Guillain–Barré syndrome in children

Guillain–Barré syndrome (GBS) is one of the reasons of acute polyneuropathy causing severe morbidity and mortality. Forty-six patients with GBS were included in our study. Clinical, laboratory, electrophysiological and prognostic features of the patients were evaluated retrospectively. Patients were divided into two groups. Group A consisted of children who attained a full recovery within 2 months from onset of the disease; group B consisted of children who experienced complete or partial recovery beyond 2 months from onset of the disease. Acute inflammatory demyelinating polyradiculoneuropathy was found in 56.5% of patients and axonal form in 43.5% patients. Antecedent events were found in 28 (60.9%) patients. Five patients (10.8%) needed mechanical ventilation and one patient (2.1%) died. Poor outcome was related with clinic stage and electrophysiological subtypes (axonal form). In our study, poor prognostic factors were related with clinic stage and electrophysiological subtypes (axonal form).     

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%.     

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.     

Guillain–Barré syndrome in Denmark: a population-based study on epidemiology,diagnosis and clinical severity

Journal of Neurology - To describe the epidemiology and clinical heterogeneity of Guillain–Barré syndrome (GBS) in Denmark and to compare a population-based cohort to prospectively...     

Axonal variants of Guillain–Barré syndrome: an update

Journal of Neurology - Axonal variants of Guillain–Barré syndrome (GBS) mainly include acute motor axonal neuropathy, acute motor and sensory axonal neuropathy, and...     

Admission sodium level and prognosis in adult Guillain–Barré syndrome

Purpose: Guillain–Barré syndrome (GBS) varies in severity and outcome. Hyponatremia predicts poor outcome but previous studies have used divergent methodology and (pseudo)hyponatremia caused by intravenous immunoglobulin administration may confound analysis. We studied if the plasma sodium level at admission was associated with GBS outcome. Methods: All 69 patients at least 16 years of age treated for GBS in Turku University Hospital in 2004–2013 were included in the study. Clinical information was obtained from patient charts. Results: Women had lower sodium levels at admission (138; IQR 135, 140) compared to men (140; IQR 138, 142; p = 0.0116) but no association of sodium levels with demographics, pre-hospital variables or basic GBS characteristics was found. Multivariate analyses showed lower admission sodium levels to be associated with worse functional status at one year from disease onset (OR 1.37; 95% CI 1.07–1.76; p = 0.0136) and probability of being discharged to another care facility from the hospital (OR 1.31; 95% CI 1.05–1.64; p = 0.0180) but not associated with need of intensive care unit care (p = 0.09) or mechanical ventilation (p = 0.45), length of hospital stay (p =0.48) or functional status at six months (p = 0.07). Conclusions: Low plasma sodium level at admission is associated with a more severe disease course and a worse outcome in GBS independently of previously identified prognostic factors. Hyponatremia does not, however, appear to be caused by disease-specific factors.     

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.     

Childhood Guillain–Barré syndrome subtypes in northern India

This prospective cross-sectional study was conducted at a tertiary care research centre in North India to describe the frequency and clinical characteristics of subtypes of childhood Guillain–Barré syndrome. Among the 68 children enrolled, 65 were finally diagnosed with Guillain–Barré syndrome (median age, 60 months); 45 (69%) were boys. The most common subtype was acute motor axonal neuropathy in 27 patients (41.5%, 95% confidence interval [CI] 29–54), followed by acute inflammatory demyelinating polyneuropathy in 15 (23%, 95% CI 13.5–35), and acute motor sensory axonal neuropathy in three (4.6%, 95% CI 1–13). Twelve patients (18.5%, 95% CI 10–30) had inexcitable nerves, and eight (12.4%, 95% CI 5.5–23) were unclassifiable. Those with acute inflammatory demyelinating polyneuropathy were more likely to have had a preceding upper respiratory tract infection. The acute motor axonal neuropathy subtype peaked in incidence during the winter and monsoon months.     

The clinical significance of neutrophil-to-lymphocyte ratio and monocyte-to-lymphocyte ratio in Guillain–Barré syndrome

Purpose/Aim of the study: Guillain Barré syndrome (GBS) is a severe peripheral nervous disease that leads to muscle weakness and areflexia. We now commonly accept a synthesis that inflammation and immunity play key role in GBS pathogenesis. Many studies pointed out that neutrophil-to-lymphocyte ratio (NLR) and monocyte-to-lymphocyte ratio (MLR) are novel promising markers of inflammation or immunity. Our study aimed to evaluate whether the NLR and the MLR were associated to GBS or not.

Materials and Methods: We measured blood cell count in 334 individuals including 117 GBS and 217 healthy controls.

Results: Our findings demonstrated that the GBS patients had higher levels of NLR and MLR than the healthy controls. The severe group also had higher levels of NLR and MLR compared to the mild group. We took the method of receiver-operating characteristic curve to find out the cut-off value of NLR for GBS occurrence and severity; it was 2.295 and 3.05, respectively. The cut-off values of MLR for GBS incidence and severity were the same, it was 0.235.

Conclusion: In the setting of GBS, the NLR and MLR were significantly increased and they may be pathophysiologically and clinically relevant in GBS. The NLR and MLR would be new biomarkers of medical application.     

Long term disability and social status change after Guillain–Barré syndrome

Objective Even if the majority of patients with Guillain–Barré syndrome (GBS) have a favourable functional outcome some residual motor and sensory signs and symptoms may remain. The aim of this study was to evaluate the long–term effect of GBS on daily life,working activities, hobbies and social status and the presence of residual symptoms. Patients and methods Seventy patients with GBS enrolled in a case–control study were examined. Information on signs or symptoms during the acute phase of the disease was retrieved from medical records and an ad–hoc questionnaire administered during hospitalization. Patients were interviewed by phone 3 to 5 years after disease onset about residual symptoms and changes in daily living. Disability and handicap were assessed using the Hughes, Rankin and Rotterdam 9–items scale. Results At follow–up 45 patients (64 %) made a complete functional recovery; 19 patients (27%) had some minor limitations in daily life although they were able to perform all their activities independently while 6 (9 %) needed aid for some hours or continuously during the day. Nineteen patients (27 %) had, however, to make substantial changes in their job, hobbies or social activities. There was no significant correlation between clinical and laboratory features during the acute phase of GBS and outcome. Conclusions Althoough over 90% of our GBS patients had a more or less complete functional recovery, almost 30% of them had to make substantial changes in daily life. These findings indicate that GBS still has a significant impact on patients' life which may go beyond their residual disability or impairment. Treatment of GBS should not be only aimed at improving patients' disability but also at limiting the impact of the disease on their social life.     

Bilateral abducens palsy in a case of cytomegalovirus-associated Guillain–Barré syndrome

Ophthalmoparesis in cytomegalovirus (CMV)-associated Guillain–Barré syndrome (GBS) is rare. We treated a 37-year-old woman with CMV-GBS who presented with an acute onset of generalized weakness and numbness in the extremities, followed by facial diplegia, which led to mechanical ventilation. She had increased IgM and IgG-type antibodies against CMV in the serum and increased IgM-type serum anti-GM2 ganglioside antibody was also noted, whereas anti-GQ1b ganglioside antibody was not found. She then developed the syndrome of inappropriate secretion of antidiuretic hormone (SIADH). After recovery of consciousness due to SIADH, she exhibited bilateral abducens palsy, together with the recurrence of limb weakness and facial diplegia. Her neurological signs gradually recovered after high-dose intravenous administrations of immunoglobulin. CMV infection should be listed in the differential diagnosis of GBS patients who present with ophthalmoparesis.