobulin (IVIG). Study design A cross-sectional case series of children with GBS, at least 2 years after recovery, was performed. Manual muscle strength testing was done on 34 muscle groups. The functional independence measure was used to assess function. A chart review was completed, identifying acute factors that may predict long-term sequelae. Results Forty-seven children with GBS were identified, of whom 30 received IVIG. Persisting long-term muscle weakness with at least one muscle group at a grade 6 level (muscle moves the joint against gravity but only minimal resistance to an applied force) was found in 23% of cases. Each patient had a perfect score on the functional independence measure. Long-term muscle weakness was predicted by young age (P = .03) and a rapid progression to maximal weakness (P = .03). Conclusions Despite the introduction of IVIG, 23% of children with GBS had evidence of long-term mild muscle weakness, with minimal impact on function. Young age and a rapid progression during the acute GBS period predicted long-term sequelae. (J Pediatr 2003;142:305-9)

IgG antiganglioside antibodies in Guillain–Barré syndrome with bulbar palsy

Some patients with Guillain–Barré syndrome (GBS) develop bulbar palsy, which may lead to serious complications during the acute phase of the illness. A serological marker that could predict the occurrence of bulbar palsy would be valuable for the treatment of acute GBS. We examined the serum levels of various IgG antiganglioside antibodies in the sera of 16 patients with GBS with bulbar palsy [GBS-BP(+)] and 72 patients with GBS without bulbar palsy [GBS-BP(−)]. Anti-GT1a antibodies were detected in a higher percentage of the GBS-BP(+) patients (10/16, 63%) than the GBS-BP(−) patients (2/72, 3%). In addition to GT1a, a new disialosylganglioside antigen was recognized by the sera of four GBS-BP(+) patients. Anti-GM1b antibodies were also frequently detected in the sera of the GBS-BP(+) cases. However, anti-GM1 and anti-GalNAc-GD1a antibodies, which are highly associated with acute axonal motor neuropathy (AMAN), were not detected in any of the GBS-BP(+) cases, while anti-GM1 antibodies were detected in 29% (21/72) and anti-GalNAc-GD1a antibodies were detected in 8% (6/72) of the GBS-BP(−) cases. These findings suggest that the presence of particular antiganglioside antibodies might be related with certain clinical manifestations of GBS. In patients who are diagnosed with GBS, the presence or absence of anti-GT1a and anti-GM1b antibodies should be tested at the early stage of GBS so that appropriate therapies that prevent the development of bulbar palsy and improve the outcome of GBS, may be initiated.

A CASE CONTROL STUDY OF ANTI‐GANGLIOSIDE AND ANTI‐CAMPYLOBACTER JEJUNI ANTIBODIES IN GBS: CORRELATION WITH ANTECEDENT EVENTS AND WITH CLINICAL FEATURES

Anti‐ganglioside antibodies have been reported frequently in Guillain‐Barré syndrome (GBS), where they were often associated with an antecedent Campylobacter Jejuni (CJ) infection. To determine whether this association is also present in an Italian population we implemented a case‐control study of GBS patients in Western Lombardia from 1996 to 1998. We collected sera from 47 GBS patients in the acute phase (39 of whom were redrawn after three weeks), 43 neurological controls (NC), and 41 non‐neurological controls (NNC). Sera were tested by ELISA for IgG, IgM, and IgA antibodies to GM1, GD1a, GD1b, GM2, GQ1b, and to CJ. None of the anti‐ganglioside antibodies tested was significantly more frequent in GBS patients during the acute phase of the disease than in NC or NNC. All anti‐ganglioside antibodies tested were more frequent during recovery than in the acute phase, even if the difference was significant only for anti‐GQ1b IgG and IgM antibodies. The frequency of anti‐GQ1b IgG was also significantly higher in convalescent GBS than in NC and NNC sera. There was no correlation between the presence of anti‐ganglioside IgM antibodies in acute sera and IgG antibodies in convalescent sera. Anti‐CJ IgG antibodies were only found in two acute GBS patients and in one NC. Both CJ‐positive GBS patients had an antecedent diarrhea and one also had high anti‐GM1 IgG. An antecedent upper respiratory tract infection (URTI) but not diarrhea was significantly more frequent in GBS than in NC/NNC. URTI in GBS patients was not associated with a higher frequency of anti‐ganglioside antibodies than in NC/NNC. In conclusion, in this study anti‐ganglioside antibodies were not associated with GBS in the acute phase, but tended to increase during the course of the disease possibly suggesting a secondary immune response. This finding might reflect the low frequency of diarrhea and CJ infection in our GBS population, supporting the hypothesis of a regional variability in the etiopathogenetic mechanism of GBS.

Short-term variability of blood pressure and heart rate in Guillain-Barreá syndrome without respiratory failure

The effect of Guillain-Barré syndrome (GBS) on the short-term variability of blood pressure and heart rate was evaluated in six patients presenting with a moderate form of the syndrome, i.e. unable to stand up unaided and without respiratory failure, at the height of the disease and during recovery. The patients were compared with six age-matched healthy volunteers. During the acute phase of the syndrome, GBS patients exhibited a significant heart rate elevation (+26 beats/min compared with healthy subjects), but the acceleratory response to atropine, or to 60 degrees head-up tilt, was maintained. Resting plasma noradrenaline levels were high in acute GBS, but the secretory response to tilt was preserved. Desensitization to noradrenaline was observed in acute GBS with a reduced pressor action of this alpha-adrenoceptor agonist. Blood pressure levels were normal and head-up tilt did not induce orthostatic hypotension in this moderate form of GBS. Power spectral analysis demonstrated marked alterations in cardiovascular variability. The overall heart period variability was markedly reduced with the reduction predominantly in the high-frequency (respiratory) range (-73%). The low-frequency component of heart period variability was also reduced (-54%). This cardiovascular profile of moderate GBS at the height of the disease could result from a demyelination of the reflex loop controlling respiratory oscillations in heart rate and from a desensitization of the arterial tree to an elevated plasma noradrenaline. Sympathetic nervous activation may contribute to the high resting heart rate in acute GBS.