Acute Inflammatory Polyradiculoneuropathy Research Articles

Clinical features and maternal and fetal outcomes in women with Guillain-Barré syndrome in pregnancy.

Guillain-Barre syndrome (GBS) is an acute inflammatory polyradiculoneuropathy rarely observed during pregnancy. In this retrospective study, we analyzed the characteristics of pregnant women with GBS (pGBS) diagnosed in French University Hospitals in the 2002-2022 period and compared them with a reference group of same-age non-pregnant women with GBS (npGBS) identified in the same institutions & timeframe. We identified 16 pGBS cases. Median age was 31years (28-36), and GBS developed in the 1st, 2nd, and 3rd trimester in 31%, 31% and 38% of cases respectively. A previous infection was identified in six cases (37%), GBS was demyelinating in nine cases (56%), and four patients (25%) needed respiratory assistance. Fifteen patients (94%) were treated with intravenous immunoglobulins, and neurological recovery was complete in all cases (100%). Unscheduled caesarean section was needed in five cases (31%), and two fetuses (12.5%) died because of cytomegalovirus (CMV) infection (1 case) and HELLP (Hemolysis, Elevated Liver enzymes and Low Platelets) syndrome (1 case). In comparison with a reference group of 18 npGBS women with a median age of 30years (27-33), pGBS patients more frequently had CMV infection (31% vs 11%), had a prolonged delay between GBS onset and hospital admission (delay > 7days: 57% vs 12%), more often needed ICU admission (56% vs 33%) and respiratory assistance (25% vs 11%), and more often presented with treatment-related fluctuations (37% vs 0%). This study shows GBS during pregnancy is a severe maternal condition with significant fetal mortality.

Current status of Guillain-Barré syndrome (GBS) in China: a 10-year comprehensive overview.

Guillain-Barré syndrome (GBS) is an acute inflammatory polyradiculoneuropathy; a disease involving the peripheral nervous system which is the most common cause of acute flaccid paralysis worldwide. So far, it is still lack of a comprehensive overview and understanding of the national epidemiological, clinical characteristics, and the risk factors of GBS in China, as well as differences between China and other countries and regions in these respects. With the global outbreak of the coronavirus disease 2019 (COVID-19), an epidemiological or phenotypic association between severe acute respiratory syndrome coronavirus-2 (SARS-CoV-2) infection and GBS has attracted great attention. In this review, we outlined the current clinical data of GBS in China by retrieving literature, extracting and synthesizing the data of GBS in China from 2010 to 2021. Besides, we compared the characteristics of epidemiology, preceding events and clinical profiles of GBS between China and other countries and regions. Furthermore, in addition to conventional intravenous immunoglobulin (IVIG) and plasma exchange (PE) therapy, the potential therapeutic effects with novel medications in GBS, such as complement inhibitors, etc., have become the research focus in treatments. We found that epidemiological and clinical findings of GBS inChina are approximately consistent with those in the International GBS Outcome Study (IGOS) cohort. We provided an overall picture of the present clinical status of GBS in China and summarized the global research progress of GBS, aiming to further understand the characteristics of GBS and improve the future work of GBS worldwide, especially in countries with the middle and low incomes.

Evidence of Lumbosacral Plexus Involvement on MR Neurography in Early Guillain-Barré Syndrome (P14-8.015)

<h3>Objective:</h3> To describe a case of early Guillain-Barré Syndrome (GBS) with novel findings of lumbosacral plexus involvement on MR neurography. <h3>Background:</h3> GBS is an acute inflammatory polyradiculoneuropathy characterized by progressive sensory loss, weakness, areflexia, CSF albumino-cytologic dissociation and cauda equina enhancement on MRI. Diagnosis of early GBS can be challenging, as CSF and electrodiagnostic studies can be normal/non-diagnostic in the first 1–2 weeks. We describe a case of early GBS with cauda equina enhancement and lumbosacral plexus abnormalities on MR neurography, seen only three days after symptoms onset and before any diagnostic abnormalities in CSF or electrodiagnostic testing were detected. <h3>Design/Methods:</h3> NA <h3>Results:</h3> A 67-year-old man presented with a 3-day history of rapidly progressive gait instability and leg weakness, 14 days after an uncomplicated L5-S1 fusion. Diffuse arm and leg weakness, distal leg sensory loss, hypoactive arm reflexes and absent leg reflexes were detected on exam. CSF analysis showed normal cells and protein (55mg/dL, reference&lt;60mg/dl). Electrodiagnostic testing showed mildly prolonged F-response latencies of the median and ulnar nerves, absent peroneal F-response, normal tibial F-response latency and absent tibial H-responses, without other findings suggestive of demyelination, and normal needle EMG. Lumbar spine MRI showed enhancement of ventrally positioned nerve roots of the cauda equina and no central or foraminal stenosis. Lumbosacral plexus MR neurography showed prominent enlargement, hyperintensity and enhancement of bilateral lower lumbosacral nerve roots and the proximal sciatic nerves. Plasma exchange was initiated with subsequent improvement in his symptoms and exam findings. <h3>Conclusions:</h3> Diagnosis of early GBS can be challenging, as CSF and electrodiagnostic testing can be normal/non-diagnostic. MRI and MR neurography are useful surrogate tests which may have greater sensitivity in detecting early GBS compared to CSF and electrodiagnostic testing. Involvement of the lumbosacral plexus by imaging is a novel finding, which can further assist in diagnosing early GBS cases. <b>Disclosure:</b> Dr. Weng has nothing to disclose. The institution of Darryl Sneag, 12603 has received research support from GE Healthcare. Dr. Pavlakis has nothing to disclose.

Clinical genetic counselling and rehabilitation treatment of a patient with Guillain–Barré syndrome after COVID-19

The authors present a clinical case of rehabilitation after the development of GuillainBarr syndrome, an acute autoimmune inflammatory polyradiculoneuropathy, in a patient who underwent a SARS CoV-2 infection. The patient previously manifested with severe comorbidities (arterial hypertension, hypercholesterolemia, type 2 diabetes mellitus, stenosing atherosclerosis of brachiocephalic arteries). A diagnostic panel of single nucleotide gene polymorphisms associated with risk factors of cardiovascular diseases, metabolic disorders, immunopathology, pharmacogenetics was applied using PCR-RT Genetic Passport test system, and the results were interpreted in order to predict potential complications, adverse drug reactions and the choice of biomarkers for preventive measures. We have compared clinical manifestations, comorbid background and the identified genotype features, as follows: minor homo- and heterozygous variants of ACE, AGT, CYP1A2, NOS3, PPARD, EDN, PALLD, SNX19 genes associated with predisposition to cardiovascular diseases, increasing the risk of dysregulation of blood pressure, development of endothelial dysfunction. The following gene variants were revealed: FXII, ITGA2, ITGB3, MTHFR, MTRR, MTR, PAI-1 that increase the risk of venous and arterial thrombosis, along with gene variants of ADRB3, FTO, INSIG2, KCNG11, LEP, PPARD, TCF7L2, ApoC3, PON1 associated with carbohydrate and lipid metabolism disorders; polymorphisms in the genes determining the immune response, i.e., IL4, IL6, IL8, IL10, CDH1, BDNF1, CRP, CCR5 (with del32 allele considered a risk factor of severe SARS-CoV-2), homozygous polymorphism of a gene of FCGR2 associated with risk of antigen-antibody-complement-mediated cytotoxicity, circulation arrest, deposition of immune complexes in endothelium of microvessels, decreased antithrombotic effects and increased procoagulant activity. Pharmacogenetic study revealed a variant of the CYP4F2 gene, a CYP2C19 gene polymorphism associated with delayed metabolism of a number of pharmaceuticals which requires higher drug dosage, or choosing a drug with a different mechanism of action; gene variants of CYP1A2, GSTP1, GSTT1 reducing efficiency of the cellular detoxification system; NAT2*5 and NAT2*6 variants determining a decrease in appropriate enzyme activities when administering a standard dose of drugs with slowdown of their detoxification, accumulation of toxic metabolites causing clinical adverse effects (hepatotoxicity, dyspepsia, lupus-like syndrome, polyneuritis). Based on the genotype that determines pathogenesis of the multifactorial pathology (including immune-mediated complications of COVID-19), a personalized approach is recommended to the patient, in terms of treatment and prevention of complications. On the basis of testing the biochemical, immunological and blood coagulation biomarkers, an adequate choice of pharmaceuticals is recommended for the patient.

CLINICAL CASE OF COVID-ASSOCIATED GUILLAIN-BARRE SYNDROME

The aim of our article is to shed light on the problem of the development of Guillain-Barre syndrome after COVID-19 infection&#x0D; Materials and methods&#x0D; The main research methods we used were scientific research and the actual description of our own clinical case. Laboratory research methods, magnetic resonance imaging, and electromyography were used to confirm the diagnosis and perform differential diagnostics.&#x0D; Results.&#x0D; In 2021, the Center for Acute Neurology and Pain Treatment treated 12 patients with Guillain-Barre syndrome. Guillain-Barre syndrome (GBS) is an acute autoimmune inflammatory polyradiculoneuropathy. The clinical picture of GBS includes progressive motor and sensory disorders in the extremities and pain.&#x0D; We bring to your attention a clinical case of GBS in patient B., who was treated at the clinical hospital "Feofania". Patient B., 32 years old, was hospitalized in the Feofania Clinical Hospital with complaints of weakness in her arms and legs, inability to walk, foreign body sensation in the throat, difficulty swallowing, chest discomfort, and difficulty breathing. A few weeks before the disease, the patient was treated for a mild SARS-CoV-2 infection. The patient's condition gradually deteriorated by day 25 of the disease, despite plasmapheresis, intravenous immunoglobulin and glucocorticoid therapy. However, from day 29, the condition gradually improved and on re-examination after 2 months, the patient remained mild distal paresis of the leg muscles (up to 4 points), decreased tendon reflexes from the lower extremities. Disorders of sensitivity and function of the pelvic organs were not observed.&#x0D; Conclusion.&#x0D; In our article, we present a case of COVID-19-associated GBS with a classic clinical picture, increased peripheral paresis, stabilization, and gradual recovery of all functions (respiration, pelvic function, motor, sensory, and reflex) through pathogenetic treatment and follow-up rehabilitation measures.

Guillain barre syndrome and its association with covid-19 infection – A clinical case series

A novel coronavirus (COVID-19) pandemic is caused by severe acute respiratory syndrome coronavirus 2 (SARs-CoV-2). This pandemic has been globally alarming in the current period. Several neurological manifestations are reported occurring with the infection. Guillain barre syndrome (GBS) or acute onset inflammatory polyradiculoneuropathy has been among the frequent manifestations observed among them. To know the pattern and outcome of GBS in COVID-19 affected individuals. We have taken six individuals admitted with flaccid quadriparesis in the last two months. All were affected recently by COVID 19 infection, which RT PCR of the nasopharyngeal swab confirmed. The study participants have undergone nerve conduction studies and have been diagnosed with Guillain Barre syndrome using Brighton criteria. We did cerebrospinal fluid (CSF) analysis after admission. We initiated all patients on Intravenous immunoglobulin according to body weight (2g/kg divided over five days). We used the Barthel index score to assess the outcome of the individuals. We observed a mean duration of 18.25 days between the COVID-19 infection and the onset of symptoms. Apart from motor quadriparesis and sensory symptoms being in common, we also noticed cranial nerves and autonomic involvement. We made the diagnosis using the nerve conduction studies and Brighton criteria. After initiating intravenous immunoglobulin, all patients had a good outcome, and quality of life was better after two months of follow up. Guillain Barre syndrome is one of the neurological manifestations of COVID-19 and has a dramatic response with intravenous immunoglobulin and better outcome with treatment.

Hepatitis E Virus Quasispecies in Cerebrospinal Fluid with Neurological Manifestations.

Hepatitis E virus (HEV) infection can lead to a variety of neurological disorders. While HEV RNA is known to be present in the central nervous system, HEV quasispecies in serum and cerebrospinal fluid (CSF) have rarely been explored. We studied the virus’ quasispecies in the blood and the CSF of five patients at the onset of their neurological symptoms. The samples of three patients suffering from meningitis, neuralgic amyotrophy and acute inflammatory polyradiculoneuropathy were taken at the acute phase of the HEV infection. The samples from the other two patients were taken during the chronic phase (5 years after HEV diagnosis) when they presented with clinical signs of encephalitis. We sequenced at least 20 randomly polyproline regions of the selected virus clones. Phylogenetic analysis of the virus variants in the blood and the CSF revealed no virus compartmentalization for the three acute-phase patients but there was clear evidence of HEV quasispecies compartmentalization in the CSF of the two patients during chronic infection. In conclusion, prolonged infection in the immunocompromised condition can lead to independent virus replication in the liver and the tissues, producing viruses in CSF.

A post COVID-19 Guillain-Barre syndrome

Guillain-Barré syndrome consists group of clinical syndromes that manifests as an acute or subacute autoimmune inflammatory polyradiculoneuropathy with resultant weakness and diminished reflexes. It is also part of so-called Yuki syndrome. Its annual incidence is 1–2/100000. The exact cause of Guillain-Barre syndrome is unknown. But two-thirds of patients report symptoms of an infection in the six weeks preceding. These include respiratory or a gastrointestinal infection (EBV, C. Jejuni) or Zika virus.

Relationship between cerebrospinal fluid protein level and electrophysiologic abnormalities in the acute inflammatory demyelinating polyradiculoneuropathy variant of Guillain-Barré syndrome.

Objective: Guillain-Barré syndrome (GBS) is an autoimmune disease characterized by weakness in limbs or cranial nerve innervated muscles. Acute inflammatory demyelinating polyradiculoneuropathy (AIDP) is the most common variant. Electrophysiologic abnormalities and elevated cerebrospinal fluid (CSF) protein are frequently present in AIDP, but the relationship between these two parameters is not well known. We aimed to fill this gap by studying this relationship.Methods: This was a prospective cross-sectional study conducted for two years in the Department of Neurology, Dr. Ruth K. M. Pfau Civil Hospital, Karachi, Pakistan. All 90 adult patients with the AIDP variant of GBS were selected. Nerve conduction studies were performed to determine the degree of demyelination through the four electrophysiologic demyelination criteria. The CSF sample was sent to lab immediately after lumbar puncture. SPSS version 20.0 was used. The CSF protein level was measured with mean ±SD. Demyelination criteria were measured in frequency and percentages. Chi-square test was applied to a number of demyelination criteria and T-test/ANOVA was applied on mean CSF protein level.Results: We found a mean CSF protein of 37.41 mg/dl (±3.69) with one demyelination criterion, 81.87 mg/dl (±17.39) with two demyelination criteria, 119.75 mg/dl (±31.42) with three demyelination criteria, and 134.00 mg/dl (±42.87) with four demyelination criteria (P-value <0.001). Conclusion: This study demonstrates a significant relationship between CSF protein levels and degree of demyelination in the AIDP variant of GBS. This is an under-researched area in GBS and this study adds favorably to limited data in this regard.

Comparative Study between COVID Associated Guillan Barre Syndrome and Non-COVID Associated Guillan-Barré Syndrome

More than 12 million people have been infected with COVID-19 worldwide, with more than 500,000 deaths to date.1 Although COVID-19 research is rapidly evolving, new findings must be thoroughly scrutinised before any conclusions or treatment protocols are established or amended.2 Although COVID-19 is most usually associated with respiratory symptoms such as cough and dyspnea; it has recently been associated with a neurotropic presentation.3 Guillain-Barré Syndrome (GBS) is best described as an acute inflammatory polyradiculoneuropathy clinically characterized by areflexia and progressive weakness of arms and legs. Though, many rare variants of GBS have been described, the commonly observed subtypes such as Acute Motor Axonal Neuropathy (AMAN), Acute Motor Sensory Axonal Neuropathy (AMSAN) and Acute Inflammatory Demyelinating Polyradiculoneuropathy (AIDP) tend to fulfil the above-mentioned criteria.4 Recently, several case reports have suggested a relationship between the occurrence of Guillain-Barré syndrome (GBS) and a previous SARS-CoV-2 infection, which preceded the GBS onset by up to 4 weeks. Therefore, a post-infectious dysregulation of the immune system, triggered by SARS- CoV2, appears to be the most probable cause COVID-19 is a systemic disorder presenting typically with fever and respiratory symptoms but neurological manifestations such as acute cerebrovascular diseases, seizures, ageusia, anosmia meningitis, encephalitis and skeletal muscle involvement were soon reported.5 More recently, an increase in case reports of Guillain-Barré syndrome (GBS) in people infected with SARS-CoV-2 has prompted concerns about a possible link.

Guillain-barré syndrome after purified vero cell rabies vaccination: An unusual case report

Guillain-Barré syndrome (GBS) can be described as a collection of clinical syndromes that manifests as an acute inflammatory polyradiculoneuropathy with resultant weakness and diminished reflexes. Regarding pathophysiology of GBS, it is believed that autoimmunity- molecular mimicry plays a role in its pathogenesis. As the vaccines have an effect on the immune system it is reasonable that immunizations may be associated with subsequent GBS. Although rabies vaccines containing neural elements are very well known cause for GBS, no such positive correlation has been proven after newer formulations of rabies vaccine, derived from chick embryo cells. Here we report a case of GBS which developed after 5 weeks of anti-rabies vaccination (Purified Vero cell rabies vaccine) which was given after dog bite.

P-PN024. The profile of cerebrospinal fluid analysis in patients with Guillain-Barré Syndrome

Introduction . Guillain-Barré Syndrome (GBS) is a clinical syndrome of an acute inflammatory polyradiculoneuropathy with ascending muscle weakness. One of the criteria to diagnose GBS is including the cerebrospinal fluid examination. Several parameters had been analysed from this examination. During the acute phase of GBS, characteristic findings on CSF analysis include albuminocytologic dissociation, which is an elevation of CSF protein (>0.55 g/L) without an elevation of white blood cells. In this study, we describe the profile of cerebrospinal fluid analysis in patients with GBS. Methods . Thirty-three adult patients with GBS were retrospectively analysed from medical record in Dr.Sardjito General Hospital, Yogyakarta, collected from January 2016 to September 2019. Results . The age of patients ranged from 18 to 77 years. The proportion of male was higher than female (21 vs 12 patients). The cerebrospinal fluid analysis found that the mean white blood cells count was 17,82 ± 44,965 cells/µL, range from 0 to 200 (normal result 0–5 cells/µL). The mean protein count was 0,13242 ± 0,147098 g/dL, ranged from 0,010 to 0,800 (normal result 0,02–0,05 g/dL). The albuminocytologic dissociation result was found in 21 of 33 patients (63,64%). The mean of glucose level was 91,4848 ± 22,439 mg/dL, ranged from 68 to 188 (normal result 50–80 mg/dL). Conclusion . Cerebrospinal fluid analysis showed the elevation of white blood cells count, protein count and glucose level in GBS patients. More than half of the results showed albuminocytologic dissociation.

Severe Headache, Paraesthesias, Facial Diplegia and Pleocytosis: A Misleading Presentation of Guillain-Barré Syndrome.

Bifacial weakness with paraesthesias (BFP) is a rare regional subtype of classic Guillain-Barré syndrome.Severe headache can be a symptom of the BFP variant.The association of headache, BFP and pleocytosis can be confusing, particularly if initial nerve conduction studies are unrevealing.

A review of the role of genetic factors in Guillain-Barré syndrome.

Guillain-Barré syndrome (GBS) is an acute inflammatory polyradiculoneuropathy syndrome. Several genetic and environmental risk factors have been recognized for GBS. AS GBS is an immune-related disorder, abnormal functions of T cells, production of autoantibodies, and dysregulation of gene expression have been detected in GBS patients. Based on the critical role of human leukocyte antigen (HLA) in the regulation of immune responses, HLA alleles are among the mostly investigated loci in GBS. A number of polymorphisms within different genes, especially those linked with the regulation of immune responses, have been associated with GBS in different populations. Moreover, several studies have demonstrated abnormal expression of cytokine-coding genes in this disorder. Investigations in the animal model of GBS have also verified the aberrant regulation of Th1/Th2/Th17/Treg cytokines. In the current review, we describe the information about the role of these factors in GBS.

Síndrome de Guillain Barré en paciente con antecedente de infección respiratoria: reporte de un caso

Introducción: El síndrome de Guillain-Barré (SGB), es una polineuropatía inflamatoria aguda, de etiología autoinmune que causa inflamación en los nervios periféricos y sus raíces espinales, se presenta como una parálisis flácida, simétrica, ascendente, arrefléxica y con antecedentes de una infección respiratoria o gastrointestinal previa. &#x0D; Objetivo: Describir un caso clínico de Guillain Barré, con antecedentes de infección respiratoria previa.&#x0D; Material y métodos Estudio descriptivo, retrospectivo, presentación de un caso clínico.&#x0D; Resultados: Se trata de una paciente femenina de 69 años sin antecedentes patológicos de interés, padeció una infección respiratoria viral hace dos semanas, acude a médico particular presentando dolor en extremidades, parestesias en miembros inferiores, se prescribe ketoprofeno 100 mg, tiocolchicócido 4 mg e ibuprofeno 400 mg en dosis únicas, con lo cual no hubo mejoría, agravándose el cuadro clínico. Doce días después acude con disminución de fuerza muscular y dificultad para la deambulación. Reflejos osteo-tendinosos ausentes y leve disminución de la sensibilidad en zona distal de ambos miembros inferiores. Entre los exámenes complementarios se observó disociación albumino-citológica en el líquido cefalorraquídeo (LCR) y una electromiografía compatible con Polineuropatía Aguda. Se diagnosticó SGB, brindándose tratamiento con inmunoglobulina humana endovenosa con una evolución y resultados favorables. Conclusiones: El SGB es una polirradiculoneuropatía aguda inflamatoria desmielinizante monofásica ascendente de origen inmunológico, cuyo diagnóstico es clínico, los exámenes complementarios ayudan a corroborar la patología y permiten clasificar la enfermedad, como tratamiento se sigue manteniendo el uso de plasmaféresis o inmunoglobulina humana intravenosa sin evidenciarse superioridad a favor de alguno de ellos.

History of acute polyradiculoneuropathy (part 1): The prehistory of Guillain-Barré syndrome.

Guillain-Barré syndrome (GBS) is an acute inflammatory polyradiculoneuropathy described in 1916 by Guillain, Barré, and Strohl. However, many similar cases had been reported earlier under various terms, with less detail and with various explanations about its pathophysiologic origin. Based on the analysis of old articles, we propose an overview of the history of acute inflammatory polyradiculoneuropathy before the official description of GBS.

Guillain-Barr\xe9 syndrome with associated unilateral ptosis without ophthalmoplegia \u2013 a rare presentation: a case report and review of the literature

BackgroundGuillain-Barré syndrome is an acute inflammatory polyradiculoneuropathy. Nearly half of patients with Guillain-Barré syndrome have cranial nerve involvement. However, isolated bilateral ptosis without ophthalmoplegia is a rare manifestation, and isolated unilateral ptosis without ophthalmoplegia in Guillain-Barré syndrome has not previously been reported in the literature. Furthermore, only few cases of Guillain-Barré syndrome with cranial nerve enhancement visualized by gadolinium-enhanced magnetic resonance imaging have previously been reported. We describe the first reported case of unilateral ptosis without ophthalmoplegia in Guillain-Barré syndrome and associated multiple cranial nerve enhancement seen by gadolinium-enhanced magnetic resonance imaging.Case presentationOur patient was a 55-year-old Sinhalese man who was admitted to a tertiary care hospital in Sri Lanka with acute-onset progressive weakness in the lower limbs followed by the upper limbs. He had bilateral symmetrical flaccid quadriparesis with absent reflexes and flexor plantar response. Left-sided isolated partial ptosis without associated ophthalmoplegia was noted with normal pupils. The patient’s neurological examination was otherwise normal. A nerve conduction study showed a severe demyelinating type of polyneuropathy. No decremental response to repetitive nerve stimulation was observed, and the result of a single-muscle-fiber electromyogram was negative. A diagnosis of Guillain-Barré syndrome was made, and the patient was treated with intravenous immunoglobulin. His condition gradually deteriorated over the next few days, and he became quadriplegic despite the completion of immunoglobulin therapy. Later he developed multiple cranial nerve palsies, including bi-lateral lower motor neuron type facial nerve palsy, and he required mechanical ventilation. By this time, he had complete left-sided ptosis with a normal right eye. He never developed ophthalmoplegia or ataxia. Magnetic resonance imaging of the brain showed contrast enhancement in the intracranial part of multiple cranial nerve roots and basal leptomeninges. He gradually improved with plasmaparesis, and ptosis was the first to improve.ConclusionsEven though Guillain-Barré syndrome was recognized a century ago, there are still many unanswered questions about it and its florid presentation. Large-scale studies are needed for better understanding of its pathophysiology and prototypes and to find answers for still-unanswered questions. The clinician must have a high index of suspicion and be familiar with mimics and prototypes to diagnose Guillain-Barré syndrome accurately without delay.

BS21. Neurophysiological assessment of autonomous nervous system in patients with Guillain–Barré syndrome in Brazil

Guillain–Barré syndrome (GBS) is an acute inflammatory polyradiculoneuropathy with a high risk of clinical and subclinical autonomic dysfunction even in early stages of evolution, which carries an increase in their morbidity and mortality. In our study, we evaluated the incidence of early and subclinical autonomic disturbance in patients with GBS in a brazilian university center. We evaluated 14 patients with GBS confirmed by the criteria of Brighton and Hadden for demyelinating GBS (AIDP) or axonal GBS (AMAN), admitted in the neurology department of University of Sao Paulo Medical School, throughout the year 2016. The cases were also classified by Erasmus’s gravity score at admission. Autonomic evaluation was conducted in the first four weeks of the onset of symptoms, with patients at rest, without autonomic interfering drugs, just before nerve conduction studies. Autonomic studies consisted of sympathetic skin response (SSR) elicited by electric stimulation and heart rate variability (HRV) during deep breathing maneuver for one minute (expiration: inspiration ratio) and spectral analysis of a five minutes long recording during spontaneous breathing. Two out of ten patients with AIDP (20%) and two out of four patients with AMAN (50%) presented evidence of autonomic dysfunction at the time of the evaluation. These four patients presented an Erasmus score greater than or equal to 4 on the occasion and evolved with clinical dysautonomia during hospitalization. HRV was abnormal in three of these cases. SSR was absent in only one case. No patient with an Erasmus score less than 4 presented neurophysiologic evidence of autonomic dysfunction, but three out of ten (30%) eventually evolved with mild clinical dysautonomia during hospitalization. General sensitivity of neurophysiologic evaluation was 57%. No patient died or suffered severe dysautonomia in our sample. HRV and SSR were able to detect early dysautonomia in patients with GBS, especially in those with Erasmus score greater than or equal to 4. HRV seems to be more sensitive than SSR.

Guillain-Barré syndrome, transverse myelitis and infectious diseases.

Guillain-Barré syndrome (GBS) and transverse myelitis (TM) both represent immunologically mediated polyneuropathies of major clinical importance. Both are thought to have a genetic predisposition, but as of yet no specific genetic risk loci have been clearly defined. Both are considered autoimmune, but again the etiologies remain enigmatic. Both may be induced via molecular mimicry, particularly from infectious agents and vaccines, but clearly host factor and co-founding host responses will modulate disease susceptibility and natural history. GBS is an acute inflammatory immune-mediated polyradiculoneuropathy characterized by tingling, progressive weakness, autonomic dysfunction, and pain. Immune injury specifically takes place at the myelin sheath and related Schwann-cell components in acute inflammatory demyelinating polyneuropathy, whereas in acute motor axonal neuropathy membranes on the nerve axon (the axolemma) are the primary target for immune-related injury. Outbreaks of GBS have been reported, most frequently related to Campylobacter jejuni infection, however, other agents such as Zika Virus have been strongly associated. Patients with GBS related to infections frequently produce antibodies against human peripheral nerve gangliosides. In contrast, TM is an inflammatory disorder characterized by acute or subacute motor, sensory, and autonomic spinal cord dysfunction. There is interruption of ascending and descending neuroanatomical pathways on the transverse plane of the spinal cord similar to GBS. It has been suggested to be triggered by infectious agents and molecular mimicry. In this review, we will focus on the putative role of infectious agents as triggering factors of GBS and TM.

Czech National Guillain-Barré Syndrome Registry

Introduction: The Guillain-Barre syndrome is an acute infl ammatory autoimmune polyradiculoneuropathy and belongs among rare neurologic diseases. Since poliomyelitis has been almost completely eliminated, it is currently the most frequent cause of acute fl accid paralysis worldwide. The registry was formed in the Czech Republic in 2012 and is an important source of prospectively recorded data. Aim: The goal of the registry is to follow up the clinical profi le, treatment and 6-month prognosis of Guillain-Barre syndrome patients. Methods: The data are collected prospectively by persons delegated by neuromuscular centres in the Czech Republic. The clinical course is assessed on the basis of the GBS disability scale and muscle strength according to the Medical Research Council sum score for facial muscles and extremity muscles. Detailed clinical investigation is done on admission, before the treatment, immediately after the treatment, 1 month and 6 months after the end of the treatment. Results: We enrolled a total 272 cases of Guillain-Barre syndrome patients in the period from 1st January 2012 to 17th August 2016. We have complete data for 6 months in 137 of them. 52 were treated with intravenous human immunoglobulin and 85 with plasma exchange. A good clinical outcome after 6 months (ability to walk unassisted) was achieved in 85% of patients. Severe residual neurological defi cit persisted after 6 months in 11% of patients. The remaining 6 patients died. We did not fi nd any diff erence in the effi ciency of immunoglobulin vs. plasma exchange in the whole group, in the subgroup of the severely disabled patients and according to sex. Conclusion: Medical care centralisation and adherence to the standard of care lead to a good clinical outcome in Guillain-Barre syndrome patients, which is comparable with relevant sources.