BiPAP in early guillain-barré syndrome may fail

BACKGROUND: Non-invasive mechanical ventilation (BiPAP) has been introduced for use in neuromuscular respiratory disease such as amyotrophic lateral sclerosis and myasthenia gravis. There is no experience in Guillain-Barré syndrome. METHODS: We describe for the first time the use of BiPAP to assist in the work of breathing in two consecutive patients with progressing Guillain-Barré syndrome (GBS) and marginal pulmonary function. RESULTS: Our initial attempts to use BiPAP in GBS and early neuromuscular respiratory failure were totally unsuccessful. There was marked initial improvement; however, emergency intubation was needed in both patients, one of which became acutely cyanotic. CONCLUSIONS: Until more experience is available, we strongly warn against using BiPAP in deteriorating patients with GBS.     

Autonomic dysfunction in childhood Guillain-Barré syndrome

This investigation correlated incidence and degree of autonomic dysfunction with the degree of motor impairment in children hospitalized with Guillain-Barré syndrome. Motor weakness varies, as does the effect on autonomic function including heart rate, vasomotor stability, sweating, continence, and blood pressure. After Institutional Review Board approval, hospitalized patients with Guillain-Barré syndrome <19 years were included for retrospective chart review. There were 26 patients (12 boys), with a mean age of 11.3 years (range, 6-17 years). The average hospital stay was 10.6 days. Twenty-four (92%) recovered by 2 to 6 months without functional disability. Bradycardia and sweating disturbances were not observed. Hypertension occurred in 18 of 26 (69%) and tachycardia in 20 of 26 (77%) patients. The proportion of children with hypertension and/or tachycardia increased, as did the motor disability grade (P < .043 and P < .018, respectively). Hypertension occurred 9 to 15 days from symptom onset and within 24 to 48 hours of maximum motor disability in 89%. Multiple autonomic disturbances compound the course of childhood Guillain-Barré syndrome.     

Autonomic dysfunction in Guillain-Barré syndrome and multiple sclerosis

This review gives an overview of autonomic dysfunction encountered in Guillain-Barré syndrome (GBS) and multiple sclerosis (MS). In GBS, cardiovascular dysregulation is common and may lead to serious bradyarrhythmias that need to be recognised for the early initiation of appropriate therapy. Although standardised autonomic tests were useful for the diagnosis of autonomic failure, they were not able to indicate vagal over-reactivity. In this regard, eyeball pressure testing may correctly identify patients at risk for impending and potentially life-threatening bradyarrhythmias which may easily be administered at the bedside. In MS, cardiovascular autonomic dysfunction is usually of minor clinical importance. However, orthostatic intolerance may be present in approximately 50% of patients and could easily be detected by routine measurements of heart rate and blood pressure during rest and during standing. More subtle alterations may require more sophisticated methods such as autonomic reflex testing or baroreflex stimulation. Several in vitro, animal and clinical studies provide evidence that there are many interactions between the sympathetic nervous system and the immune system giving rise to the hypothesis that autonomic dysfunction in MS may not only be a consequence of the disease, but may in itself affect the course of MS.     

Guillain-Barré syndrome in children

In industrialized nations with widespread immunization programs, Guillain-Barré syndrome is the most common cause of acute paralytic illness in children and adults. The incidence of the disease has been estimated to range from 0.5 to 1.5 in 100,000 in individuals less than 18 years of age. Approximately 15% of children with Guillain-Barré syndrome develop respiratory failure and require mechanical ventilatory support. Prospective randomized treatment trials in childhood Guillain-Barré syndrome are wanting; however, smaller case series studies using historical controls suggest that both plasmapheresis and administration of human immunoglobulin could be helpful in reducing morbidity in children with Guillain-Barré syndrome. The prognosis for recovery in children is generally excellent, with the majority of children achieving a complete functional recovery within 6 months from the onset of illness. Studies using an animal model of human Guillain-Barré syndrome, experimental allergic neuritis, have expanded our understanding of the pathogenesis of the disease and suggest new directions for exploration in the treatment of this disorder.     

Progress in Guillain-Barré syndrome

This editorial review summarizes and critically analyses reports published in the preceding 18 months on the pathogenesis of Guillain-Barré syndrome, with particular emphasis on the role of ganglioside antibodies, antecedent infections, and the concept of molecular mimicry. It concludes with an appraisal of currently available and proposed therapies.     

Pain in Guillain-Barré syndrome

Evaluation of: Ruts L, Drenthen J, Jongen JL et al. Pain in Guillain-Barré syndrome: a long-term follow-up study. Neurology 75, 1439-1447 (2010). Pain has been recognized as an important symptom of Guillain-Barré syndrome (GBS). The article under review prospectively studied the phenomenon of pain in a cohort of 156 GBS patients for a period of 1 year. It confirmed that pain of significant intensity is relatively common in all subtypes of GBS. It may start before the onset of other symptoms. It correlates with sensory loss, severity of the GBS at its nadir and the presence of diarrhea. In the recovery/chronic stages it correlates with weakness, disability and fatigue. Up to a third of patients have pain at 1 year.     

Pain in Guillain-Barré syndrome

The clinical features of pain were prospectively analyzed in 29 consecutive patients with Guillain-Barré syndrome (GBS). Sixteen (55%) had characteristic pain early in the illness described as similar to the muscular discomfort following exercise ("charley horse"). Pain preceded weakness by one to five days in four patients. The anterior and posterior aspects of the thighs, the buttocks, and the low part of the back were most frequently affected. Pain was frequently worse at night. Specific clinical signs or electrophysiologic abnormalities were not associated with pain, but serum creatine kinase level was elevated in ten of 13 patients with pain and only one of eight without pain. A review of previously reported pathologic material in five patients with GBS failed to disclose a relation between inflammation of dorsal root ganglia and pain. These results suggest that alterations in muscle related to neurogenic changes may cause the typical pain of GBS.     

Seasonal variation of guillain-barré syndrome admission in a large tertiary referral center in southern iran: a 10 year analysis

Introduction: Guillain-Barré syndrome (GBS) is an acute, acquired, monophasic peripheral neuropathy which has become the most common cause of acute flaccid paralysis. This is an epidemiological report on the seasonal and monthly distribution of GBS and certain patient characteristics in Shiraz, Iran. Methods: We extracted data from Namazi hospital records retrospectively in a 10 year period (January 2000 to December 2009), the largest tertiary referral center in the south of Iran. In order to compare the frequency of GBS in different months and seasons we used the Chi Square test. In a separate analysis a comparison was made between two subsets of patients regarding sex, duration of admission, month and season of admission. Results: From 389 cases of GBS, 232 (59.6%) were male and 157 (40.4%) were female. There was seasonal (P=0.004) and monthly (P=0.046) variation. Spring and winter had the most amount of patients, with admissions from the month of February through June inclusive accounting for 50% of all cases. Conclusion: Our study shows that there is significant monthly and seasonal variation in the admission rate of patients with GBS in Shiraz.     

Twenty-four-hour heart rate power spectrum for evaluation of autonomic dysfunction in Guillain-Barré syndrome.

Long-term fluctuations of the heart rate have an important prognostic impact after myocardial infarction, in patients with chronic heart failure and even in elderly subjects. Autonomic dysfunction is a common complication in patients with Guillain-Barré syndrome, and particularly vagally-mediated bradyarrhythmias require early recognition for immediate initiation of appropriate preventive therapy. This study aimed at investigating (1) whether the 24-h heart rate power spectrum (HRPS) could be used as a measure of autonomic dysfunction also in patients with GBS, and (2) whether the slope of the regression line of power on frequency of the 24-h HRPS could indicate potentially fatal bradyarrhythmias in these patients. In 13 patients with GBS, the heart rate was continuously recorded in the intensive care unit during the early stages of the disease, averaged at 1-min intervals and stored for 4 to 82 days. The HRPS (n = 76, 2 to 14 per patient, median 5) was calculated by Fourier analysis of 24-h recordings and logarithmically transformed. The slope was estimated by regression analysis of log(power) on log(frequency) between 10(-4) and 4x10(-3) Hz demonstrating an inverse power law behaviour in all 76 HRPS. The slope of the regression line ranged from -0.66 to -2.18, and was significantly steeper in patients with tachycardia (median -1.51, interquartile range -1.35 to -1.71) than in those with vagal overreactivity (median -1.14. -1.02 to -1.23) who are suspected to be at risk for fatal arrhythmias. Correlation analysis suggested that the slope was moderately associated with the spectral components of 5-min epochs, but not significantly to standard tests of autonomic hypofunction. Patients with and without vagal overreactivity were better discriminated by the 24-h HRPS than by conventional measures of autonomic function. Therefore, the 24-h HRPS may be a useful adjunct to autonomic nervous system testing, and might be a promising tool to predict serious bradyarrhythmias in patients with GBS.     

Guillain-Barré syndrome in northwest Greece

OBJECTIVES: We present the epidemiological and clinical-laboratory features of Guillain-Barré syndrome (GBS) in northwest Greece over a 9.5-year period. MATERIALS AND METHODS: We studied all the patients with GBS who were admitted to our neurology inpatient service from January 1996 to May 2005 and compared them with previously published series. RESULTS: Forty-six patients were hospitalized during this period. The average crude incidence rate was 1.22/100,000 populations per year, and males were more susceptible than females. There was a spring clustering, as 52.17% presented the syndrome during spring. The axonal type of GBS was recorded in 13.04% of the patients. The most frequent presenting symptom was dysesthetic numbness (52.17%). A large number of patients (56.52%) had up to three times the elevation of liver function values that resolved in a few weeks. Most patients had an excellent recovery and no deaths were recorded. CONCLUSIONS: In our series, there was no difference in the incidence rate and subtypes of GBS but there was a significant seasonality with spring clustering. A transient elevation of transaminases of undetermined etiology was noted in more than a half of our patients. Although seven patients (15.21%) had significant neurologic sequelae, no deaths occurred.     

Tracheostomy in Guillain-Barré syndrome.

Specific treatment has been shown to shorten the duration of mechanical ventilation in Guillain-Barré syndrome (GBS) and could obviate the need for tracheostomy in a significant proportion of patients. However, the factors predictive of prolonged ventilation are undetermined, and the timing and use of tracheostomy in patients with GBS have not been systematically studied. The medical records of 60 patients ventilated for GBS were reviewed. Only 13 patients (22%) could be weaned within 3 weeks. Patients ventilated longer were significantly older (P = 0.04), and 21% had underlying pulmonary disease. Median duration of ventilation in patients treated with plasma exchange (n = 31) was not shortened. Fifty-two patients (87%) received a tracheostomy at a median of 9 days after intubation. In this series, where patients with comorbidity were included, tracheostomy was still necessary in the majority of ventilated patients. This procedure can be anticipated in elderly patients and in the presence of preexisting pulmonary disease.     

Physiologic-pathologic correlation in Guillain-Barré syndrome in children

OBJECTIVE: To correlate electrophysiologic patterns with sural nerve pathology in children with Guillain-Barré syndrome (GBS). BACKGROUND: Based on electrophysiologic and pathologic observations, GBS has been divided into demyelinating and axonal subtypes. The acute motor axonal neuropathy (AMAN) involves predominantly motor nerve fibers with a physiologic pattern suggesting axonal damage, whereas the acute inflammatory demyelinating polyneuropathy (AIDP) involves both motor and sensory nerve fibers with a physiologic pattern suggesting demyelination. In this study, we sought to confirm these observations by correlating sural nerve pathology with electrophysiologic findings in GBS patients. METHODS: Biopsies of sural nerve from 29 of 50 prospectively studied GBS patients were obtained. Nerves were examined by light and electron microscopy, and with immunocytochemistry for macrophages, lymphocytes, and complement activation products. RESULTS: Sural nerves from AMAN patients were normal or had only a few (0.1% to 0.7%) degenerating fibers without lymphocytic infiltration or complement activation. One patient with reduced sural sensory nerve action potential classified as acute motor sensory axonal neuropathy (AMSAN) had many degenerating fibers (2.3%) in the sural nerve. All three AIDP patients displayed active demyelination, and in two patients, lymphocytic infiltration and complement activation products were observed on the abaxonal Schwann cell surface. CONCLUSION: Classification of Guillain-Barré syndrome subtypes based on motor conduction studies correlates closely with pathologic changes seen in sural nerve. In acute motor axonal neuropathy cases, the sural nerve is almost completely spared pathologically. In acute inflammatory demyelinating polyneuropathy cases, macrophage-mediated demyelination and lymphocytic infiltration are common in the biopsies of sural nerves.