Incidence of Guillain‐Barré syndrome in Chile: a population‐based study

The Guillain‐Barré syndrome (GBS) incidence rate (IR) varies between 0.16 and 3.00 cases per 100,000 inhabitants. Little data exist on the epidemiology of GBS in Latin American countries. Our objective was to describe GBS epidemiology based on a national database in a Latin American country and to contribute to the global map of GBS epidemiology. This was a retrospective study that included all reported GBS cases in Chile between 2001 and 2012. Gender, age, seasonal occurrence, and geographical distribution were analyzed. A total of 4,158 GBS cases were identified from 19,513,655 registries. The mean age was 37 ± 24 years, and 59% of patients were male (male to female ratio of 1.5 : 1). Gender IR was 2.53/100,000 for males and 1.68/100,000 for females. The overall standardized IR was 2.1/100,000, although this varied between 1.61/100,000 (2001) and 2.35/100,000 (2010). The seasonal distribution was as follows: autumn 22%; winter 25%; spring 27%; and summer 26%. The geographical IR were as follows: far North 1.49/100,000; North 1.94/100,000; Central 1.97/100,000; South 3.18/100,000; and far South 2.78/100,000. The reported IR of GBS in Chile was similar to other studies based on national databases. In Chile, IR was greater in men and in the south.     

Update on Guillain‐Barré syndrome

Understanding of Guillain‐Barré syndrome (GBS) has progressed substantially since the seminal 1916 report by Guillain et al. Although Guillain, Barré, and Strohl summarised the syndrome based on observations of two French infantrymen, 2012 saw the beginning of an ambitious collaborative study designed to collect detailed data from at least 1,000 patients worldwide (IGOS, www.gbsstudies.org/about‐igos ). Progress has been made in many areas even since GBS was last reviewed in this journal in 2009. GBS subsequently received prominent attention in light of concerns regarding H1N1 influenza vaccinations, and several large‐scale surveillance studies resulted. Despite these developments, and promising pre‐clinical studies, disease‐modifying therapies for GBS have not substantially altered since intravenous immunoglobulin was introduced over 20 years ago. In other areas, management has improved. Antibiotic prophylaxis in ventilated patients reduces respiratory tract infection, thromboprophylaxis has reduced the risk of venous thromboembolism, and there is increasing awareness of the benefit of high‐intensity rehabilitation. This article highlights some of the interesting and thought‐provoking developments of the last 3 years, and is based on a plenary lecture given at the 2012 Peripheral Nerve Society (PNS) meeting.     

Clinico‐electrophysiological findings and prognosis of Guillain‐Barré syndrome – 10 years’ experience

Soysal A, Aysal F, Calıskan B, Dogan Ak P, Mutluay B, Sakallı N, Baybas S, Arpacı B. Clinico‐electrophysiological findings and prognosis of Guillain‐Barré syndrome – 10 years’ experience.
Acta Neurol Scand: 2011: 123: 181–186.
© 2010 John Wiley & Sons A/S. Objective – To assess correlation between the prognosis and epidemiological, clinical, laboratory, electrophysiological findings in patients with Guillain‐Barré syndrome (GBS). Methods – We reviewed the medical records of 104 GBS patients who were hospitalized and followed up at our outpatient clinic during October 1997–November 2007. Results – Guillain‐Barré syndrome patients were followed up with a median period of 232 days. Full recovery or minor deficits were observed in 41% of patients in the first month, 71% in the third month, 86% in the sixth month and 92% in the first year. We found that there was a correlation between Medical Research Council (MRC) sum scores at admission, clinical subtypes, respiratory distress, interference pattern and prognosis. Conclusions – Demographic, clinical and electrophysiological findings of our GBS cases were highly similar to those of the previous reports. Two of our cases were presented with preceding tuberculosis infection, which was not reported before in the literature.     

Clinical and epidemiological features of Guillain‐Barré syndrome in the Western Balkans

The aim of this study was to define features of Guillain‐Barré syndrome in a large cohort of patients from three Western Balkans countries. Data from adult Guillain‐Barré syndrome (GBS) cases from 2009 to 2013 were retrospectively obtained from all tertiary health care centers. During the 5‐year period, 327 new cases of GBS were identified with a male to female ratio of 1.7 : 1. The most common GBS variants were demyelinating (65%) and axonal (12%). At nadir 45% of patients were chair‐bound, confined to bed, or required assisted ventilation, while 5% died. The crude incidence of GBS in Serbia and Montenegro was 0.93 per 100,000 population, and age‐adjusted incidence according to the world standard population was 0.86 per 100,000. Incidence was particularly high in 50‐ to 80‐year‐old men. Statistically significant seasonal variations of GBS were not observed. This study of patients with GBS in the Western Balkans allows us to prepare the health system better and to improve the management of patients. This study also opens opportunities for international collaboration and for taking part in the multinational studies on GBS.     

Guillain-Barré syndrome in immunocompromised patients: A report of three patients and review of the literature

Both humoral and cell-mediated autoimmune mechanisms have been implicated in the pathogenesis of Guillain-Barré syndrome (GBS). Therefore, its occurrence in severely immunocompromised patients is not expected. We identified 3 severely immunocompromised patients who developed GBS. Two of the 3 patients had acquired immunodeficiency syndrome with CD4 counts of 5 and 4 cells/mm³, respectively. One post-cardiac transplant patient was taking azathioprine and cyclosporine at the time of onset of GBS. In all 3 patients, immunocompromise was induced by infectious or chemotherapeutic agents which preferentially suppress T-lymphocyte responses. All 3 had severe lymphocytopenia and incomplete recovery. We conclude that GBS can occur in patients with severe t-cell suppression. Although no conclusion regarding prognosis can be drawn from our small group of patients, their incomplete recovery is consistent with the idea that T-cells are important for recovery. © 1997 John Wiley & Sons, Inc. Muscle Nerve 20: 1002–1007, 1997     

What's new in Guillain‐Barré syndrome in 2007–2008?*

The years 2007 and early 2008 have been an exciting time for Guillain‐Barré syndrome (GBS) research. Epidemiological studies have shown that the incidence of GBS remains stable at about 2/100,000 per year but that there have been changes in hospitalization use, likely due to the widespread availability of IVIg. Research into mechanisms has shown the importance of single amino acids in Campylobacter jejuni and the importance of ganglioside conformation. In a murine model of anti‐ganglioside antibody‐mediated neuropathy, Eculizumab was effective in reversing clinical disease and preventing pathology. This suggests trials of Eculizumab in GBS should be considered. Unfortunately, there are no new randomized controlled trials in GBS to report although the unmet need is great.     

Health‐related quality of life in Guillain‐Barré syndrome patients: a systematic review

Guillain‐Barré syndrome (GBS) encompasses a broad spectrum of health‐related quality of life (HRQL) determinants, including mobility, fatigue, pain, and depression. We systematically reviewed the literature on functional outcome domains in which GBS patients experience limitations in the short and long terms and evaluated determinants of HRQL in GBS patients. MEDLINE and EMBASE were systematically searched by two independent reviewers for articles covering HRQL data of GBS patients. Of 730 abstracts screened, 17 articles covering data of 14 studies matched the selection criteria. The included articles showed that many GBS patients experienced physical limitations, even years after the acute phase of the disease, while results were inconsistent for perceived levels of pain, fatigue, and general mental well‐being. Only three papers covered HRQL assessments at more than one time point, generally showing large improvements in HRQL in the first year after GBS onset, but not thereafter. We appraised the methodological quality of included studies using a 13‐item checklist; none of the articles fulfilled all items and only seven articles presented data on correlations between HRQL and determinants. In conclusion, the majority of studies on HRQL in GBS patients are cross‐sectional and of low methodological quality. This paper provides guidance for much needed high‐quality studies on patterns of patient‐perceived recovery after GBS onset.     

Long‐term outcome of Guillain‐Barré syndrome in children

The objective of this study is to determine the long‐term outcome and consequences of Guillain‐Barré syndrome (GBS) in children. This is an observational cross‐sectional cohort study of children diagnosed with GBS (0–18 years old) at the Sophia Children's Hospital in Rotterdam from 1987 to 2009. All patients were invited for a structured interview, questionnaires, and full neurologic exam to record their current clinical condition focused on complaints and symptoms, neurological deficits, disabilities, behavior, and quality of life. Thirty‐seven patients participated, 23 were now adults, with a median age of 20 years (range 4–39 years) and a median follow‐up time of 11 years (range 1–22 years). Residual complaints were reported by 24 (65%) patients, including paresthesias (38%), unsteadiness of gait in the dark (37%), painful hands or feet (24%), and severe fatigue (22%). Four patients had severe neurological deficits, including facial diplegia and limb weakness. Two patients had had a recurrence of GBS. In 10 patients (26%), GBS had a negative impact on their school career. Questionnaires identified a wide range of behavioral problems. Quality of life was below normal on the subscale vitality, and above normal on the subscales social functioning and positive emotions in the adult group. Most children show good recovery of neurological deficits after GBS, but many have persisting long‐term residual complaints and symptoms that may lead to psychosocial problems interfering with participation in daily life.     

Limb motor nerve dysfunction in Miller Fisher syndrome

Typical Miller Fisher syndrome (MFS) lacks limb muscle weakness, but some patients may unpredictably progress to severe Guillain‐Barré syndrome. The compound muscle action potential (CMAP) scan is a recently developed non‐invasive, painless, and reproducible method for detecting early changes in motor nerve excitability. This technique was used to monitor subclinical limb motor nerve dysfunction during disease course in typical MFS. Three Miller Fisher patients with preserved limb muscle strength and normal routine nerve conduction studies were included. Frequent serial CMAP scanning of the median nerve was performed during acute phase and follow‐up and was related to clinical course and outcome. All patients showed an abnormal increase in the range of stimulus intensities at the day of hospital admission, indicating reduced motor nerve excitability already at the earliest stage of disease. Median nerve dysfunction progressed in parallel or even before clinical deterioration, and improved with clinical recovery. Our study shows that typical MFS is a more general neuropathy, affecting peripheral motor nerves even in patients with preserved limb strength and conduction velocity. CMAP scanning is a sensitive technique for early detection of subclinical motor nerve dysfunction and for monitoring disease activity in immune‐mediated neuropathies.