A comparison of immunomodulation therapies in mechanically ventilated patients with Guillain Barré syndrome

A comparison of the effectiveness of immunomodulatory therapies in patients with Guillain Barré syndrome (GBS) who require mechanical ventilation (MV) is important for patient treatment and cost. We aimed to compare the effectiveness of three modes of intervention on the outcome of patients with GBS receiving MV: intravenous immunoglobulin (IVIgG); small volume plasmapheresis (SVP) and large volume plasmapheresis (LVP). Patients with GBS satisfying National Institute of Neurological and Communicative Disorders and Stroke 1990 criteria and requiring MV between 1997 between 2007 were analyzed. The primary outcome parameters evaluated were mortality, duration of MV, hospital stay and Hughes scale at discharge from hospital. Of the 173 (Male: Female, 118:55) patients who required MV during the study, 106 patients received single modality treatment (IVIgG 31, LVP 45, SVP 30) based on availability, affordability and feasibility. Patients receiving IVIgG had a higher incidence of severe weakness and bulbar involvement. The mean duration of MV (p = 0.61), total hospital stay (p = 0.44) and Hughes scale at discharge (p = 0.31) did not differ among the three groups. Complications were similar in the three treatment groups except for hypoalbuminemia and anemia, which were more common in patients in the LVP group. In conclusion, the outcome of patients treated with these three immunomodulatory treatment modalities did not vary. The beneficial effects of SVP in our study warrant further randomized control trials especially in resource-constrained settings.     

Selective Immune Adsorption Treatment of Severe Guillain Barré Syndrome in the Intensive Care Unit

Background  

The effectiveness of plasma exchange and intravenous application of immunoglobulins (IVIG) for the treatment of the Guillain Barré syndrome (GBS) has been demonstrated in large collectives. In contrast, there are only a few investigations in GBS patients with severe symptoms admitted to the intensive care unit (ICU) and treated with selective immune adsorption (SIA). We compared the efficacy and safety of SIA only versus SIA followed by IVIG in patients with severe GBS.     

Tracheostomy or Not: Prediction of Prolonged Mechanical Ventilation in Guillain–Barré Syndrome

Background

Respiratory insufficiency occurs in 20 % of Guillain–Barré syndrome (GBS) patients, and the duration of mechanical ventilation (MV) ranges widely. We identified predictors of prolonged MV to guide clinical decision-making on tracheostomy.

Methods

We analyzed prospectively collected data from 552 patients with GBS in the context of two clinical trials and three cohort studies in The Netherlands. Potential predictors for prolonged MV, defined as duration of ≥14 days, were considered using crosstabs. Selected predictors were analyzed using Cox regression analysis.

Results

On a total of 150 (27 %) patients requiring MV, 106 (71 %) patients needed prolonged MV. The median duration of MV was 28 days (Interquartile Range [IQR] 12–60 days). The strongest observed predictors of prolonged MV were muscle weakness and axonal degeneration or unexcitable nerves on nerve conduction studies. Patients who are unable to lift the arms from the bed (bilateral Medical Research Council [MRC] of deltoid muscles of 0–2) at 1 week after intubation have an 87 % chance to require prolonged MV versus 69 % in patients who are able to lift the arms from the bed (bilateral MRC of deltoid muscles of 3–10). Patients in this last group who had axonal degeneration or unexcitable nerves on nerve conduction studies also have a 90 % chance to require prolonged MV.

Conclusions

Ventilated GBS patients who are unable to lift the arms from the bed and patients who have axonal degeneration or unexcitable nerves at 1 week are at high risk of prolonged MV, and tracheostomy should be considered in these patients.

     

The study of sleep disorder factors in patients with Guillain–Barré syndrome

In this study, we explore the sleep disorders and its associated factors in patients with Guillain–Barré syndrome (GBS), so as to work out appropriate interventions to promote early recovery of the patients. This study subjects included 49 patients with GBS who had been admitted to the Department of Neurology at The Affiliated Hospital of Hebei University, fulfilling National Institute of Neurological and Communicative Diseases and Stroke (NINCDS) criteria for GBS; 37 cases were male and 12 female (age: 27–68 years). Patients were evaluated once daily for two consecutive weeks. By using Wong and Baker Face Scale (WBFS) to evaluate the numbness and pain in patients, 0 points representing completely no pain and 10 points represents the most severity of the pain reactions; the same applies for numbness. The GBS Disability Scale (GBS DS) is used to evaluate the severity of GBS. The Hospital Anxiety and Depression Scale (HADS) is used to evaluate the anxiety and depression the patient is experiencing. All patients take routine EMG and sleep EEG. The sleep quality of the subjects was evaluated by the Pittsburgh Sleep Quality Index Scale (PSQI) and Richard Campbell Sleep Rating Scale. This study found that the application of ventilators, numbness, anxiety and severe limb movement disorders are the main factors causing sleep disorders. Cerebrospinal fluid (CSF) protein concentration is also associated with sleep disorders. But, no obvious abnormalities were found in sleep EEG. The application of the ventilator, numbness, anxiety and severe limb movement disorder are main factors causing sleep disorders. CSF protein concentration is also associated with sleep disorders.     

The role of cytokines in Guillain–Barré syndrome

Cytokines play an important role in the pathogenesis of autoimmune diseases including Guillain–Barré syndrome (GBS) and its animal model experimental autoimmune neuritis (EAN). In this article, we reviewed the current knowledge of the role of cytokines such as TNF-α, IFN-γ, IL-1β, IL-6, IL-12, IL-18, IL-23, IL-17, IL-10, IL-4 and chemokines in GBS and EAN as unraveled by studies both in the clinic and the laboratory. However, these studies occasionally yield conflicting results, highlighting the complex role that cytokines play in the disease process. Efforts to modulate cytokine function in GBS and other autoimmune disease have shown efficiency indicating that cytokines are important therapeutic targets.     

Selective Digestive Tract Decontamination Decreases Time on Ventilator in Guillain–Barré Syndrome

Background

Ventilator-associated pneumonia (VAP) occurs in more than half of mechanically ventilated patients with Guillain–Barré syndrome (GBS) and is associated with prolonged mechanical ventilation (MV). We investigated the impact of selective decontamination of the digestive tract (SDD), an intervention that reduces hospital acquired infections in ICU patients, on duration of MV in GBS and neurological outcome at 6 months.

Methods

We performed a retrospective study in mechanically ventilated GBS patients in the Netherlands. We compared patients treated with and without SDD. Main outcomes were duration of MV and the ability to walk independently at 6 months. Statistical comparison was done with logistic and ordinal regression analyses.

Results

We included 124 GBS patients on MV at 2 weeks after first symptoms (SDD, n = 54 and non-SDD, n = 70). The median duration of MV without SDD was 42 days (interquartile range, IQR 25–77 days) versus 29 days with SDD (IQR 17–45 days). Median duration of MV for all included patients was 35 days. The adjusted odds ratio (OR) for duration of MV > 35 days in the SDD versus the non-SDD cohort was 0.37 (95% CI 0.17–0.77). SDD did not affect neurological recovery after 6 months from first symptoms. VAP occurred in 12% (95% CI 2–22%) in the SDD cohort and in 47% (95% CI 35–59%) in the non-SDD cohort.

Conclusions

SDD in mechanically ventilated GBS patients reduced the time on the ventilator, probably by preventing VAP, but did not affect neurological recovery after 6 months.     

Long-term outcome in patients with Guillain–Barré syndrome requiring mechanical ventilation

We aimed to determine long-term disability and quality of life in patients with Guillain–Barré syndrome (GBS) who required mechanical ventilation (MV) in the acute phase. Our retrospective cohort study included 110 GBS patients admitted to an intensive care unit and requiring MV (01/1999–08/2010) in nine German tertiary academic medical centers. Outcome was determined 1 year or longer after hospital admission using the GBS disability scale, Barthel index (BI), EuroQuol-5D (EQ-5D) and Fatigue Severity Scale. Linear/multivariate regression analysis was used to analyze predicting factors for outcome. Mean time to follow up was 52.6 months. Hospital mortality was 5.5 % and long-term mortality 13.6 %. Overall 53.8 % had a favorable outcome (GBS disability score 0–1) and 73.7 % of survivors had no or mild disability (BI 90–100). In the five dimensions of the EQ-5D “mobility”, “self-care”, “usual activities”, “pain” and “anxiety/depression” no impairments were stated by 50.6, 58.4, 36.4, 36.4 and 50.6 % of patients, respectively. A severe fatigue syndrome was present in 30.4 % of patients. Outcome was statistically significantly correlated with age, type of therapy and number of immunoglobulin courses. In GBS-patients requiring MV in the acute phase in-hospital, and long-term mortality are lower than that in previous studies, while long-term quality of life is compromised in a large fraction of patients, foremost by immobility and chronic pain. Efforts towards improved treatment approaches should address autonomic dysfunction to further reduce hospital mortality while improved rehabilitation concepts might ameliorate long-term disability.     

Campylobacter jejuni DNA-binding protein from starved cells in Guillain–Barré syndrome patients

Campylobacter jejuni enteritis is frequently associated with an axonal form of Guillain–Barré syndrome (GBS) and C. jejuni DNA-binding protein from starved cells (C-Dps) induces paranodal myelin detachment and axonal degeneration through binding with sulfatide in vivo. Here we investigated the invasion of C-Dps into hosts with C. jejuni-related GBS. Our analyses of patient sera found that both C-Dps and anti-C-Dps antibodies were most commonly detected in sera from C. jejuni-related GBS patients (5/27, 14.8% and 15/24, 62.5%; respectively). These findings suggest that C-Dps invades the host and may potentially contribute to the peripheral nerve damage in C. jejuni-related GBS.     

Clinical and electrophysiological features of Guillain–Barré syndrome in Iran

We evaluated the clinical and electrophysiological characteristics of 121 consecutive patients admitted with Guillain–Barré syndrome (GBS) to a tertiary referral hospital in Tehran, Iran, from 1997 to 2007. The mean age of patients was 38.9 (standard deviation 19.7) years. The predominant subtype of GBS was the demyelinating form. Miller Fisher syndrome was present in 3.3% of patients. There was no significant seasonal clustering among the three subtypes, but axonal variants tend to occur in summer. In contrast with other subtypes, the majority of patients with acute motor-sensory axonal neuropathy (AMSAN) were female (72.3%). AMSAN patients also had significantly longer hospitalization time (p = 0.002) and intensive care unit (ICU) admission (p = 0.017), while none of the acute motor axonal neuropathy patients needed ICU admission. Involvement of cranial nerves and symmetry of signs were significantly detected in the demyelinating variant (p = 0.021 and p = 0.040, respectively). The overall mortality was 3.3%.     

Can Subdural Hematoma Be a Trigger for Guillain–Barré Syndrome?

Guillain–Barré syndrome (GBS) is an acute inflammatory polyneuropathy which follows a precipitating event in approximately two thirds of cases. Although its pathogenesis is unclear, it is likely to be a consequence of an immune-mediated process. In the literature there are three case reports of GBS following subarachnoid hemorrhage, subdural hematoma, and facial bone fracture after head trauma.The unique feature of our case with GBS after subdural hematoma is the presence of cerebellar symptoms. We believe that GBS results from an aberrant immune response following trauma that somehow mistakenly attacks the nerve tissue of its host, and we discuss the effects of the trauma of head injury on cellular and humoral immunities and the absence of antiganglioside antibody (anti-GD1b IgG, which is accused of ataxia and cerebellar symptoms) in this case report.     

Relapsing Guillain Barré Syndrome and nephrotic syndrome secondary to focal segmental glomerulosclerosis

A 49-year-old man developed simultaneously a Guillain Barré Syndrome (GBS) and a nephrotic syndrome (NS). The patient relapsed twice, despite treatment with intravenous immunoglobulins (IVIg) after a full or partial recovery, and became resistant to IVIg. Renal biopsy revealed focal segmental glomerulosclerosis (FSGS). He responded to plasmapheresis and corticosteroids with simultaneous recovery of his GBS and NS, suggesting a common pathogenesis of the two conditions.     

Contact heat-evoked potentials as a useful means in patients with Guillain–Barré syndrome

Few objective methods have been utilized to identify the small myelinated fiber impairment causing neuropathic pain in Guillain–Barré syndrome (GBS). In this study, contact heat-evoked potentials (CHEPs) were applied to study the nociceptive pathway in GBS. Sixty GBS patients and fifty healthy controls were enrolled. The 60 GBS patients were divided into two subgroups presenting with or without subjective lower limb paresthesia (21/39). CHEPs were recorded at Cz and Pz with a peak thermal stimuli of 47 °C applied to the skin of the leg above the internal malleolus (AIM) and of the waist at the anterior superior iliac spine (ASIS) level. The N2 latency and N2–P2 amplitude of CHEPs were compared. When the skin of the leg AIM was stimulated, the N2 latency was significantly postponed (425.23 ± 28.66 vs. 402.30 ± 19.48 ms, P < 0.05) and the N2–P2 amplitude significantly decreased in GBS patients as compared to controls (32.71 ± 7.49 vs. 42.77 ± 8.71 μV, P < 0.05). Slower nerve conduction velocity was observed in GBS patients (11.84 ± 1.45 vs. 13.28 ± 0.66 ms, P < 0.05). However, no differences in N2 latency or N2–P2 amplitude were detected between the two subgroups of GBS patients with or without subjective lower limb paresthesia (P all >0.05). Moreover, there were no differences in N2 latency and N2–P2 amplitude among different groups when the waist was stimulated at the ASIS level. Our study suggested that CHEPs could be utilized as an objective and non-invasive tool to detect small myelinated fiber damage in GBS patients, especially for those without subjective paresthesia.     

Vital Capacity Versus Maximal Inspiratory Pressure in Patients with Guillain–Barré Syndrome and Myasthenia Gravis

Background

The objective is to determine whether maximal inspiratory pressure (P _imax) measurement is more sensitive than vital capacity (VC) measurement to detect acute respiratory muscle failure considering a theoretical curvilinear relationship between volume and pressure.

Methods

Review of VC and P _imax of all patients hospitalized in ICU for Guillain?CBarré syndrome (GBS) and myasthenia gravis (MG) exacerbation.

Results

84 consecutive caucasian patients between 19- and 70-years-old hospitalized in intensive care unit from April 2008 to December 2010, for MG exacerbation (44 patients) and GBS (40 patients). The regression curve between VC and P _imax was linear rather than exponential (r?=?0.599, P?>?0.0001). The contingency table demonstrated agreement between VC and P _imax (??^2?=?26.7, P?=?0.0001), with similar number of patients having abnormal P _imax associated to normal VC and normal P _imax associated to abnormal VC (9 (10.7%) vs. 8 (9.5%) respectively). Six of the patients developed an important decrease of VC from normal value to less than 60% of the predicted value and did not present evident curvilinear relationship between VC and P _imax during this follow-up.

Conclusions

Because the regression between VC and P _imax was linear rather than curvilinear, P _imax was not more sensitive than VC for early detection of respiratory muscle failure in patients hospitalized in ICU for GBS and MG exacerbation. Therefore, VC remains well suited to assess acute respiratory muscle failure and P _imax gives poor additional information.     

Preventive and curative effects of cyclophosphamide in an animal model of Guillain Barrè syndrome

The immunosuppressive agent cyclophosphamide (CY) was tested in rat experimental allergic neuritis (EAN), a preclinical model of Guillain Barrè syndrome (GBS). CY prophylaxis (day 0 and 14 post-immunization [p.i.]) effectively prevents clinical and histological signs of EAN and also reduces the cytokine and the NF-kappaB p65 expression in the nervous tissue. When administered therapeutically (day 14th p.i.) to rats with established disease CY only affects the clinical symptoms. Both the prophylactic and therapeutic treatment with CY reduced ex vivo antigen-specific T cell proliferative responses. These results warrant studies with CY in those cases of GBS resistant to conventional therapies.     

Diagnosis of Guillain–Barré syndrome in children and validation of the Brighton criteria

To describe the key diagnostic features of pediatric Guillain–Barré syndrome (GBS) and validate the Brighton criteria. Retrospective cohort study of all children (<18 years) diagnosed with GBS between 1987 and 2013 at Sophia Children’s Hospital, Erasmus MC, Rotterdam. Clinical information was collected and the sensitivity of the Brighton criteria was calculated. 67 children (35 boys) were included, with a median age of 5.0 years [interquartile range (IQR) 3.0–10.0 years]. Bilateral limb weakness was present at hospital admission in 93% of children, and at nadir in all patients. Children presented with tetraparesis in 70% or with paraparesis in 23%. Reduced reflexes in paretic limbs were observed at hospital admission in 82% and during follow-up in all children. The progressive phase lasted median 6 days (IQR 3–8 days) and less than 4 weeks in all children. A monophasic disease course was seen in 97%, including 5 children with a treatment-related fluctuation. Two children had a later relapse at 9 weeks and 19 weeks after onset. 77% of the children showed an elevated protein level in CSF. Nerve conduction studies showed evidence for a poly(radiculo)neuropathy in 91% of the children. 46 children had a complete data set, the sensitivity of the Brighton criteria level 1 was 72% (95% CI 57–84) and 96% (95% CI 85–99) for level 2 and 98% (95% CI 88–100) for level 3. The majority of the pediatric GBS patients presented in this cohort fulfilled the current diagnostic criteria.     

Ein Fall von Polyradiculoneuritis acuta curabilis (Syndrome de Guillain et Barré)

Ohne Zusammenfassung     

Guillain–Barré Syndrome following sepsis after stereotactic aspiration for spontaneous pontine hemorrhage

A 52-year-old female was treated with CT-guided stereotactic aspiration for acute spontaneous pontine hemorrhage. On postoperative day 7, the patient was complicated by Acinetobacter baumannii sepsis. As sepsis was stabilized, she developed flaccid weakness and autonomic dysfunction on postoperative day 21. Investigations including neurophysiological studies and cerebral spinal fluid analysis prompted the diagnosis of acute motor axonal neuropathy, a variant of Guillain-Barré syndrome. Intravenous administration of immunoglobulin resolved her potentially life-threatening autonomic instability. At 1-year follow-up, she was able to stand with significant assistance. Although Guillain-Barré syndrome rarely occurs, clinicians should be alert to the possibility of this potentially life-threatening consequence after cranial surgery with severe respiratory infection.