Acute inflammatory demyelinating polyneuropathy (Guillain-Barré syndrome)

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.

Guillain-Barré syndrome (GBS) is the most common cause of acute paralysis in the United States. Campylobacter jejuni is a common trigger for GBS, igniting autoimmunity as a result of molecular mimicry between C.jejuni lipooligosaccharide (LOS) and host gangliosides. Evidence also suggests an active role for cell-mediated and innate immunity in pathogenesis of GBS. Infection alone is not enough for GBS to develop, infection with the same strain might yield different outcomes in different patients. C.jejuni strains with low to absent molecular mimicry to self-antigens can cause full-blown GBS with positive autoantibodies. A role for T helper 17 and IL-17 in acute phase of GBS is also identified. Currently, no biological treatment is validated for severe, ventilation-dependent patients with GBS, who might not benefit from either IVIG or plasma exchange therapy. Use of biologic agents in treatment-resistant GBS, especially anti-IL-17 agents, such as secukinumab, ixekizumab, and brodalumab, is to be hoped. This review covers up-to-date knowledge on autoimmune mechanisms responsible in different subtypes of GBS: acute inflammatory demyelinating polyneuropathy and acute motor axonal neuropathy; as well as the experimental autoimmune neuritis (EAN), a commonly used animal model of GBS.

Guillain-Barré syndrome (GBS) is the most common cause of acute paralysis, yet the pathogenesis has still not been fully elucidated and specific evidence-based consensus management guidelines have not been developed. This paper reviews the research of the past year dedicated to determining the pathogenesis of GBS, optimizing current management, and searching for more efficacious treatment alternatives. Several recent studies have investigated whether there are any particular factors that might predict the course of illness and, thereby, dictate the optimal treatment. Proposed evidence-based guidelines for elective intubation, admission to the intensive care unit, and overall management of GBS are summarized. Therapeutic plasma exchange and intravenous immunoglobulin are both treatments that have been shown to have a beneficial effect on the course of GBS. Various modifications of plasmapheresis, as well as other alternative therapies, are currently being investigated.

Peripheral neuropathies may complicate all stages of infection with human immunodeficiency virus (HIV). Acute inflammatory demyelinating polyneuropathy, sensory ganglioneuritis, and acute cranial nerve palsy all may occur 2 to 3 weeks after acute HIV infection. Acute inflammatory demyelinating polyneuropathy, chronic inflammatory demyelinating polyneuropathy, and polyradiculopathy may occur with otherwise asymptomatic HIV virus infection. Neuropathy is one of the most common neurological manifestations of the acquired immunodeficiency syndrome (AIDS)-related complex, occurring in as many as 20% of these patients. Acute or chronic inflammatory demyelinating polyneuropathy (38%) and mononeuropathy multiplex (29%) are most frequently seen, and usually there is a good prognosis, with the neuropathy resolving spontaneously or with steroids or plasmapheresis. Neuropathy occurring with AIDS is reportedly uncommon but probably is underreported, especially in seriously ill patients. By contrast with AIDS-related complex, the neuropathy associated with AIDS is usually a distal symmetrical polyneuropathy (72%), with inflammatory neuropathy, mononeuropathy multiplex, and polyradiculopathy occurring rarely. The pathogenesis of acute or chronic inflammatory demyelinating polyneuropathy and possibly of mononeuropathy multiplex is probably autoimmune. The pathogenesis of distal symmetrical polyneuropathy is less clearly established and may be infectious, toxic, or nutritional. Polyradiculopathy most likely is infectious; cytomegalovirus is a leading contender for infectious agent, but herpes simplex virus and HIV are other possibilities.

Guillain–Barre syndrome (GBS) is an acute onset, usually monophasic immune-mediated disorder of the peripheral nervous system. The term GBS is often considered to be synonymous with acute inflammatory demyelinating polyradiculoneuropathy (AIDP), but with the increasing recognition of variants over the past few decades, the number of diseases that fall under the rubric GBS have grown to include axonal variants and more restricted variants, such as Miller Fisher syndrome (MFS) [Table 1].[1] Table 1 Guillain–Barre syndrome—clinical variants Epidemiology The reported incidence rates for GBS are 1–2 per 100,000 population.[2–4] The lifetime likelihood of any individual acquiring GBS is 1:1000.[5] The subtypes of GBS have different incidence rates in different parts of the world. In Europe and North America AIDP is dominant contributing to 90% of the cases. In contrast in China and Japan AMAN being the most common subtype.[6,7] The picture is intermediate when we look at other population. In Indian series the incidence of AIDP and AMAN are virtually equal although AMAN is more common in younger patients.[8] There seems to be a slight preponderance of AIDP in studies by Gupta et al[9] and by Meena et al (unpublished data from NIMS, Hyderabad). Available Indian literature indicates a peak incidence between June–July and Sept–October.[10] In western countries, GBS is common in the 5th decade,[11] but in India it occurs more commonly at a younger age.[10,12] GBS is equally common in men and women and can occur at any age. There is a male preponderance among the hospitalized population.[10,12]

A previously healthy, unimmunized, 3-year-old Caucasian boy presented to the emergency department with right-sided facial droop, clumsiness, and intermittent bilateral hip pain. Two weeks ago, he had 24 hours of self-resolving rhinorrhea and fever. Examination was significant for right facial nerve palsy, lower extremity pain, areflexia of his right lower extremity, and diminished reflexes of his left lower extremity. He was admitted for urgent magnetic resonance imaging of the brain. Cerebrospinal fluid (CSF) protein was 85 mg/dL with elevated albumin and immunoglobulin, and CSF white blood cell was 3 cells/mm3. Serum Mycoplasma immunoglobulin (Ig) M and IgG were elevated. There was concern for Guillain-Barré syndrome (GBS). He was started on intravenous IG (IVIG) and was treated for presumed Mycoplasma infection. Weakness and gait disturbances in a child can present the clinician with a diagnostic challenge. Gait disturbance may indicate a neurological lesion anywhere from the central nervous system to the peripheral nerves, neuromuscular junction, or muscle. In the present case, the combination of peripheral facial palsy, presumed neuropathic pain, gait difficulties, and areflexia in the setting of an antecedent respiratory illness were suggestive of GBS. The cornerstone treatments involve hospitalization to facilitate continuous monitoring for serious sequelae, such as acute respiratory failure and cardiac dysrhythmia, followed by immunotherapy with IVIG or plasma exchange. Gait disturbance and weakness in a child is a diagnostic challenge. GBS is the most common cause of acute paralysis in the Western world and should remain high on the clinician’s differential diagnosis. However, patients with GBS may also present nonclassically with extremity pain and cranial nerve palsies.

Source: Baxter R, Bakshi N, Fireman B, et al. Lack of association of Guillain-Barré syndrome with vaccinations. Clin Infect Dis. 2013; 57(2): 197– 204; doi: 10.1093/cid/cit222Investigators from the Kaiser Permanente Medical Group and Vaccine Study Center and the Centers for Disease Control and Prevention (CDC) retrospectively evaluated a cohort of individuals with documented Guillain-Barré syndrome (GBS) to see if there was an association with vaccination in the 6 or 10 weeks prior to onset. Cases were classified based on a review of medical records by a neurologist according to the Brighton Collaboration case definition criteria.1 The study population was comprised of the >3 million individuals in Northern California insured by Kaiser Permanente. Cases included all individuals hospitalized with first occurrence of verified GBS between 1995 and 2006. Only those who received any vaccine dose within a year of onset of GBS (9 months for influenza vaccine to avoid confounding from the previous year’s vaccine) were included. Odds ratios were calculated by comparing the observed odds that vaccination of cases occurred within the risk interval prior to GBS onset compared to the expected odds derived from the proportion of the vaccinated health plan population, matched for age and sex, who were vaccinated with that same vaccine within the same risk interval.Electronic medical record review revealed 415 confirmed cases of GBS during the study follow-up period of over 32 million person-years, yielding an incidence of 1.27 cases/100,000 person-years. Two thirds of cases had a documented respiratory or gastrointestinal illness in the 90 days preceding onset of GBS. Cases peaked in March and were more common in winter compared to nonwinter months (P = .003). Among the 415 cases, 25 (6%) had received any vaccine in the 6 weeks prior to onset. The odds ratio for receipt of influenza vaccine (TIV) within 6 weeks of onset of GBS compared to the prior 9 months was 1.1 (95% CI, 0.4–3.1). The odds ratios of receipt of tetanus-diphtheria-containing vaccine, 23-valent pneumococcal vaccine, or all vaccines combined in the 6 weeks prior to onset of GBS compared to the prior 12 months were 1.4 (95% CI, 0.3–4.5), 0.7 (95% CI, 0.1–2.9), and 1.3 (95% CI, 0.8–2.3), respectively. Using the 10-week risk interval, 37 (9%) of the individuals with GBS had been vaccinated. However, only injectable typhoid vaccine had an elevated odds ratio (10.75; 95% CI, 1.14–285.04) for GBS. For childhood vaccines, there were no cases of GBS during the risk intervals, despite administration of >8 million doses. Of the 18 persons with onset of GBS within 6 weeks of receipt of TIV, 13 (72%) had a documented antecedent respiratory or gastrointestinal infection.The authors conclude that there is no evidence for an association of GBS with antecedent vaccination, including influenza vaccination. They point out that a very small increased risk of GBS cannot be excluded, however, because of the rarity of the outcome (despite the large numbers included in the study).Dr Tolan has disclosed no financial relationship relevant to this commentary. This commentary does not contain a discussion of an unapproved/investigative use of a commercial product/device.The use of a case-centered approach and a large cohort of individuals renders this study a valuable addition to the evidence that vaccines are rarely, if at all, associated with GBS (since the 1976 A/ New Jersey swine influenza vaccine was removed from the market).2 As pointed out in the editorial commentary that accompanies this article,3 the power of the study was insufficient to completely exclude any association between vaccines and GBS. Further, the reviewing neurologist was not blind to the diagnosis and possible vaccine triggers documented in the charts, although the criteria used to assign a GBS diagnosis were strict. Most important, however, is the potential that vaccine uptake was underestimated by relying solely on the electronic medical record. In fact, this population was 1 of 4 cohorts analyzed to ascertain the sensitivity of the electronic medical record for TIV receipt.4 Although blinded to particular site, only 51% to 89% of TIV doses actually received were captured in the electronic record. Thus, an important number of TIV doses (11%–49%) were not identified in this study.This important study addresses both influenza and other vaccines, and provides reassurance that childhood vaccines are not associated with GBS. Even if there is a risk on the order of 1 or 2 cases of GBS per million doses of TIV, this risk pales in comparison to the lives saved by influenza vaccination.GBS, an acute inflammatory polyneuropathy, has been associated with one universally administered vaccine, the 1976 Swine influenza vaccine – and, despite continuing concerns as underscored by this report, none since. Pediatricians might well be reminded that the most frequent antecedents of GBS are infectious. Campylobacter jejuni, the most commonly associated agent, is a preventable food-borne and zoonotic pathogen.

To estimate the burden of disease of Guillain-Barré syndrome (GBS) in Brazil in 2014, 1year before the Zika virus epidemic, and in 2015 and 2016 during the epidemic. The burden of disease of GBS was estimated using the summary measure of population health: Disability Adjusted Life Years (DALY), that combines both mortality (Years of Life Lost YLLs) and morbidity (Years Lived with Disability) components. The study population was composed of GBS hospitalised cases and deaths from the information systems of the Brazilian Unified Health System. The GBS incidence rate in 2014, 2015 and 2016 was 0.74, 0.96, 1.02/100000 respectively, and the mortality rate in the same period was 0.08, 0.009 and 0.11/100000 habitants. The DALYs calculated using the point estimate of GBS disability weight and its values of the confidence interval (0.198 and 0.414) were 5725.90 (5711.79-5742.89) in 2014, 6054.61 (6035.57-6077.54) in 2015 and 7588.49 (7570.20-7610.51) in 2016. The DALYs were high among the male population and in age groups between 20 and 50years. The increase in DALYs in the years 2015 and 2016 compared to 2014 probably resulted from the introduction of ZIKV in Brazil, reinforcing the importance of investments in the prevention of ZIKV infection and in the care of GBS patients.

22 - Inflammatory Mechanisms in Guillain–Barré Syndrome

Introduction Background: Guillain-Barr e syndrome (GBS) usually presents with lower motor neuron (LMN) symmetrical weakness, areflexia, and hypotonia. GBS has rarely been reported with up going plantars’ response. We report a case of GBS with up going plantars during the course of the disease. Citation: Ahmad SF, Al-Bader SY, Hashel JY (2014) Guillain-Barr E Syndrome with Bilateral Extensor Plantar Reflexes. J Neurol Neurol Disord 1(1): 105 . doi: 10.15744/2454-4981.1.105 Case presentation: A 52-year-old woman developed acute paralysis with areflexia following a diarrheal illness. There was no fever at the time of the onset. She developed numbness and asymmetrical weakness of both lower limbs which progressed to involve the upper limbs bilaterally. There were no cranial nerves, respiratory or sphinecteric involvements. Few days later, the plantars’ became upgoing bilaterally, with absent of other upper motor neuron signs. Cerebrospinal fluid (CSF) analysis showed albuminocytological dissociation. Early Nerve conduction study (NCS) showed absent H-reflex dispersed F-waves with prolonged maximum F-latencies and M-amplitude showed no clear block. MRI brain and spine were unremarkable. She was treated with intravenous immunoglobulin (IVIG). Repeated NCS two weeks after the onset, definitely confirmed conduction block. She was able to walk with support three weeks after the onset. Conclusion: GBS should be considered as a differential diagnosis in a patient with acute quadriparesis, even if there is asymmetrical muscle weakness and extensor plantars’’ response. Guillain-Barre syndrome (GBS) is an acute autoimmune polyradiculoneuropathy. Clinical features include progressive, symmetrical ascending muscle weakness usually of more than two limbs, and areflexia with or without sensory, autonomic or brainstem involvements. Weakness is prominent in the lower limbs muscles compared to the upper limbs; there is absence of fever at the onset of neural symptoms [1]. Although, the diagnosis of GBS is based on clinical criteria, the presence of suggestive findings in the nerve conduction studies (NCS) or albuminocytological dissociation in the cerebrospinal fluid (CSF) analysis help to confirm the diagnosis [2].The involvement of the central nervous system (CNS) in the GBS is rare [3]. We report a case of GBS with asymmetrical weakness and up going plantars’ during the course of the disease. A 52-year-old female, known case of diabetes, hypertension, and hypothyroidism, was admitted with acute weakness of both lower limbs. She had history of diarrhea 4 days prior to presentation, which lasted 2 days and improved with symptomatic treatment. Then she developed numbness and weakness of both lower limbs that involved the left side more than the right. The weakness started distally and ascended up proximally. The weakness started to involve the upper limbs 4 days later. There were no cranial nerves, respiratory muscles involvement, nor sphincteric symptoms. There was no fever at the time of onset and no history of recent vaccination. On examination, there was weakness of both lower limbs with hypotonia. The weakness of both lower limbs was asymmetrical with proximal muscle power of 2/5 (Medical Research Council grading) in the left side and 4/5 in the right side, while the power of the distal muscles was 1/5 in the left side and 3/5 in the right side. Examination of the upper limbs showed weakness of distal muscles of 4/5. Deep tendon reflexes were absent all over, and the plantars’ were flexors bilaterally. Decrease in fine touch and temperature sensations were seen in L4, L5, S1 distribution of the left side with loss of joint position and vibration sense bilaterally. There was no cranial nerves involvement. The investigations showed normal total and differential leukocyte counts, erythrocyte sedimentation rate (4 mm/h), creatine kinase level, and electrolytes (Na, Ca, K, Mg, phosphate). Vasculitis and paraneoplastic work up were all negative. CSF examination showed albuminocytologic dissociation. CSF sugar was 6.3 mmol/L (plasma glucose 11 mmol/L), protein was 1517 mg/L, and there are only 2 cells (100% lymphocytes) were detected. All causes of

Guillain–Barré syndrome (GBS) is an immune-mediated polyradiculoneuropathy, which causes acute flaccid paralysis. Most cases of GBS report either a gastrointestinal or respiratory tract infection in the 3–4 weeks preceding symptom onset. Molecular mimicry between the lipopolysaccharides of the infectious organism and the glycosphingolipids or similar nerve antigens in our body triggers the immune response.[1] Martic, et al. have presented an original study on the GBS followed by coronavirus disease 2019 (COVID-19) infection, vaccination, and other precipitating factors during the pandemic in Serbia and Montenegro.[2] They retrospectively recruited 109 GBS patients between January and April 2022 into the following three groups: COVID-19 infection-associated (19 cases), COVID-19 vaccination-associated (16 cases), and other causes (74 cases). Individuals positive for severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) on polymerase chain reaction (PCR) testing were classified as COVID-19-positive. A COVID-19 infection or vaccination was considered a trigger if it occurred between 3 days and 6 weeks before symptom onset. No differences were noted in the clinical presentation, electrophysiology, or outcome between the three groups of GBS patients. Demyelinating neuropathy was the most common electrophysiological subtype, the most common vaccine associated was Sinopharm (8/16 cases), and most events occurred after the first dose. Unlike the Zika virus pandemic, epidemiological studies have not demonstrated COVID-19 infection to be associated with GBS.[3,4] Experts have argued that an increase in GBS incidence might be masked by a decrease in GBS incidence due to other infectious triggers because of better hygiene, lockdown, and social distancing.[5] Studies from Japan and Brazil have reported a decreased incidence of infectious diseases during the lockdown because of these containment measures,[6,7] thereby indirectly reducing the occurrence of GBS. The “GBS consortium,” established in India with 26 centers,[5] found a reduction in the number of GBS cases during the COVID-19 pandemic compared with the same duration in the previous year in both adult[5] and pediatric groups.[8] Similar findings were reported by an epidemiological study conducted in the United Kingdom.[3] These studies[3,5] also reported no significant differences between GBS patients triggered by COVID-19 and those without the infection. Demyelinating GBS is the most common subtype worldwide and was unsurprisingly the most common in this study as well. Older age was associated with a worse prognosis. This was also expected as increasing age is associated with worse GBS outcomes. The relative rarity of COVID-19 vaccine-induced GBS was also in accordance with global data. The authors reported 16 cases of COVID-19 vaccine-induced GBS in the background of 54.1% coverage in adults in a population of 7,806,891 people.[2] The first dose of vaccines was the most common dose implicated, with Sinopharm use associated with most cases. AstraZeneca and Covaxin were the vaccines most commonly used in India, and very few vaccine-associated GBS cases have been reported.[9] A study from Kerala showed a mildly raised incidence of GBS associated with ChAdOx1-S/nCoV-19 vaccination.[10] A retrospective study conducted in the West and North Midlands of the United Kingdom between January and June 2021 reported only 12 cases of COVID-19 vaccine-induced GBS.[11] Most of these individuals had received AstraZeneca vaccines. They reported a higher likelihood of cranial nerve involvement in such GBS cases, similar to Martic, et al.[2] It should be added that this GBS occurrence post-vaccination does not necessarily prove causation. Temporal associations do not imply causality. Also, the COVID-19 vaccine benefits far outweigh the risk of such rare side effects. Finally, the authors mention that the patients were treated with intravenous immunoglobulin (IVIg) and plasma exchange in the standard way. In our experience during the pandemic, the proportion of GBS patients receiving IVIg was significantly higher than that of those receiving plasma exchange (PLEX).[5] The reasons were probably manifold: logistical (greater physician and nursing workforce were working in COVID-19 designated facilities) and difficulty in conducting plasma exchanges within COVID-19 facilities and patient choice (considering they were without accompanying family members in the hospital). The pandemic has shown the need for high-quality prospective longitudinal studies on Guillain–Barré syndrome in low- to middle-income countries. The International Guillain–Barré Syndrome Outcome Study (IGOS) and the recently established GBS consortium in India are good models for replicating in other parts of the world.

Background and Aim: Guillain-Barre syndrome (GBS) described as a syndrome manifesting clinically as an acute inflammatory polyradiculoneuropathy (AIDP) with concomitant, symmetrical muscular weakness with absent or diminished deep tendon reflexes. Autonomic dysfunction (AD) is frequent and clinically important complication of GBS, which potentially contributes to adequate sickness and even mortality. The present study aimed to determine the frequency of AD in GBS patients. Patients and Method: This cross-sectional study investigated 100 patients presenting with new onset Guillain Barre Syndrome in the department of Neurology, Mayo Hospital, Lahore from October 2024 to April 2025. Patients aged 18-65 years of either gender presented with new onset GBS were enrolled. Diagnosis of GBS was established through clinical assessment and supported by relevant investigations, including cerebrospinal fluid analysis and nerve conduction studies. Demographic information and illness duration recorded. All patients were evaluated for the presence of autonomic dysfunction (AD) using a standardized operational definition. Data analysis done using SPSS version 27. Results: The overall mean age was 37.82±4.62 years. Among the 100 patients diagnosed with Guillain-Barre syndrome, autonomic dysfunction (AD) seen in 38% of cases. The most common manifestations of AD included fluctuation in blood pressure (26%), cardiac arrhythmia (18%), and urinary retention (12%). AD seen more often in patients over 40 years of age and in patients with severe motor weakness. No significant gender-based differences in the frequency of AD observed. Conclusion: Autonomic dysfunction (AD) represents a frequent and significant complication in Guillain-Barre syndrome, affecting over one-third of affected individuals. Timely recognition and monitoring of autonomic symptoms are vital, as they play an important role in determining clinical outcomes and are associated with high risks of morbidity and mortality.

Case presentation: An 83-year-old female presented to the Emergency Department with speech alteration, sphincter release, and a fall within the last 24 hours. Examination revealed left-sided hemiparesis, hypoesthesia, and disorientation. Her medical history included systemic arterial hypertension, hypothyroidism, and dyslipidemia, for which she was taking levothyroxine, simvastatin, and losartan. A preliminary diagnosis of stroke in the anterior cerebral region was made, along with a suspicion of Dengue B. Cranial computed tomography (CT) and angio-CT showed no acute lesions or cerebral artery occlusions. She was placed on antiplatelet therapy with clopidogrel and monitored in an acute stroke unit. Laboratory confirmed positive NS1 and a platelet count of 81,000; hence, Clopidogrel was discontinued due to thrombocytopenia. On the fifth day of hospitalization, her neurological deficits worsened, and she developed dyspnea, dysarthria, dysphagia, and tetraparesis. Guillain-Barré syndrome (GBS) was suspected, and further investigation was initiated. Cerebrospinal fluid (CSF) analysis confirmed the diagnosis of GBS by showing albuminocytologic dissociation (1 cell, 72 proteins). Treatment with immunoglobulin was initiated. Despite immunoglobulin treatment, the patient remained globally weak, necessitating plasmapheresis as an additional treatment. During the treatment, the patient required mechanical ventilation and received Meropenem for ventilator-associated pneumonia. After 48 days post-GBS diagnosis, the patient achieved clinical stability and discharged to a rehabilitation facility. Discussion: Dengue, an acute febrile infectious disease endemic in tropical regions, had over 5.2 million cases in Brazil in 2024 transmitted by the mosquito Aedes aegypti, with symptoms ranging from mild (fever, headache, myalgia, arthralgia, rash, and retro-orbital pain) to severe, with 1-25% potential neurological complications like GBS. GBS, an acute polyradiculoneuropathy, often follows infections or vaccination, with a mortality rate of 3-10%. It involves autoimmune mechanisms damaging nerve structures, presenting as ascending muscle weakness and hyporeflexia. Diagnosis includes clinical evaluation, CSF analysis, electromyography, and imaging. Treatment comprises immunoglobulins and plasmapheresis. The association between GBS and dengue is rarely documented in the literature. Nevertheless, GBS can occur as a neurological complication of dengue fever, especially with DENV-2 and DENV-3 serotypes. Inflammatory cytokines from the immune response to dengue may increase blood-brain barrier permeability, contributing to GBS pathogenesis. Moreover, cross-reactive IgM or IgG antibodies against flavivirus antigens are more common in GBS patients. Final comments: GBS, characterized by muscle weakness and immune system involvement, can occur post-infection, with dengue being one of the triggering viruses.

Objective To investigate the clinical features of Guillain-Barre syndrome (GBS) and clarify the long-term prognosis of acute inflammatory demyelinating polyneuropathy (AIDP) and acute motor axonal neuropathy (AMAN). Methods We conducted an analysis of the clinical data of 50 patients with GBS treated in our hospital between 2003 and 2007. According to the eleetrophysiological criteria, the eases were classified into AIDP (n=24) and AMAN eases (n=22), with 4 eases of unknown classification. The patients unable to walk upon discharge were followed up for more than 6 months, and the clinical features and prognosis of the two groups were compared. Results The age, gender, cranial nerve involvement, Hughes grade at the initial neurological examination and at the time of symptom peak did not differ significantly between the patients with AIDP and AMAN (P>0.05), and most of the AMAN patients had a good recovery. The number of patients capable of walking at one month after the onset was comparable between the two groups (P>0.05). In AMAN group, the percentages of patients with slow recovery and those having rapid recovery were significantly higher than those in AIDP group (P<0.05). Conclusion The clinical recovery of AMAN patients can be either rapid or prolonged, and rigorous immunotherapy should be administered to achieve early recovery and ensure more favorable outcomes of the patients. Key words: Guillain-Barre syndrome; Acute inflammatory demyelinating polyneuropathy; Acute motoraxonal neuropathy

Guillain-Barré syndrome (GBS) is an acute polyradiculoneuropathy. Symptoms may vary greatly in presentation and severity. Besides weakness and sensory disturbances, patients may have cranial nerve involvement, respiratory insufficiency, autonomic dysfunction and pain. To develop an evidence-based guideline for the diagnosis and treatment of GBS, using Grading of Recommendations, Assessment, Development and Evaluation (GRADE) methodology a Task Force (TF) of the European Academy of Neurology (EAN) and the Peripheral Nerve Society (PNS) constructed 14 Population/Intervention/Comparison/Outcome questions (PICOs) covering diagnosis, treatment and prognosis of GBS, which guided the literature search. Data were extracted and summarised in GRADE Summaries of Findings (for treatment PICOs) or Evidence Tables (for diagnostic and prognostic PICOs). Statements were prepared according to GRADE Evidence-to-Decision (EtD) frameworks. For the six intervention PICOs, evidence-based recommendations are made. For other PICOs, good practice points (GPPs) are formulated. For diagnosis, the principal GPPs are: GBS is more likely if there is a history of recent diarrhoea or respiratory infection; CSF examination is valuable, particularly when the diagnosis is less certain; electrodiagnostic testing is advised to support the diagnosis; testing for anti-ganglioside antibodies is of limited clinical value in most patients with typical motor-sensory GBS, but anti-GQ1b antibody testing should be considered when Miller Fisher syndrome (MFS) is suspected; nodal-paranodal antibodies should be tested when autoimmune nodopathy is suspected; MRI or ultrasound imaging should be considered in atypical cases; and changing the diagnosis to acute-onset chronic inflammatory demyelinating polyradiculoneuropathy (A-CIDP) should be considered if progression continues after 8 weeks from onset, which occurs in around 5% of patients initially diagnosed with GBS. For treatment, the TF recommends intravenous immunoglobulin (IVIg) 0.4 g/kg for 5 days, in patients within 2 weeks (GPP also within 2-4 weeks) after onset of weakness if unable to walk unaided, or a course of plasma exchange (PE) 12-15 L in four to five exchanges over 1-2 weeks, in patients within 4 weeks after onset of weakness if unable to walk unaided. The TF recommends against a second IVIg course in GBS patients with a poor prognosis; recommends against using oral corticosteroids, and weakly recommends against using IV corticosteroids; does not recommend PE followed immediately by IVIg; weakly recommends gabapentinoids, tricyclic antidepressants or carbamazepine for treatment of pain; does not recommend a specific treatment for fatigue. To estimate the prognosis of individual patients, the TF advises using the modified Erasmus GBS outcome score (mEGOS) to assess outcome, and the modified Erasmus GBS Respiratory Insufficiency Score (mEGRIS) to assess the risk of requiring artificial ventilation. Based on the PICOs, available literature and additional discussions, we provide flow charts to assist making clinical decisions on diagnosis, treatment and the need for intensive care unit admission.