Complex Interplay of COVID-19 ARDS with Guillain-Barré Syndrome and Cerebral Infarction: A Case Study.

BACKGROUND Coronavirus disease (COVID-19) can cause various complications. We report a case of severe COVID-19 acute respiratory distress syndrome (ARDS) in a patient receiving veno-venous extracorporeal membrane oxygenation (V-V ECMO), complicated by Guillain-Barre syndrome (GBS) and cerebral infarction, as well as pulmonary embolism. CASE REPORT A 55-year-old Japanese man with a history of ulcerative colitis was admitted for COVID-19. His respiratory status worsened and progressed to ARDS, requiring intubation on hospital day (HD) 3. On HD 16, contrast computed tomography revealed PE. On HD 18, his respiratory condition worsened, and V-V ECMO was initiated. On HD 23, V-V ECMO was successfully discontinued. He regained consciousness on HD 44, but he had quadriplegia. Deep-tendon reflexes were absent in all limbs. Cranial nerve involvement, specifically bilateral facial nerve weakness, was noted. Magnetic resonance imaging showed bilateral scattered cerebral infarctions on HD 76. Nerve conduction studies indicated severe axonal neuropathy. Cerebrospinal fluid examination showed albuminocytologic dissociation. The antibody to the ganglioside GD1a was positive. These findings were consistent with the diagnosis of GBS. He received immunoglobulin treatment on HD 89, and his neurological findings slightly improved. CONCLUSIONS This study emphasized that in COVID-19, neurological complications are not rare, are difficult to diagnose, and are prone to delays in detection.

Therapeutic Plasma Exchange in Neurological Conditions: An Observation from the Eastern Part of India.

INTRODUCTION: Therapeutic plasma exchange (TPE) is an extracorporeal process of separation of plasma from the cellular components of blood and its replacement with analogous fluids. This process is effective in treatment of disease conditions from dysregulation of the humoral immune system by removal of various humoral pathogenic substances like antibodies, immune complexes, monoclonal proteins, toxins or cytokine(s) and/or the replenishment of a specific plasma factor. AIM AND OBJECTIVE: To evaluate major indications of therapeutic plasma exchange in neurological disorders. To identify major complications associated and factors associated with premature cessation of the therapeutic plasma exchange cycle. Materials and Methods: This is a hospital based retrospective study conducted by analyzing medical records of patients, who had undergone therapeutic plasma exchange (TPE) for various neurological disorders at IMS & SUM hospital. Medical records total 118 patients who underwent TPE from January 2016 to December 2021 were analyzed. The demographic data, blood group pattern and indications for TPE were enumerated from the records. Various complications of TPE and reasons for incomplete TPE cycle were documented and analyzed. RESULTS: A total of 508 TPE procedures were performed on 118 patients. In this study 61 patients were male and 57 patients were female. O-blood group was commonest blood group among the patients. GBS is the commonest indication of TPE. 57.6 % of patients could complete all sessions TPE cycle. Blockage of vascular access is the commonest cause of incomplete TPE session. Cramps (33%) and mild transient hypotension (27.1%0 were the commonest complications observed. CONCLUSION: TPE is a safe and effective treatment option for various immune-mediated neurological disorders and should be considered in managing these disorders.

Résumé Introduction:L'échange de plasma thérapeutique (TPE) est un processus extracorporel de séparation du plasma des composants cellulaires du sang et de son remplacement par des fluides analogues. Ce processus est efficace dans le traitement des conditions de la maladie à partir de la dérégulation du système immunitaire humoral par élimination de diverses substances pathogènes humorales comme les anticorps, les complexes immunitaires, les protéines monoclonales, les toxines ou les cytokines et / ou la réapprovisionnement d'un facteur plasmatique spécifique. Objectif et objectif: évaluer les principales indications de l'échange de plasma thérapeutique dans les troubles neurologiques. Pour identifier les complications majeures associées et les facteurs associés à la cessation prématurée du cycle d'échange de plasma thérapeutique.Matériel et méthodes:Il s'agit d'une étude rétrospective en milieu hospitalier menée en analysant les dossiers médicaux des patients, qui avaient subi un échange de plasma thérapeutique (TPE) pour divers troubles neurologiques de l'hôpital IMS & Sum. Les dossiers médicaux au total 118 patients ayant subi un TPE de janvier 2016 à décembre 2021 ont été analysés. Les données démographiques, le modèle de groupe sanguin et les indications pour le TPE ont été énumérés à partir des enregistrements. Divers Les complications du TPE et les raisons du cycle TPE incomplet ont été documentées et analysées.Résultats:Un total de 508 procédures TPE ont été effectuées sur 118 patients. Dans cette étude, 61 patients étaient des hommes et 57 patients étaient des femmes. Le groupe de sang O était le groupe sanguin le plus commun chez les patients. Le GBS est l'indication la plus courante du TPE. 57,6% des patients pourraient terminer toutes les séances du cycle TPE. Le blocage de l'accès vasculaire est la cause la plus courante de la session TPE incomplète. Les crampes (33%) et l'hypotension transitoire légère (27,1% 0 étaient les complications les plus courantes observées.Conclusion:TPE est une option de traitement sûre et efficace pour divers troubles neurologiques à médiation immunitaire et doit être pris en compte dans la gestion de ces troubles.

Controversies in COVID-19: Diagnostic Evaluation and Management in Neuro-COVID.

SARS-CoV-2 is a virus in the coronavirus family originating out of Wuhan, China clinically known as COVID-19. While traditionally thought of as a respiratory virus, COVID-19 can have a multi-organ impact due to its invasion and widespread distribution throughout the body and via the angiotensin converting enzyme. Neurologic events due to COVID-19 are common, especially in the critically ill, and are called Neuro-COVID. Among these events are peripheral and central nervous system diseases such as Guillain-Barré, ischemic stroke, and various types of encephalitis. The impact of Neuro-COVID is devastating and is often far more severe than its non-COVID-19 form. Immunosuppressive or immunomodulatory therapy is often a mainstay of treatment, such as for encephalitis and Guillain-Barré, respectively, while management may fall in line with conventional processes in most cases, such as ischemic stroke. Much remains to be studied about the evaluation and management of Neuro-COVID.

[Immune-mediated neuropathies: pathophysiology and management].

Immune-mediated neuropathies (IMN) are a heterogenous group of disorders affecting the peripheral nervous system, due to dysregulation of the immune system. It mainly includes Guillain-Barre syndrome, chronic inflammatory demyelinating polyradiculoneuropathy, multifocal motor neuropathy and so on. Most of these diseases can be clinically improved by appropriate immunotherapy, but some patients still have unsatisfactory results. Therefore, studying the pathophysiology of the occurrence and development of diseases can reveal the nature of diseases and provide a theoretical basis for the prevention, diagnosis and treatment of diseases. In this paper, the pathophysiological mechanism of various IMNs is described in detail, with emphasis on immunological mechanism, and the progress of diagnosis and treatment of various IMNs is briefly introduced.

A short-term functional recovery comparison of therapeutic plasma exchange and immunoadsorption in severe acute neuroimmune diseases.

OBJECTIVE: To compare the differential impact of recombinant protein A immunoadsorption (PAIA) or therapeutic plasma exchange (TPE) on neurological functional improvement and quality of life in patients afflicted with severe acute neuroimmune diseases, including Guillain-Barré syndrome (GBS), myasthenia gravis (MG), neuromyelitis optica spectrum disorder (NMOSD), and anti-NMDA receptor encephalitis (NMDARE). METHODS: The retrospective study included 29 patients with moderate to severe disability (modified Rankin scale, mRS≥3) due to acute neuroimmune diseases at the second Xiangya hospital from January 2021 to January 2023. The clinical efficacy of PAIA and TPE in improving neurological function (ΔmRS≥1) and the difference in favorable functional outcomes (mRS 0-2) at three months were evaluated. The impact of both treatments on patients' health-related quality of life (HRQoL) was assessed using a visual analog scale (EQ-VAS) score ranging from 0 to 100. RESULTS: The findings revealed that the PAIA group exhibited a significantly higher rate of improvement in modified Rankin scale (mRS) scores (ΔmRS≥1) at the three-month follow-up compared to the TPE group (94.4 % vs. 54.5 %, p = 0.018). However, no statistically significant difference was observed between the two treatment modalities in terms of favorable neurological functional outcomes at the three-month mark. Furthermore, the PAIA group demonstrated a significantly higher EQ-VAS score at 14 days post-treatment compared to the TPE group (60.0 vs. 47.7, p = 0.017). CONCLUSION: In the short-term management of severe acute neuroimmune diseases, PAIA may present a greater probability of improving neurological function and facilitating an earlier enhancement of quality of life compared to TPE.

Multiple cerebral infarctions after intravenous immunoglobulin for Guillain-Barré syndrome: two case reports and review of the literature.

Background: Guillain-Barré syndrome (GBS) is a polyradiculoneuropathy mediated by the immune system and is the primary reason for acute flaccid paralysis. Intravenous immunoglobulin (IVIg) is a recognized immunotherapeutic drug that can accelerate recovery from GBS. Limited literature exists concerning cerebral infarction complications with IVIg following its use in the treatment of GBS. Case presentation: A patient was diagnosed with the acute inflammatory demyelinating polyradiculoneuropathy subtype of GBS, while another patient was diagnosed with the acute bulbar palsy variant of GBS 2 years prior and experienced a relapse of GBS. Both patients received immunoglobulin therapy, during which multiple acute cerebral infarctions were detected using magnetic resonance imaging. Both patients had a history of coronary artery atherosclerotic heart disease and vertebral artery stenosis, and D-dimer and fibrinogen degradation products were significantly elevated after immunoglobulin therapy. Conclusions: The risk of cerebral infarction associated with IVIg is generally low in patients with different GBS variants. Nevertheless, the occurrence of cerebral infarction associated with IVIg might not be insignificant in older patients with vascular risk factors and should be carefully monitored.

Clinical Characteristics and Prognosis of ICU-Admitted Patients with Guillain-Barre Syndrome: A Report from a Large Teaching Hospital in South Iran.

Background: Guillain-Barre Syndrome (GBS) is the most prevalent acute peripheral polyneuropathy disorder. The disparities between populations and variations in the major risk factors highlight the importance of country-specific studies. This study aimed to report clinical characteristics and outcomes of ICU-admitted patients with GBS in an academic medical center in Iran. Methods: The data were collected retrospectively from all patients with GBS admitted to Namazi Hospital, affiliated with Shiraz University of Medical Sciences, (Shiraz, Iran), between March 2016 to March 2021. Specialized neurological information and the Acute Physiology and Chronic Health Evaluation (APACHE II) score were recorded. The SPSS software was used to analyze the data. The analyzed data were reported as numbers and percentages, or mean±SD, or median(Interquartile). Results: The study included 132 GBS patients, with an average age of 47.87±15.4 years and a male-to-female ratio of 1.69:1. More than half of the patients (58.5%) were classified as having an axonal disease. In patients with axonal illness, 51.4% of patients had lower limb powers<3, while only 36% of those had the demyelinating disease. This group also required mechanical ventilation more frequently (54% vs. 46%) and for a longer duration (26 [9-37] vs. 10 [1-61]) days. Pneumonia and sepsis were each observed in 16% of patients, and 12% developed a urinary tract infection. The most common type of GBS was acute inflammatory demyelinating polyneuropathy (AIDP). Only 6 (3.8%) patients died. Conclusion: The axonal type of GBS was more frequent, and these patients required mechanical ventilation more frequently and for a longer duration than those in other electrophysiological categories. A preprint version of the manuscript is available at DOI: https://doi.org/10.21203/rs.3.rs-2181605/v1.

Emerging treatment landscape for Guillain-Barré Syndrome (GBS): what's new?

INTRODUCTION: Guillain-Barré syndrome (GBS) is a monophasic immune neuropathic disorder characterized by acute paralysis. A significant portion of patients are left with residual deficits, which presents a considerable global healthcare challenge. The precise mechanisms underlying GBS pathogenesis are not fully elucidated. Recent studies have focused on postinfectious molecular mimicry and identified involvement of IgG autoantibodies and innate immune effectors in GBS. Intravenous immunoglobulins (IVIg) and plasma exchange (PE) are two established evidence-based immunomodulatory treatments for GBS, but a significant proportion of GBS patients fails to respond adequately to either therapy. This emphasizes an urgent need for novel and more potent treatments. AREAS COVERED: We discuss novel immunomodulatory therapies presently at different phases of preclinical and clinical investigation. Some drugs in development target pathophysiologic mechanisms such as IgG autoantibody catabolism and complement activation, which are relevant to GBS. EXPERT OPINION: There is an unmet need for more effective immune therapies for GBS. New immunomodulatory therapies under development may provide more potent options for GBS patients who do not respond to IVIg or PE. Future directions may include incorporating neuroprotective interventions based on evolving understanding of mechanisms underlying nerve injury and axonal degeneration.

A retrospective analysis of the clinical profile and factors associated with mortality and poor hospital outcomes in adult Guillain-Barre syndrome patients.

Guillain-Barré syndrome (GBS) is an acute autoimmune polyneuropathy with substantial geographic variations in demography, antecedent events, clinical manifestations, electrophysiological sub-types, diagnostic findings, treatment modalities, and prognostic indicators. However, there is limited contemporary data on GBS patient profiles and prognostic factors from low-resource settings like Ethiopia. The objective of this study is to investigate the clinical profile, factors associated with mortality, and hospital outcomes among GBS patients admitted to Tikur Anbessa Specialized Hospital (TASH) in Addis Ababa, Ethiopia. A retrospective cross-sectional study was conducted among 60 GBS patients admitted to TASH from January 2018 to December 2022. Data on demographics, clinical features, treatments, complications, and outcomes were extracted from medical records. Bivariate and multivariate logistic regression analyses identified factors associated with mortality and poor hospital outcomes. The cohort had a mean age of 28.5 years, with 76.7% aged 14-34 years. Males comprised 61.7% of cases. Ascending paralysis (76.7%) was the predominant presentation. Absent or reduced reflexes were seen in 91.7% of patients. The most common antecedent event was gastroenteritis (26.7%), followed by upper respiratory tract infection (URTI) (15%) and vaccination (11.7%). The mean interval from symptom onset to hospital presentation was 8.77 days, and the peak symptom severity was 4.47 days. The axonal variant (75.5%) was the most common subtype, followed by the demyelinating variant (24.5%). Intravenous immunoglobulin was administered to 41.7% of patients. Respiratory failure requiring invasive mechanical ventilator (MV) support occurred in 26.7% of cases. The mortality rate was 10%, with mechanical ventilation being the only factor significantly associated with mortality (95% CI 2.067-184.858; P < 0.010). At discharge, 55% had a good outcome, and 45% had a poor outcome, according to the Hughes Functional Disability Scale (HFDS). Mechanical ventilation (AOR 0.024, 95% CI 0.001-0.607) and a GBS disability score > 3 (AOR 0.106, 95% CI 0.024-0.467) were factors significantly associated with poor hospital outcomes. GBS in this cohort primarily affected individuals of young age, commonly preceded by gastroenteritis and characterized by a high frequency of the axonal variant. Mechanical ventilation was found to be significantly linked to mortality. Alongside mechanical ventilation requirements, severe disability upon presentation emerged as a crucial determinant of poor outcomes upon discharge, underscoring the importance of early identification of high-risk patients and prompt interventions.

Clinical presentation and symptomatology of Guillain-Barré syndrome: A literature review.

Guillain-Barré Syndrome (GBS) is a rare but potentially life-threatening neurological disorder characterized by acute onset ascending paralysis and sensory abnormalities. This article provides a comprehensive overview of GBS, covering its epidemiology, etiology, clinical presentation, diagnostic evaluation, management and treatment, prognosis, psychosocial impact, recent advances in research, public health implications, and ethical considerations. Epidemiological data reveal variations in GBS prevalence, incidence rates, and geographical distribution influenced by climate, infectious disease prevalence, and genetic susceptibility. Etiological factors include preceding infections, vaccinations, and autoimmune mechanisms, although the precise pathophysiology remains incomplete. Clinical presentation encompasses prodromal symptoms, motor deficits, sensory abnormalities, autonomic dysfunction, and variants such as Miller-Fisher Syndrome and Bickerstaff brainstem encephalitis. Neurological examination findings include weakness, paralysis, sensory deficits, and reflex changes, while autonomic dysfunction manifests as cardiovascular, respiratory, and gastrointestinal symptoms. Diagnostic evaluation relies on clinical criteria, laboratory tests (e.g., cerebrospinal fluid analysis, nerve conduction studies), and consideration of differential diagnoses. Management strategies encompass supportive care, immunomodulatory therapies (e.g., intravenous immunoglobulin, plasma exchange), and rehabilitation interventions to optimize functional outcomes and promote recovery. Prognosis varies depending on clinical features, treatment response, and complications such as respiratory failure and autonomic instability. Psychosocial impact encompasses psychological effects on patients and caregivers, highlighting the importance of coping strategies and support systems. Recent advances in research focus on emerging treatments, genetic predisposition, and biomarker discovery, offering promise for improving GBS outcomes. Public health implications include vaccination safety concerns and healthcare system considerations for GBS management. Ethical considerations encompass patient autonomy, resource allocation, and end-of-life decision-making.

[Guillain-Barre syndrome after allogeneic hematopoietic stem cell transplantation: a case report and literature review].

Guillain-Barre syndrome rarely develops after allogeneic hematopoietic stem cell transplantation (allo-HSCT), and only a few reports exist in China. Guillain-Barre syndrome is an acute and life-threatening condition that requires early diagnosis and treatment. A patient with acute myeloid leukemia underwent allogeneic HSCT for >5 months and gradually developed limb muscle weakness and limited eye movement after coexisting with delayed acute intestinal graft-versus-host disease. After the examination of cerebrospinal fluid and electromyography, the diagnosis of Guillain-Barre syndrome was confirmed. After a high-dose intravenous immunoglobulin (IVIg) treatment, muscle strength gradually recovered, and the prognosis was good.

Guillain-Barré Syndrome and Encephalitis Following a Cytomegalovirus Infection in an Immunocompetent Adult: A Case Report.

BACKGROUND Cytomegalovirus (CMV) is a common herpesvirus that often causes asymptomatic or mild infections. In immunocompromised patients, CMV can lead to severe complications, including Guillain-Barre syndrome (GBS) and encephalitis. While these conditions have been described in the immunocompetent population, simultaneous presentation of CMV-associated GBS and encephalitis in such individuals has not been previously reported. CASE REPORT We present a case of a 58-year-old woman with poorly controlled diabetes who developed concurrent GBS and encephalitis following a CMV infection. The patient experienced bilateral ascending paraparesis 1 week after self-limited gastrointestinal symptoms. Despite initial treatment with plasma exchange therapy, her condition deteriorated with altered mental status and generalized tonic-clonic seizures, necessitating orotracheal intubation. Laboratory analysis revealed the presence of CMV in her cerebrospinal fluid. After treatment with further sessions of plasma exchange therapy and ganciclovir, her muscular strength in the extremities improved. However, she developed acute lung edema and failed extubation, leading to cardiorespiratory arrest with neurological sequelae. Palliative care was institutionalized, and she died 2 weeks later due to pneumonia. CONCLUSIONS This case highlights an unusual clinical presentation of overlapping CMV-associated GBS and encephalitis in an immunocompetent individual, with diabetes as the only identified risk factor. It underscores the importance of considering CMV as a potential etiological factor in such complex cases and the need for prompt diagnosis to improve patient outcomes. Further research is warranted to explore the underlying mechanisms and implications of this rare overlapping neurological manifestation.

Risk factors and outcome of hyponatremia in patients with Guillain-Barré syndrome.

The objective of the present study was to evaluate the risk factors and outcomes associated with hyponatremia in patients with Guillain-Barré syndrome (GBS). We retrospectively studied 80 consecutive patients with GBS who visited our hospital and compared clinical, laboratory, and electrophysiological findings of patients with and without hyponatremia. Disability was evaluated using the Hughes grading system. Of the 80 patients, 18 (23%) had hyponatremia. Hyponatremia was significantly associated with older age (P = 0.003), urinary retention (P < 0.0001), Hughes grade ≥ 4 at admission and nadir (P = 0.003 and P < 0.001, respectively), acute inflammatory demyelinating polyneuropathy subtype (P = 0.017), sepsis (P = 0.001), mechanical ventilator support (P = 0.013), longer hospitalization length of stay (P < 0.0001), and inability to walk independently at 6 months (P < 0.001). Multivariate analysis performed to assess the risk factors of hyponatremia revealed that urinary retention (odds ratio [OR] 30.7, 95% confidence interval [CI] 3.6-264.4; P = 0.002) and mechanical ventilator support (OR 13.8, 95% CI 1.6-118.0; P = 0.017) were significant independent risk factors of hyponatremia. In assessing the outcomes of patients with hyponatremia, multivariate analysis showed that hyponatremia was independently associated with hospitalization length of stay ≥ 60 days and inability to walk independently at 6 month, with the former showing statistical significance but the latter not (OR 9.3, 95% CI 1.8-47.7; P = 0.007 and OR 4.9, 95% CI 0.9-26.3; P = 0.066, respectively). Therefore, we demonstrate that, along with mechanical ventilator support, urinary retention-possibly indicating autonomic dysfunction-is a risk factor of hyponatremia in GBS. Moreover, we confirm that hyponatremia is associated with poor outcome in GBS.

Successful Combination of Fresh Frozen Plasma and Albumin 5% in Plasma Exchange for a Patient with Concurrent Thyroid Storm and Guillain-Barré Syndrome: A Rare Case Report.

Thyroid storm is an endocrine emergency, and treatment must ensure primary goals, including reducing the production and release of thyroid hormones, mitigating the effects of thyroid hormones, increasing the elimination of thyroid hormones, treating systemic disturbances, and managing triggering factors. However, in a few cases where thyroid storm does not respond to initial treatment, therapeutic plasma exchange (TPE) should be considered. A 50-year-old male patient was admitted to the University Medical Center Ho Chi Minh City due to hypotonia and sensory disturbances gradually spreading from the lower extremities to the entire body. The patient was diagnosed with Guillain-Barré syndrome (GBS) and newly discovered hyperthyroidism. During the treatment course, the patient developed hospital-acquired pneumonia, acting as a trigger factor for a thyroid storm. Despite aggressive treatment for thyroid storm, the patient's condition worsened, leading to the decision to perform TPE. The replacement fluid was a combination of fresh frozen plasma (FFP) and albumin 5%. Subsequently, the patient returned to a euthyroid state and was discharged. Combining FFP and albumin 5% in TPE advantages FFP's high thyroid hormones-binding capacity and albumin's cost-effectiveness, safety, and efficiency. This reduces the drawbacks associated with high volumes of FFP and offers a balanced and effective approach to managing thyroid storms. Moreover, the concurrent presence of GBS and thyroid storm is extremely rare. Through this case, we aim to discuss the role of TPE in the treatment of thyroid storms and the effectiveness of the combination of FFP and albumin 5% as the replacement fluid.

Navigating the complexity of Guillain-Barré syndrome and Miller-Fisher syndrome overlap syndrome: a pediatric case report.

Guillain-Barré syndrome/Miller-Fisher syndrome (GBS/MFS) overlap syndrome is an extremely rare variant of Guillain-Barré syndrome (GBS) in which Miller-Fisher syndrome (MFS) coexists with other characteristics of GBS, such as limb weakness, paresthesia, and facial paralysis. We report the clinical case of a 12-year-old patient, with no pathological history, who acutely presents with ophthalmoplegia, areflexia, facial diplegia, and swallowing and phonation disorders, followed by progressive, descending, and symmetrical paresis affecting first the upper limbs and then the lower limbs. An albuminocytological dissociation was found in the cerebrospinal fluid study. Magnetic resonance imaging of the spinal cord showed enhancement and thickening of the cauda equina roots. The patient was treated with immunoglobulins with a favorable clinical outcome.

Caesarean section for a primipara with Guillain-Barré syndrome under combined spinal epidural anaesthesia.

A primigravida in mid 30s presented to hospital at 30+2 weeks gestation, due to progressive neurological symptoms including ascending limb weakness and paraesthesia bilaterally as well as dysphagia, facial weakness and dysphasia.The patient was diagnosed with Guillain-Barré syndrome after physical examination and electromyography, which showed a patchy demyelinating sensorimotor polyneuropathy. The patient underwent a 5-day course of intravenous immunoglobulin, beginning the day after admission. Markers of severity including forced vital capacity improved thereafter until delivery.With limited evidence favouring one particular anaesthetic technique in parturients with Guillain-Barré syndrome, combined spinal epidural anaesthesia was preferred over general anaesthesia in order to avoid the potential for prolonged intubation postoperatively and to allow careful titration of neuraxial blockade. Delivery by caesarean section at 34+1 weeks due to pre-eclampsia was uncomplicated. Thereafter the patient's condition deteriorated, requiring a further 5-day course of intravenous immunoglobulin with symptoms gradually improving over a 6-month admission.

New chronic inflammatory demyelinating polyneuropathy/Guillain-Barré syndrome guidelines - impact on clinical practise.

PURPOSE OF REVIEW: There is no diagnostic biomarker that can reliably detect Guillain-Barré syndrome (GBS) or chronic inflammatory demyelinating polyneuropathy (CIDP). Diagnosis relies upon integrating key clinical characteristics and relevant supportive data. Consequently, misdiagnosis and delayed diagnosis are common. Diagnostic criteria have proven valuable resources to improve diagnosis, but are underutilized during routine clinical care. RECENT FINDINGS: In 2021, the EAN/PNS CIDP criteria was published, and were followed by the EAN/PNS GBS criteria in 2023. Both guidelines utilized GRADE methodology to formulate evidence-based recommendations that are intended to be used by adult and paediatric clinicians across diverse care settings to optimize diagnostic accuracy and improve patient outcomes during routine clinical care. SUMMARY: The EAN/PNS GBS and CIDP criteria detail specific clinical, electrophysiological, and laboratory features that raise diagnostic confidence, and call attention to diagnostic mimics. The sensitivity of EAN/PNS and other modern criteria to detect GBS and CIDP is high, but utilization during clinical practice is low. Complexity is one factor limiting widespread application. Strategies are needed to optimize criteria adoption during routine clinical care such that GBS and CIDP diagnosis can be achieved with greater speed and accuracy.

[Fisher Syndrome].

Fisher syndrome is recognized as a variant of Guillain-Barré syndrome, encompassing acute onset immune-mediated neuropathies marked by the classical triad of ataxia, areflexia, and ophthalmoplegia. Generally, Fisher syndrome follows a self-limited course with a good prognosis. Ophthalmoplegia, typically bilateral, progresses to complete external ophthalmoplegia within 1-2 weeks. Ataxia, often very severe, may cause an inability to walk without support despite normal strength. Fisher syndrome is also frequently concomitant with additional clinical features, including ptosis, internal ophthalmoplegia, facial nerve palsy, sensory deficits, and bulbar palsy. The confirmation of an antecedent infection is often established. Among the ganglioside antibodies, anti-GQ1b antibodies exhibit positivity in over 80% of patients. The syndrome manifests in three distinct types: a partial subtype exhibiting only a subset of the triad symptoms, Bickerstaff's brainstem encephalitis marked by impaired consciousness and pyramidal tract signs, and an overlapping subtype with Guillain-Barré syndrome, characterized by weakness in the extremities.

[Guillain-Barré syndrome].

Guillain-Barré syndrome (GBS), an acute immune-mediated neuropathy, occurs following immunological stimulation, such as infection, with complement-mediated neuropathy implicated in the pathophysiology of this condition. Glycolipid antibodies produced by molecular mimicry are detected in approximately 60% of cases. Recent studies have suggested the role of cell-mediated immunity in the pathogenesis of GBS. Intravenous immunoglobulin and plasma exchange are established immunotherapies. In this article, based on the latest knowledge, we describe the pathophysiology, diagnosis, treatment, prognosis, and prognostic prediction of GBS. Furthermore, we discuss some GBS guidelines published by the European Academy of Neurology/Peripheral Nerve Society.

PRESenting a Challenge: Posterior Reversible Encephalopathy Syndrome in Pediatric Patients With Guillain-Barré Syndrome: A Case Series and Review of Literature.

BACKGROUND: Guillain-Barré syndrome (GBS) is an autoimmune disorder characterized by demyelination of peripheral nerves. GBS-associated posterior reversible encephalopathy syndrome (PRES) is a rare and potentially life-threatening complication in the pediatric population. We aimed to report and analyze the clinical features, management, and outcomes of three cases of GBS-associated PRES in our setting in the light of the existing literature. METHODS: Medical records of 75 pediatric patients with GBS were reviewed for autonomic changes and GBS-associated PRES. Thirty-one developed dysautonomia while three were identified to have PRES. Clinical, radiological, laboratory, and treatment data were collected and analyzed. RESULTS: All three patients were male and presented with symptoms of acute flaccid paralysis and respiratory distress requiring mechanical ventilation. All three patients experienced various complications, including hypertension, seizures, and hyponatremia, and were subsequently diagnosed with PRES. Multimodal intensive care resulted in patient improvement and discharge in an ambulatory state after an average of 104 days of care. CONCLUSIONS: GBS-associated PRES is a rare and potentially life-threatening complication that can occur in pediatric patients with GBS. Our findings suggest that early recognition, prompt intervention, and multimodal intensive care can improve patient outcomes. Further studies are needed to determine optimal treatment strategies for GBS-associated PRES.