Cardiac arrhythmias in Guillain-Barre syndrome. An overview of the diagnosis of a rare but potentially life-threatening complication

Autonomic neuropathy is an important complication of Guillain-Barré syndrome (GBS). In particular, over-reactivity of vagal function may lead to serious cardiac disturbances ranging from bradycardia to asystole. Early recognition of these events is essential for appropriate intervention such as the insertion of a cardiac pacemaker. Recent studies indicate that serious bradyarrhythmias could be observed in severely and even less severely affected patients who were still able to walk more than 5 meters. Conventional measures such as the presence of tachycardia, heart rate and blood pressure fluctuations, or short-term heart rate variability and standardized autonomic function tests were not useful in predicting serious bradyarrhythmias. However, abnormal sensitivity to eyeball pressure testing correctly identified two of three patients who needed cardiac pacing or cardiopulmonary resuscitation due to heart arrest; eight of ten patients without bradyarrhythmic events showed normal responses. New methods such as the 24-hour heart rate power spectrum may yield sensitive and specific markers for assessing the risk of impending and potentially life-threatening arrhythmias in patients with GBS.     

Autonomic involvement in Guillain-Barré syndrome]

Guillain-Barré syndrome (GBS) is an acute self-limited motor-dominant neuropathy, in which autonomic nervous system is frequently involved. Cardiovascular complications, such as hypertension, hypotension, bradyarrhythmias, and tachyarrythmias, are particularly important because they are sometimes life-threatening. Antiganglioside antibodies are frequently present in the acute-phase sera of GBS. They are considered to be useful for diagnosis and to be involved in the pathogenetic mechanisms. No association between autonomic dysfunction and antiganglioside antibody however has been reported. Recently, we performed the quantitative sudomotor axon reflex test (QSART) in GBS patients and found that patients with high scores in QSART had anti-GQ1b IgG antibodies. It indicates the association between postganglionic sudomotor dysfunction and anti-GQ1b antibodies. Further investigation on larger number of patients is needed to clarify the role of antiganglioside antibodies in the development of autonomic dysfunction.     

Neuropsychiatric debut as a presentation of Guillain-Barré Syndrome: An atypical clinical case and literature review

IntroductionGuillain Barré Syndrome (GBS) is an acute immune-mediated polyneuropathy most frequently presenting two to four weeks after an acute mild-moderately severe infection as progressive muscular weakness of the lower limbs extending proximally with dysreflexia and autonomic dysfunction. While GBS is typically believed to be isolated to the Peripheral Nervous System, Central Nervous System (CNS) and psychiatric manifestations as a sequela of the disease have been described in different imaging and clinical studies. Many variants of presentation of GBS have been recognized, however a case presenting with primarily psychiatric and autonomic dysfunction preceding muscle weakness has not been cited in the literatures to date.Case presentationWe describe a 24-year-old previously healthy male presenting with behavioral symptoms including depression, anxiety, and amnesia, and autonomic dysfunction which preceded muscle weakness by two weeks. CNS imaging and blood work results were unremarkable. GBS was confirmed upon cerebral spinal fluid analysis remarkable for an important cytoalbuminologic dissociation and markedly elevated protein concentration. The patient responded well to five cycles of inpatient plasmapheresis and short-term selective serotonin reuptake inhibitor treatment with complete recovery of both neurological and behavioral symptoms.ConclusionThough GBS is typically considered a peripheral neuropathy, evidence for CNS involvement exists; GBS should be considered within the differential diagnosis, and neurological features should be monitored, in a patient with new onset unclear psychiatric and CNS symptoms.     

Guillain-Barré syndrome: diagnostic criteria, epidemiology, clinical course and prognosis

Thirty-four patients were identified with Guillain-Barré syndrome (GBS) on review of 266 neuropathy cases admitted to a Copenhagen county hospital from June 1977 to January 1984. The age-adjusted incidence rate of GBS is 2.0 x 10(-5) years-1. The natural history of the disease, antecedent events, symptoms and signs, autonomic dysfunction, sequelae, CSF findings and mortality are described. Six cancer patients with GBS differed significantly from the non-cancer patients in a more protracted disease course and failure to improve. The National Institute of Neurological and Communicative Disorders and Stroke (NINCDS) criteria for the diagnosis of GBS are discussed, and it is concluded that the criteria, although useful in comparative studies, are too restrictive when used in clinical practice.     

Serial nerve conduction studies provide insight into the pathophysiology of Guillain-Barré and Fisher syndromes

The electrodiagnosis of Guillain-Barré syndrome (GBS) can be broadly divided into acute inflammatory demyelinating polyneuropathy (AIDP) and acute motor axonal neuropathy (AMAN). Fisher syndrome (FS) is a variant of GBS, although the underlying neuropathy of FS has yet to be established. Serial nerve conduction studies (NCS) can provide further insight into the likely pathophysiology by further subtyping of GBS and FS. We present a patient with an initial diagnosis of AIDP in whom repeated NCS revealed the AMAN variant. This led us to investigate serial NCS in five patients with GBS, FS and FS/GBS overlap presenting over a period of a year. Three patients with AIDP showed a gradual increase in distal motor latencies during the acute phase of illness. NCS of two patients with FS and FS/GBS overlap showed no demyelinating features suggesting underlying axonal neuropathy in this group of patients. The importance of serial NCS in establishing the underlying pattern of neuropathy in GBS and FS is further emphasized in this study. Larger studies incorporating serial NCS are required to confirm the observations seen in our case series especially when pathological studies are often not justified in this group of patients.     

Changing cerebral blood flow velocity by transcranial Doppler during head up tilt in patients with diabetes mellitus

ObjectiveDiabetes mellitus is an independent risk factor for poor prognosis in patients with ischemic stroke. It is known that diabetes mellitus directly affects cerebral vasculature as a secondary, long-term complication of cerebral circulation, and causes cerebral blood flow abnormalities. The abnormalities of cerebral autoregulation also poorly affects the prognosis of ischemic stroke. In this study, we aimed to show the cerebral autoregulation with transcranial Doppler (TCD) ultrasound in diabetic patients with autonomic nervous system abnormalities, determined with electrophysiological studies.Material and methodTwenty healthy controls and 39 patients, who had at least 2 years of diabetes mellitus, were evaluated (age ranges: 42–75 years). The patients were divided into two groups according to sympathetic skin response and R–R interval variation studies: (1) patients with autonomic neuropathy; (2) patients without autonomic neuropathy. Blood flow velocities were measured during supine position and after the patients were raised upright position on head up tilt table. Arterial blood pressures and heart rates were also evaluated.ResultsMean blood flow velocities of diabetic patients with autonomic neuropathy were found more decreased at 90 s after the patients were raised upright position.DiscussionAutonomic neuropathy due to diabetes mellitus affects cerebral autoregulation, and by this way cerebral perfusion loses protection against hemodynamical changes.     

Cardiovascular autonomic dysfunction in Guillain-Barré syndrome. Therapeutic implications of Swan-Ganz monitoring

Guillain-Barré syndrome (GBS) is an acute inflammatory polyneuropathy that may lead to quadriparesis, ventilatory failure, and autonomic dysfunction. While significant mortality due to ventilatory failure has been associated with this syndrome, this complication can now be readily managed. However, an estimated mortality of 5% to 20% remains attributable to medical complications (pulmonary embolus, sepsis) and to acute cardiovascular collapse due to autonomic failure. In this report, detailed sequential changes in hemodynamic parameters, as measured by Swan-Ganz catheter, associated with severe autonomic cardiovascular instability in a patient with GBS are described. Knowledge of changes in these hemodynamic parameters led to optimal therapy. Long-term Swan-Ganz monitoring in an intensive care setting may dramatically benefit the critically ill patient with GBS and cardiovascular autonomic dysfunction, and may help to eliminate the residual morbidity and mortality associated with this disease.     

Diabetic autonomic neuropathy

Diabetic autonomic neuropathy is the most common and troublesome complication of diabetes mellitus. Although involvement of the autonomic nervous system is generally diffuse, symptoms may be confined to a single target organ or organ system. Complications of diabetic autonomic neuropathy contribute greatly to the morbidity, mortality, and reduced quality of life of the person with diabetes and are the major source of increased costs of caring for the diabetic patient. Factors in the pathogenesis of these complications are altered metabolism, vascular insufficiency, loss of growth factor trophism, and autoimmune destruction of nerves in a visceral and cutaneous distribution. The clinical manifestations and the complications of diabetic autonomic neuropathy are reviewed. Future therapeutic strategies that are developed from a better understanding of the pathogenetic processes underlying this disorder can be directed at the cause rather than the manifestations. There are studies in progress that suggest that autonomic nerves can be induced to regenerate, and the future for patients with diabetic autonomic neuropathy is brighter.     

Severe dysautonomic onset of Guillain-Barré syndrome with good recovery. A clinical and autonomic follow-up study

A 39 year old man with acute panautonomic and mild somatic neuropathy had severe postural hypotension 1 week after onset. Porphyric neuropathy was excluded. The final diagnosis was Guillain-Barré syndrome (GBS). After 2 months he began to recover progressively and after 9 months he presented asymptomatic postural hypotension. We consider the hypothesis of a spectrum of clinico-pathological entities at one end of which lies GBS with autonomic signs and at the other acute pure dysautonomia. The site of the autonomic lesion, might have been in post-ganglionic sympathetic fibers and vagus nerve.     

Abnormal cough reflex in canine acrylamide neuropathy

Coughing in response to irritation of the airways is a fundamental protective reflex that is dependent on rapidly adapting bronchopulmonary receptors and their vagal afferent fibers; reflex airway constriction, which is effected by vagomotor efferent fibers, usually accompanies coughing. Although dysfunction of vagally mediated cardiovascular and gastrointestinal reflexes is a well-documented complication of autonomic neuropathy, to date there have been no studies of the effect of peripheral autonomic failure on the cough reflex. In the study reported here, we examined the effect of acrylamide-induced neuropathy, a distal axonopathy, on the ventilatory and tracheomotor components of the cough reflex in conscious dogs. There was a reduction in the cough reflex in response to mechanical irritation of the large airways in the preclinical phase of the neuropathy, and the cough reflex was virtually abolished when the dogs had moderate neuropathy. Following withdrawal of the neurotoxin, there was a substantial recovery of the cough reflex in surviving animals. It is possible that the cough reflex may be reduced in patients with vagal neuropathy and that this might compromise protection of the airway.     

First pathological report of a de novo CD5‐positive diffuse large B‐cell lymphoma patient presenting with Guillain–Barré syndrome‐like neuropathy due to neurolymphomatosis

Peripheral neuropathy occurs in approximately 5% of the patients with lymphoma. Two major causes of peripheral neuropathy associated with lymphoma are neurolymphomatosis and paraneoplastic neuropathy such as demyelinating neuropathy. The differential diagnosis between neurolymphomatosis and demyelinating neuropathy is difficult, because electrophysiological findings suggestive of demyelination are frequently observed even in patients with neurolymphomatosis. Here, we report a patient with de novo CD5‐positive diffuse large B‐cell lymphoma (DLBCL) who presented with Guillain–Barré syndrome (GBS)‐like neuropathy. Demyelination due to paraneoplastic neuropathy was clinically suspected. However, autopsy demonstrated that the cause of the neuropathy was neurolymphomatosis. Clinical courses of neurolymphomatosis vary and neurolymphomatosis cases presenting with GBS‐like neuropathy are reported. In addition, DLBCL is the most frequent histological type of malignant lymphoma that develops neurolymphomatosis. Furthermore, “CD5‐positive” DLBCL may tend to develop neurolymphomatosis. If a patient with “CD5‐positive” DLBCL develops peripheral neuropathy, neurolymphomatosis should be considered and imaging studies performed and, if possible, nerve tissue biopsy, regardless of clinical symptoms of the neuropathy. To our knowledge, this is the first report of a patient with de novo CD5‐positive DLBCL with neurolymphomatosis who presented with GBS‐like neuropathy.     

Ocular neuropathy in peripheral neuropathies

Ocular movements and coordination require complex and integrated functions of somatic and autonomic nervous systems. Neurological disorders affecting these nervous systems may cause ocular dysfunction involving extraocular muscles and pupils. In this article, the prevalence, clinical presentations, and management of ocular neuropathy related to certain peripheral neuropathies, including diabetic neuropathy, Guillain–Barré syndrome (GBS), chronic inflammatory neuropathies, human immunodeficiency virus (HIV)‐associated neuropathy, and hereditary neuropathies, are examined in detail. Muscle Nerve 46: 681–686, 2012     

Guillain‐Barré syndrome: subtypes and predictors of outcome from India

There is a paucity of large studies evaluating the subtypes of Guillain‐Barré syndrome (GBS) and their outcome from Southeast Asia. We report cliniconeurophysiological subtypes of GBS and their correlation with triggering events and 3‐month outcome from northern India. Three hundred and twenty eight consecutive patients with GBS were clinically evaluated, including their triggers, severity, autonomic involvement, cranial nerve palsy, and respiratory paralysis. Nerve conduction study (NCS) was repeated at 3 weeks if the initial study was normal. They were categorized into acute inflammatory demyelinating polyradiculoneuropathy (AIDP), acute motor axonal neuropathy (AMAN), acute motor sensory axonal neuropathy (AMSAN), inexcitable motor nerve, and equivocal. Clinically, 204 (62.2%) patients had pure motor, 106 (32.3%) motor sensory, 16 (4.9%) Miller Fisher syndrome, and 2 (0.6%) pure sensory GBS. Based on NCS, 242 (73.8%) had AIDP, 44 (13.4%) AMAN, 15 (4.6%) AMSAN, 8 (2.4%) inexcitable motor nerves, and 27 (8.2%) equivocal GBS. AIDP patients were older, more common in summer, had lesser peak disability, and better outcome compared to those with AMAN. Eleven (3.4%) patients died and 48 (14.6%) had poor outcome at 3 months. The poor outcome was related to severity, dysautonomia, and inexcitable motor nerves. AIDP is the commonest variant of GBS in our study and has better outcome compared to AMAN.     

Inspiratory muscle weakness is associated with autonomic cardiovascular dysfunction in patients with type 2 diabetes mellitus

Introduction  

Diabetic autonomic neuropathy is a complication of diabetes mellitus (DM) that can cause cardiovascular and respiratory abnormalities. The association between respiratory muscle weakness and autonomic cardiovascular neuropathy has not yet been studied. The aims of the present study were to assess respiratory muscle strength, pulmonary function, and heart rate (HR) variability in diabetic patients with and without diabetic autonomic neuropathy.     

Factors predicting poor outcome in patients with fulminant Guillaine-Barré syndrome

This paper describes three patients with acute fulminant Guillain-Barrι Syndrome (GBS) with electrophysiologically inexcitable peripheral nerves not responding to two courses of intravenous immunoglobulin. Their clinical profile is compared with two other GBS patients having similar severity of disease but with demyelinative features, managed similarly during the same period. Patients who failed to respond were elderly with a mean age of 60 years, had prodromal diarrhea, rapid progression of muscle weakness requiring mechanical ventilation within 24 hours, dense weakness of all four limbs with cardiovascular autonomic symptoms and inexcitable peripheral nerves. The remaining two who recovered well were relatively younger with a mean age of 50 years, had no prodromal diarrhea, required ventilatory support by fourth day of illness, no cardiovascular autonomic symptoms and demyelinative neuropathy.     

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.     

Association between acute motor axonal neuropathy and ophthalmoplegia during pregnancy

Different subtypes producing the clinical picture of Guillain-Barré syndrome (GBS) and overlapping forms of GBS subtypes have been described. GBS as a complication in pregnancy has been reported rarely. The present report describes the clinical, electrophysiological and prognostic features of a pregnant woman with overlapping forms of GBS subtypes, acute motor axonal neuropathy and ophthalmoplegia.     

Severe dysautonomic onset of Guillain-Barré syndrome with good recovery. A clinical and autonomic follow-up study

A 39 year old man with acute panautonomic and mild somatic neuropathy had severe postural hypotension 1 week after onset. Porphyric neuropathy was excluded. The final diagnosis was Guillain-Barré syndrome (GBS). After 2 months he began to recover progressively and after 9 months he presented asymptomatic postural hypotension. We consider the hypothesis of a spectrum of clinico-pathological entities at one end of which lies GBS with autonomic signs and at the other acute pure dysautonomia. The site of the autonomic lesion, might have been in post-ganglionic sympathetic fibers and vagus nerve.

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Sommario Abbiamo osservato un uomo di 39 anni colpito da una severa neuropatia autonomica acuta associata a una lieve neuropatia somatica. La diagnosi di porfiria fu esclusa e la diagnosi finale è stata di sindrome di Guillain-Barré (GBS). Dopo 2 mesi il paziente cominciò a migliorare e 9 mesi dopo presentava solo una lieve ipotensione ortostatica asintomatica. Viene discussa l'ipotesi di uno spettro di entità clinico-patologiche da un lato del quale risiede la GBS e dall'altro le forme di neuropatia autonomica acuta. Nel nostro caso la sede della lesione autonomica potrebbe essere nelle fibre simpatiche post-gangliari e nel nervo vago.

     

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.     

A longitudinal cardiovascular autonomic function study in mild Guillain-Barré syndrome

To identify subclinical autonomic dysfunction in mild Guillain-Barré syndrome (GBS), a set of autonomic function tests was serially performed for up to 6 months in 5 GBS patients with mild disability at the nadir. Parasympathetic autonomic function tests consisted of Valsalva ratio and R-R interval variation during rest and deep breathing. Sympathetic autonomic function was evaluated by blood pressure responses to sustained handgrip, hand immersion in ice water, and active standing. The results showed that abnormal parasympathetic and sympathetic function was frequently encountered in all 5 patients during the acute stage of the illness. Autonomic dysfunction occurred both in axonal and demyelinating types of GBS. There was a trend of improvement in most autonomic function tests after 3 months, comparable to the recovery of motor function. In conclusion, subclinical autonomic dysfunction was present in mild GBS. It was temporary and would resolve spontaneously.