Cause Of Acute Neuromuscular Paralysis Research Articles

Practical approach to the patient with acute neuromuscular weakness.

Acute neuromuscular paralysis (ANMP) is a clinical syndrome characterized by rapid onset muscle weakness progressing to maximum severity within several days to weeks (less than 4 wk). Bulbar and respiratory muscle weakness may or may not be present. It is a common neurological emergency which requires immediate and careful investigations to determine the etiology because accurate diagnosis has significant impact on therapy and prognosis. Respiratory failure caused by neuromuscular weakness is considered as more critical than lung disease because its development may be insidious or subtle until sudden decompensation leads to life threatening hypoxia. Also, the arterial blood gas finding of severe hypoxemia, hypercapnia, and acidosis may not be apparent until respiratory failure is profound. Hence, the requirement for respiratory assistance should also be intensively and promptly investigated in all patients with neuromuscular disease. The disorder is classified based on the site of defect in motor unit pathway, i.e., anterior horn cells, nerve root, peripheral nerve, neuromuscular junction or muscle. Identification of the cause is primarily based on a good medical history and detailed clinical examination supplemented with neurophysiologic investigations and sometimes few specific laboratory tests. Medical history and neurological examination should be focused on the onset, progression, pattern and severity of muscle weakness as well as cranial nerves testing and tests for autonomic dysfunction. Associated non neurological features like fever, rash or other skin lesions etc. should also be noted. Globally, Guillain-Barré syndrome is the most frequent cause of ANMP and accounts for the majority of cases of respiratory muscles weakness associated with neuromuscular disorders. Newly acquired neuromuscular weakness in intensive care unit patients consist of critical illness polyneuropathy, critical illness myopathy and drug induced neuromuscular weakness which may arise as a consequence of sepsis, multi-organ failure, and exposure to certain medications like intravenous corticosteroids and neuromuscular blocking agents.

PP13.11 – 2904: Severe onset and rapidly progressive acute motor axonal neuropathy in a 2-year-old male

Objective Guillain-Barre syndrome (GBS) is an immunomediated polyneuropathy and an important cause of acute neuromuscular paralysis. AMAN is a relatively aggressive disease comparing to Acute Inflammatory Demyelinating Polyradiculoneuropathy (AIDP) and its onset is usually more severe. Methods Herein we present an AMAN case which was presented with rapidly progressive flaccid paralysis. Results 2 year-old male patient was brought to emergency room because of losing ability to walk. According to his parents' statements, he started to have difficulties while walking 12 hours ago, and he couldn't walk at all in the last hour. Patient lost motor control of his head in the follow up on the sixth hour. Because of this rapid and progressive onset and ascending flaccid tetraplegia patient was taken into the intensive care unit. On the thirty-sixth hour of the observation, patient required intubation and mechanical ventilation, due to cranial neuropathy and respiratory failure. His cerebrospinal fluid protein level was 48 mg/dl (15–45 mg/dl), and no cells were detected. Similarly, stool culture was negative for campylobacter jejuni. GQ1B, GD1B, GD1A, GM1 antibodies were studied and results were negative. Lumbosacral spinal magnetic resonance imaging was performed and contrast enhancement in conus medullaris level was detected. Electromyography was performed and findings were associated to AMAN. Initial treatment regime was 400 mg/kg/day intravenous immunoglobulin for five days. However, because of the ongoing progression, consecutive intravenous immunoglobulin and plasma exchange therapies were given. After four weeks of progression, patient stayed in the plateau phase for another four weeks, and descendent healing began subsequently. Conclusion There are a variety of forms of the GuillainBarre Syndrome. Although it can be seen in all age groups, it is relatively rare in patients under 2 years of age In selected cases like this one, consecutive plasmapheresis and intravenous immunoglobulin therapies should be considered.

PP08.7 – 2364: Atypical Guillain Barré syndrome with preserved deep tendon reflexes: report of three cases

Objective Guillain Barre syndrome is a postinfectious polyneuropathy involving mainly motor but sometimes also sensory and autonomic nerves. Guillain Barre syndrome is now the world's most common cause of acute neuromuscular paralysis. Methods We present here 3 patients with preserved deep tendon reflexes. Case 1: An 11 year-old girl was admitted with a complaint of weakness confined to the lower extremities and in a lesser extent to the upper extremities over 4 days. On physical examination muscle strength was 2/5 on lower extremities and 4/5 in upper extremities. Case 2: A 17-year-old boy with known spondylarthritis presented with complaints of weakness of lower extremities over 10 days. On physical system examination muscle strength was 4/5 on lower extremities and 5/5 on upper extremities. Case 3: A 9-year-old boy was admitted with weakness and pain in the lower extremities over 10 days. His CK was 2988 (150–499 U/L). He was diagnosed as myositis and painkillers were prescribed. Then the weakness gradually worsened involving upper extremities. On physical system examination muscle strength was 4/5 on lower extremities and 2/5 on upper extremities. Results The deep tendon reflexes were normal in upper and lower extremities on repeated examinations in all of 3 cases. The diagnosis of Guillain Barre Syndrome was made by electrophysiological studies. Conclusion Although hypo- or areflexia is necessary for clinical diagnosis of GBS, preserved deep tendon reflexes do not exclude the diagnosis of Guillain-Barre syndrome. The electrophysiological studies play a very important role in differentiating it from other causes.

Guillain-Barré Syndrome Induced by Campylobacter jejuni

Purpose of Review: Guillain-Barre syndrome (GBS) is a neurologic disease that produces ascending paralysis that affects people all over the world. Several infectious agents have been associated with GBS and many reports suggest that infection with Campylobacter jejuni, a common enteric pathogen, may cause GBS by triggering demyelination of peripheral nerves. This review provides an update on the C. jejuni infections engaged in the developing of GBS. Summary and Results: Guillain–Barre syndrome is the most common cause of acute neuromuscular paralysis, yet its cause and pathogenesis are unknown. In approximately two thirds of patients, neuropathic symptoms follow an infection — often a mild, undiagnosed respiratory or gastrointestinal illness. The organism that has most frequently been described in association with GBS is C. jejuni, a gram-negative rod that is now the most common cause of bacterial gastroenteritis in developed countries. Although there has been a plethora of case reports and studies documenting the association, the specific clinical and epidemiologic features are not well Review Article Honarmand and Moghadam; BMRJ, 6(2): 71-83, 2015; Article no.BMRJ.2015.060 72 known. In addition, there is controversy about whether those with preceding C. jejuni infection have a more severe form of the GBS. C. jejuni can cause the disease by a mechanism called molecular mimicry. C. jejuni contains ganglioside-like epitopes in the lipopolysaccharide (LPS) moiety that elicit autoantibodies which can react with peripheral nerve targets. It seems that heterogeneity in the LPS structure determines the specificity of the antiglycolipid response and thereby the clinical features in patients with a post-campylobacter infection neuropathy.

Pharyngeal-Cervical-Brachial Variant of Guillain-Barre Syndrome

Guillain-Barre syndrome (GBS) is the most common cause of acute neuromuscular paralysis in the Western world. We describe the only known case presentation of pharyngeal-cervical-brachial variant following a subacute myocardial infarction and Vibrio cholerae vaccination. A 55-year-old Filipino man presented to our emergency department with a one-day history of progressive oropharyngeal and cervicobrachial weakness and areflexia in the upper limbs. His review of systems was unremarkable except for receiving “Dukoral” traveller’s diarrhea vaccine 3 weeks prior. His physical exam was compatible with GBS. EMG and nerve conduction studies showed low motor amplitudes, prolonged distal latency and absent F-waves in the arms. Lumbar puncture showed a protein level of 579 mg/L. Nuclear perfusion scan was consistent with a subacute myocardial infarction. He received a 2-day course of intravenous immunoglobulin. He showed significant improvement in his weakness over 3 days. Recent vaccination and myocardial infarction may be important triggers for GBS onset. J Neurol Res. 2014;4(2-3):88-90 doi: http://dx.doi.org/10.14740/jnr270w

Pharyngeal-Cervical-Brachial Variant of Guillain-Barre Syndrome

Guillain-Barre syndrome (GBS) is the most common cause of acute neuromuscular paralysis in the Western world. We describe the only known case presentation of pharyngeal-cervical-brachial variant following a subacute myocardial infarction and Vibrio cholerae vaccination. A 55-year-old Filipino man presented to our emergency department with a one-day history of progressive oropharyngeal and cervicobrachial weakness and areflexia in the upper limbs. His review of systems was unremarkable except for receiving “Dukoral” traveller’s diarrhea vaccine 3 weeks prior. His physical exam was compatible with GBS. EMG and nerve conduction studies showed low motor amplitudes, prolonged distal latency and absent F-waves in the arms. Lumbar puncture showed a protein level of 579 mg/L. Nuclear perfusion scan was consistent with a subacute myocardial infarction. He received a 2-day course of intravenous immunoglobulin. He showed significant improvement in his weakness over 3 days. Recent vaccination and myocardial infarction may be important triggers for GBS onset. J Neurol Res. 2014;4(2-3):88-90 doi: http://dx.doi.org/10.14740/jnr270w

The role of molecular mimicry in the etiology of Guillain Barré Syndrome

Molecular mimicry between host tissue structures and microbial components has been proposed as the pathogenic mechanism for triggering of autoimmune diseases by preceding infection. Recent studies stated that molecular mimicry as the causative mechanism remains unproven for most of the human diseases. Still, in the case of the peripheral neuropathy Guillain-Barré syndrome (GBS) this hypothesis is supported by abundant experimental evidence. GBS is the most frequent cause of acute neuromuscular paralysis and in some cases occurs after infection with Campylobacter jejuni (C. jejuni). Epidemiological studies, showed that more than one third of GBS patients had antecedent C. jejuni infection and that only specific C. jejuni serotypes are associated with development of GBS. The molecular mimicry between the human gangliosides and the core oligosaccharides of bacterial lipopolysaccharides (LPSs) presumably results in production of antiganglioside cross-reactive antibodies which are likely to be a contributory factor in the induction and pathogenesis of GBS. Antiganglioside antibodies were found in the sera from patients with GBS and by sensitization of rabbits with gangliosides and C. jejuni LPSs animal disease models of GBS were established. GBS as prototype of post-infection immune-mediated disease probably will provide the first verification that an autoimmune disease can be triggered by molecular mimicry.

Guillain‐Barré Syndrome Animal Model: The First Proof of Molecular Mimicry in Human Autoimmune Disorder

Molecular mimicry between self and microbial components has been proposed as the pathogenic mechanism of autoimmune diseases, and this hypothesis is proven in Guillain-Barré syndrome. Guillain-Barré syndrome, the most frequent cause of acute neuromuscular paralysis, sometimes occurs after Campylobacter jejuni enteritis. Gangliosides are predominantly cell-surface glycolipids highly expressed in nervous tissue, whilst lipo-oligosaccharides are major components of the Gram-negative bacterium C. jejuni outer membrane. IgG autoantibodies to GM1 ganglioside were found in the sera from patients with Guillain-Barré syndrome. Molecular mimicry was demonstrated between GM1 and lipo-oligosaccharide of C. jejuni isolated from the patients. Disease models by sensitization of rabbits with GM1 and C. jejuni lipo-oligosaccharide were established. Guillain-Barré syndrome provided the first verification that an autoimmune disease is triggered by molecular mimicry. Its disease models are helpful to further understand the molecular pathogenesis as well as to develop new treatments in Guillain-Barré syndrome.

Guillain–Barré syndrome: a review

Guillain-Barré syndrome is an autoimmune disease which is a common cause of acute neuromuscular paralysis and should be suspected in all patients with unexplained motor weakness. Specific management strategies are more effective if given early in the course of the disease.

What’s new in Guillain-Barré syndrome?

Guillain-Barre syndrome (GBS) has become the major cause of acute neuromuscular paralysis in the Western world since the eradicationof polio, with an annual incidence of 1–2 per 100,000. It can...

Clinical features, pathogenesis, and treatment of Guillain-Barré syndrome

Guillain-Barré syndrome (GBS) is an important cause of acute neuromuscular paralysis. Molecular mimicry and a cross-reactive immune response play a crucial part in its pathogenesis, at least in those cases with a preceding Campylobacter jejuni infection and with antibodies to gangliosides. The type of preceding infection and patient-related host factors seem to determine the form and severity of the disease. Intravenous immunoglobulin (IVIg) and plasma exchange are effective treatments in GBS; mainly for practical reasons, IVIg is the preferred treatment. Whether mildly affected patients or patients with Miller Fisher syndrome also benefit from IVIg is unclear. Despite medical treatment, GBS often remains a severe disease; 3-10% of patients die and 20% are still unable to walk after 6 months. In addition, many patients have pain and fatigue that can persist for months or years. Advances in prognostic modelling have resulted in the development of a new and simple prognostic outcome scale that might also help to guide new treatment options, particularly in patients with GBS who have a poor prognosis.

C5 inhibitor rEV576 protects against neural injury in an in vitro mouse model of Miller Fisher syndrome

Guillain-Barré syndrome and its clinical variants, including the anti-GQ1b ganglioside-mediated Miller Fisher syndrome (MFS), comprise the world's leading cause of acute neuromuscular paralysis. Presently, no specific drug therapies exist. The complement cascade, which is activated in these patients, forms an attractive drug target. In this study, we tested whether the complement C5-inhibiting recombinant protein, rEV576, was able to prevent neural injury in a previously developed in vitro mouse model for MFS. Mouse hemidiaphragm preparations were treated with anti-GQ1b antibody and normal human serum as a source of complement with added rEV576 or control protein. Immunohistology in control tissue showed deposition of C3c and membrane attack complex at neuromuscular junctions (NMJs), along with terminal motor axonal neurofilament degradation as well as ethidium homodimer-2 staining showing perisynaptic Schwann cell (pSC) injury. Electrophysiological and functional analyses showed block of synaptic transmission at the NMJ after an initial period of a dramatically high level of asynchronous acetylcholine release. In tissue treated with rEV576, all these indicators of motor neuronal damage were absent, except for the presence of C3c, indicating effective inhibition of C5. These results demonstrate that rEV576 effectively prevents development of neuronal and pSC damage in experimental murine neuropathy.

Intravenous immunoglobulin per se or combined with intravenous methylprednisolone in children with GBS; comparing the effects.

Objective Guillain-Barre syndrome (GBS) is the most common cause of acute neuromuscular paralysis in children, its pathogenesis most probably involving an autoimmune response to Schwann cell or peripheral nerve myelin antigens. Steroid regimes improve demyelinating diseases such as chronic GBS. We assessed the benefit of high dose methylprednisolone (MP) combined with Intravenous immunoglobulin (MP-IVIG) and compared the effects with those of IVIG per se in children with GBS. Materials& Methods Thirty-six children, aged between 1-12 years were randomized to receive IV MP 20mg/kg/day combined with IVIG 400 mg/kg/day (MP-IVIG) or IVIG per se at same dose for 5 days. All patients were diagnosed by standard clinical criteria and entered the trial within less than 2 weeks of the onset of neurological symptoms. All patients were too weak to walk. Functional grade (FG) was at 3 or more (able to walk with support). Results In the MP-IVIG group, FG improved at least one grade after 5 days of treatment. Conclusion This study suggests that combined treatment with MP-IVIG in children with GBS does cause rapid improvement in the acute phase, but does not result in any significant difference in the long term outcome.

A case of Guillain-Barrè syndrome following Staphylococcus aureus endocarditis

Guillain-Barrè syndrome (GBS) is the most important cause of acute neuromuscular paralysis in western countries and it is preceded in almost all cases by an infectious disease such as Campylobacter Jejuni or Cytomegalovirus. However, GBS associated with previous bacterial endocarditis is very rare. We report the case of a 74-year-old man with GBS following Staphylococcus Aureus endocarditis affecting aortic valve. Although the absolute incidence of GBS is low, the present case stresses the need to consider GBS in patients developing neurological symptoms following any infectious illness, such as endocarditis, and highlights the challenging problem of rehabilitation and surgical management in these patients.

Carbohydrate mimicry between human ganglioside GM1 and Campylobacter jejuni lipooligosaccharide causes Guillain-Barre syndrome.

Molecular mimicry between microbial and self-components is postulated as the mechanism that accounts for the antigen and tissue specificity of immune responses in postinfectious autoimmune diseases. Little direct evidence exists, and research in this area has focused principally on T cell-mediated, antipeptide responses, rather than on humoral responses to carbohydrate structures. Guillain-Barré syndrome, the most frequent cause of acute neuromuscular paralysis, occurs 1-2 wk after various infections, in particular, Campylobacter jejuni enteritis. Carbohydrate mimicry [Galbeta1-3GalNAcbeta1-4(NeuAcalpha2-3)Galbeta1-] between the bacterial lipooligosaccharide and human GM1 ganglioside is seen as having relevance to the pathogenesis of Guillain-Barré syndrome, and conclusive evidence is reported here. On sensitization with C. jejuni lipooligosaccharide, rabbits developed anti-GM1 IgG antibody and flaccid limb weakness. Paralyzed rabbits had pathological changes in their peripheral nerves identical with those present in Guillain-Barré syndrome. Immunization of mice with the lipooligosaccharide generated a mAb that reacted with GM1 and bound to human peripheral nerves. The mAb and anti-GM1 IgG from patients with Guillain-Barré syndrome did not induce paralysis but blocked muscle action potentials in a muscle-spinal cord coculture, indicating that anti-GM1 antibody can cause muscle weakness. These findings show that carbohydrate mimicry is an important cause of autoimmune neuropathy.

Guillain-Barre syndrome and Campylobacter jejuni infection

Guillain-Barre syndrome (GBS) is the most common cause of acute neuromuscular paralysis, usually due to acute inflammatory demyelinating polyradiculoneuropathy. The presence of activated T lymphocytes and antibodies against peripheral nerve myelin suggests an autoimmune pathogenesis, although there is wide heterogeneity. Gangliosides are sialylated glycolipids widely distributed in nervous system membranes. GBS is usually preceded by an infection, most frequently Campylobacter jejuni enteritis, but also cytomegalovirus, Mycoplasma pneumoniae or Epstein-Barr virus. Patients with GBS and C. jejuni infection are more likely to have neurophysiological features of axonal neuropathy, antibodies to ganglioside GM1, pure motor GBS, a less elevated CSF protein concentration and a worse outcome than other GBS patients. Although molecular mimicry between peripheral nerve gangliosides and epitopes present on C. jejuni lipopolysaccharide could explain some of these associations, this hypothesis is inadequate to account for many aspects of the pathogenesis of GBS.

Guideline for Neuromuscular Rehabilitation in Guillain-Barré Syndrome

Introduction. Originally described by Landry in 1859 and Guillain, Barré and Strohl in 1916, Guillain-Barré Syndrome (GBS) is the most common cause of acute neuromuscular paralysis selflimited in developed countries. The annual incidence of GBS is 1.5 per 100,000. Although it had a favorable prognosis (“maladie bénigne et spontanément curable”) it’s mortality rate is about 5% and 10% of patients remaining severely disabled one year after neurological onset. Specialist teams, intensive care and rehabilitation are essential for patient management and should be provided in appropriate hospital units. Objective. To guide the health professionals about the utilization of the physical and respiratory techniques in patients with GBS, as well as it’s indication. Method. Update of articles about GBS and Rehabilitation, on the last years of the data bases Bireme, ScienceDirect, PubMed and SciELO. Conclusion. There is currently no consensus on the management of these patients in the acute, sub-acute and chronic settings. Many of them are being discharged without access to rehabilitation services. Our results suggest that the rehabilitation makes a measurable and significant difference and should be available to all patients with GBS.

CAMPYLOBACTER JEJUNI INFECTION AS A CAUSE OF THE GUILLAIN-BARRÉ SYNDROME

A previously unrecognized sequelum of infection with Campylobacter jejuni, a common cause of gastroenteritis, is Guillain-Barré syndrome (GBS). GBS is the most common cause of acute neuromuscular paralysis; 30% to 40% of cases are preceded by C. jejuni infection. Both patient and bacterial characteristics likely play a role in the pathogenesis of C. jejuni-induced GBS. Molecular mimicry between the LPS of some campylobacters and structures present on the gangliosides of the peripheral nerve may explain how this acute infectious diarrheal illness triggers GBS.

Guillain-Barr?? Syndrome

Guillain-Barré syndrome (GBS) is the commonest cause of acute neuromuscular paralysis in the developed world today. Patients present most commonly with a rapidly ascending paralysis together with sensory symptoms and variable autonomic involvement. The diagnosis is clinical, but lumbar puncture and nerve conduction studies are helpful in excluding other conditions. The improvement in prognosis in recent years is largely due to advances in respiratory intensive care management. Careful monitoring of cardiorespiratory function and in particular regular measurements of the vital capacity will help to predict which patients will require elective ventilation to prevent impending neuromuscular respiratory failure. The paralysed patient is susceptible to all the complications of immobility, in particular venous thromboembolism and hypostatic pneumonia, and good nursing care and physiotherapy are therefore mandatory. Autonomic involvement may predispose to cardiac arrhythmias and labile blood pressure. The prolonged nature of the illness predisposes to psychiatric complications, particularly depression, and this should be treated appropriately. Specific treatment is aimed at reducing the period of maximum disability. Both plasma exchange (PE) and intravenous immune globulin (IVIg) have been shown to be effective in randomised controlled trials. A multicentre trial is currently under way to determine whether PE or IVIg or PE followed by IVIg is the most effective treatment for this condition. Steroids alone have not been shown to be of value, although a trial is under way comparing the combination of IVIg and methylprednisolone with IVIg alone. The prognosis of GBS is generally good, with about 80% of patients making a full recovery, although about 5% die of complications.(ABSTRACT TRUNCATED AT 250 WORDS)