Antecedent Infection Research Articles

Outcomes in hematopoetic cell transplantation in the setting of mold infections in patients with chronic granulomatous disease.

Chronic granulomatous disease (CGD) is a disorder of immunity characterized by phagocyte dysfunction. Mold infections in patients with CGD are often severe and disseminated. We present patient characteristics, microbiological data, and outcomes for 26 patients with CGD who received hematopoietic cell transplantation (HCT) or gene therapy-modified cells (GT) between 2008 and 2019, with proven fungal infection either before or during their transplant. All patients engrafted, and all but one GT recipient had neutrophil recovery and evidence of functional correction. Eighteen patients (69%) are currently alive and 19 patients (73% of total, 90% of patients with repeat imaging performed) had evidence of radiographic improvement. With 3 exceptions, deaths were not principally related to the fungal infection and duration of antecedent infection did not correlate with death. Aspergillus species accounted for the majority of disease (50%), followed by Phellinus species (18%). Osteomyelitis and disseminated disease were common, as only 11 patients (42%) had disease restricted to pneumonia. Triazole therapy was used in all 26 patients, with combination therapy used in 25 (96%). HCT or gene therapy, with appropriate antifungal therapy, are viable therapies for refractory fungal infections in patients with CGD.

The spectrum of anti-GQ1B antibody syndrome: beyond Miller Fisher syndrome and Bickerstaff brainstem encephalitis.

Since the initial identification of Miller Fisher syndrome (MFS) and Bickerstaff brainstem encephalitis (BBE),significant milestones have been achieved in understanding these diseases.Discoveries of common serum antibodies (IgG anti-GQ1b), antecedent infections, neurophysiological data, andneuroimaging suggested a shared autoimmune pathogenetic mechanism rather than distinct pathogenesis, leadingto the hypothesis that both diseases are part of a unified syndrome, termed "Fisher-Bickerstaff syndrome". The subsequent identification of atypical anti-GQ1b-positive forms expanded the classification to a broader condition known as "Anti-GQ1b-Antibody syndrome". An exhaustive literature review was conducted, analyzing a substantial body of research spanning from the initialdescriptions of the syndrome's components to recent developments in diagnostic classification and researchperspectives. Anti-GQ1b syndrome encompasses a continuous spectrum of conditions defined by a common serological profilewith varying degrees of peripheral (PNS) and central nervous system (CNS) involvement. MFS and BBE represent theopposite ends of this spectrum, with MFS primarily affecting the PNS and BBE predominantly involving the CNS.Recently identified atypical forms, such as acute ophthalmoparesis, acute ataxic neuropathy withoutophthalmoparesis, Guillain-Barré syndrome (GBS) with ophthalmoparesis, MFS-GBS and BBE-GBS overlap syndromes,have broadened this spectrum. This work aims to provide an extensive, detailed, and updated overview of all aspects of the anti-GQ1b syndromewith the intention of serving as a stepping stone for further shaping thereof. Special attention was given to therecently identified atypical forms, underscoring their significance in redefining the boundaries of the syndrome.

Photographic grading to evaluate facial cleanliness and trachoma among children in Amhara region, Ethiopia.

Promotion of facial cleanliness is recommended for the elimination of blinding trachoma, largely because of observational studies that have found an association between various measures of facial uncleanliness and trachoma. However, when a field grader assesses both facial cleanliness and trachoma, associations may be biased. Assessment of photographs of the face and conjunctiva by masked graders may provide a less biased estimate of the relationship between facial cleanliness and trachoma. Face photographs, conjunctival photographs, and conjunctival swabs were obtained on a random sample of 0-9-year-old children from each of 40 communities in Amhara region, Ethiopia. Face photographs were assessed for the presence of seven measures of an unclean face (i.e., wet nasal discharge, dry nasal discharge, wet ocular discharge, dry ocular discharge, food, dust/dirt, and flies) by three independent masked photo-graders. Conjunctival photographs were similarly graded in a masked fashion for signs of clinically active trachoma. Conjunctival swabs were processed for Chlamydia trachomatis DNA. Of 2073 children with complete data, 808 (39%) had evidence of clinically active trachoma, 150 (7%) had evidence of ocular chlamydia infection, and 2524 (91%) had at least one measure of an unclean face. Dry ocular discharge had the strongest association with clinically active trachoma (age- and sex-adjusted prevalence ratio [PR] 1.4, 95% CI 1.2-1.6) and ocular chlamydia infection (PR 1.9, 95%CI 1.3-2.9), although significant associations were observed between each of the measures of facial uncleanliness and trachoma. Masked assessment of face and conjunctival photographs confirmed prior observational studies that have noted associations between various measures of facial uncleanliness and trachoma. The causal relationship between facial uncleanliness and trachoma is unclear since many features used to measure facial cleanliness (e.g., ocular discharge, nasal discharge, and flies) could be consequences of antecedent ocular chlamydia infection. NCT02754583, clinicaltrials.gov.

Journey of Guillain Barre syndrome from the pre-pandemic era to the pandemic era: A 4-year retrospective study.

To study demographic and clinical profiles of Guillain Barre syndrome (GBS) in the pre-pandemic and coronavirus disease 2019 (COVID-19) pandemic era and to compare the GBS incidence, severity, and its outcome in the pre-pandemic and pandemic eras. This is a 4-year retrospective study done in a tertiary care hospital in Delhi, India, between March 2018 and March 2022. Patients were divided into the pre-pandemic era and pandemic era (2 years before and 2 years after March 2020). The number of patients (N) was 25 in the pandemic/vaccine era, while N = 49 in the pre-pandemic era. The mean duration of hospitalization was significantly higher (P = 0.03) during the pandemic era (10.68 ± 6.67 days) compared to the pre-pandemic era (7.59 ± 3.55 days). There was no statistical difference in age (P = 0.56), gender (P = 0.70), GBS variants (P = 0.40), clinical spectrum, antecedent infection (P = 0.91), Hughes Disability Score on admission and discharge (P = 0.93 and P = 0.52, respectively), respiratory involvement requiring a ventilator (P = 0.19), and mortality (P = 0.26) in both the eras. Our study showed no association of the incidence of GBS with the ongoing COVID-19 pandemic. The mean hospitalization days were significantly increased during COVID-19 in view of associated respiratory involvement. The commonly held hypothesis of the increase in GBS cases during the pandemic/vaccine era has not been observed in our study.

Concomitant Central and Peripheral Nervous System Involvement Associated With Mycoplasma pneumoniae Infection in Pediatric Patients: Two Case Reports and Literature Review.

Mycoplasma pneumoniae ( M. pneumoniae ) is a common pathogen for community-acquired pneumonia and is also implicated in a broad array of extra-pulmonary manifestations. M. pneumoniae infection is rarely associated with concurrent central nervous system (CNS) and peripheral nervous system (PNS) involvement in children. We report 2 patients who presented with acute encephalitis and polyradiculitis due to M. pneumoniae infection and review the literature to discuss the pathogenesis and treatment of concomitant CNS and PNS involvement associated with M. pneumoniae infection. We report two 6-year-old boys with M. pneumoniae antecedent infection who presented initially with impaired consciousness followed by limb weakness, limb pain and urinary retention, and responded well to immunotherapy. We described 2 patients who presented symptomatic combined CNS and PNS involvement with persistent urinary retention associated with M. pneumoniae infection. We found autoimmunity plays an important role and recommend that antibiotics and immunomodulators should be administered with concurrent CNS and PNS involvement associated with M. pneumoniae .

Superacute onset of Guillain-Barré syndrome after elective spinal surgery: A case report and literature review.

Guillain-Barré syndrome (GBS) epitomizes an acute peripheral neuropathy hallmarked by an autoimmune retort directed at the myelin sheath enwrapping peripheral nerves. While it is widely acknowledged that a majority of GBS patients boast a history of antecedent infections, the documentation of postoperative GBS occurrences is progressively mounting. Drawing upon an exhaustive compendium of recent case reports, the disease's inception spans a gamut from within 1 hour to 1.2 years. At this juncture, we proffer a singular case: an instance involving a 51-year-old gentleman who underwent lumbar spine surgery, only to encounter immediate debilitation of limb and respiratory musculature. Post elimination of variables linked to anesthetic agents, encephalon, and spinal cord pathologies, a potent suspicion of superacute GBS onset emerged. Subsequent to immunoglobulin therapy, plasmapheresis, and adjunctive support, the patient's ultimate demise became manifest. No progress was found to date. Given GBS's potential to instigate paralysis, respiratory collapse, and autonomic nervous system aberrations, alongside other pernicious sequelae, coupled with the exceptional rarity of the temporal onset in this particular instance, it undeniably proffers an imposing conundrum for anesthetists in the realm of differential diagnosis and therapeutic conduct. During the postoperative convalescence phase under anesthesia, should the patient evince deviant limb musculature vigor and compromised respiratory sinews, the prospect of GBS must not be consigned to oblivion. Precision in diagnosis conjoined with apt therapeutic measures could well be the harbinger of a divergent denouement for the afflicted patient.

Critical Care in Guillain–Barré Syndrome

AbstractGuillain–Barré syndrome (GBS) is an autoimmune polyneuropathy characterized by hyporeflexic neuromuscular paralysis and albuminocytologic dissociation in the cerebrospinal fluid. It is a postinfectious disorder. The most common antecedent illnesses are respiratory tract infection and Campylobacter jejuni infection. After the antecedent infection, specific antibodies are generated that cross-react with gangliosides in the host culminating in demyelination of the peripheral nerves or nerve roots. Complement activation also contributes to nerve degeneration. Bilateral symmetrical progression of the limb weakness occurs over a period of a few days followed by a plateau phase, after which a recovery phase follows. Generalized hypotonia and hyporeflexia characterize the limb weakness. Cerebrospinal fluid analysis shows albuminocytologic dissociation. About one-third of patients develop respiratory failure. Neuropathic pain is a disturbing symptom in GBS. Dysautonomia is very characteristic of GBS. Erasmus GBS respiratory insufficiency score predicts the need for mechanical ventilation. The weaning process from mechanical ventilation mainly depends on the recovery of vital capacity and inspiratory force. The definitive treatment for GBS consists of plasma exchange or intravenous immunoglobulin therapy both of which are equally efficacious. Seasonal variation has been observed in the occurrence and recovery of GBS. Prognosis of GBS varies widely. Erasmus GBS outcome scale scoring system predicts the ability of the patient to walk independently after 6 months. Several GBS cases have been reported globally during recent pandemic of coronavirus disease 2019. Though GBS is a self-limiting disease, there are quite a few research questions that still remain to be answered.

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.

Comparison of Clinical Features, Short-Term Outcome of Guillain-Barré Syndrome Between Adults and Children: A Retrospective Study in Vietnam

BackgroundDespite extensive research on Guillain-Barré syndrome (GBS) in adults and children, there is a lack of comparison regarding short-term outcomes in various age groups. Our study aims to elucidate the differences in clinical features and short-term outcomes in Vietnam. MethodsAfter retrospective data collection, we compared clinical features in patients with GBS aged ≤16 years at Children's Hospital 2 and aged >16 years at University Medical Center Ho Chi Minh City from 2017 to 2021. A positive short-term outcome was recorded if patients had a GBS Disability Score of 0 to 2 at hospital discharge. ResultsWe analyzed 109 adults (58.7% males; mean age 50.6 ± 17.7) and 111 children (58.6% males; mean age 7.2 ± 4.9). Comparable antecedent infection and immunization incidence in both groups were observed (35.8% vs 45.9%, P > 0.05). Pain and sensory disturbance were the most common onset symptom in adults (57.8%), whereas lower limb weakness predominated in children (61.3%). Ophthalmoplegia (18.3% vs 5.4%), pain, sensory disturbance (85.3% vs 67.6%), ataxia (33.0% vs 15.3%) were more prevalent in adults (P < 0.05). The axonal subtype was prominent in both adults (51.4%) and children (53.2%). Patients were classified into: classic GBS (49.5% and 68.5%), GBS variants (11.0% and 15.3%), classic Miller Fisher syndrome (MFS) (1.8% and 1.8%), MFS variants (2.8% and 0%), and GBS/MFS overlap (34.9% and 14.4%). Short-term outcomes did not significantly differ based on age. ConclusionsAge-related variations in clinical features were observed, but adults and children exhibited similar short-term functional outcomes.

Amifampridines are the Most Effective Drugs for Treating Lambert-Eaton Myasthenic Syndrome With a Focus on Pediatric Lambert-Eaton Myasthenic Syndrome.

In 1983, the first successful trial of 3,4-diaminopyridine (3,4-DAP) in Lambert-Eaton myasthenic syndrome (LEMS) was reported. Efficacy of amifampridine (3,4-DAP and 3,4-diaminopyridine phosphate [3,4-DAPP]) for symptomatic treatment in LEMS was proven by seven randomized studies in 3,4-DAP and two randomized studies in 3,4-DAPP. US Food Drug Administration approved 3,4-DAPP usage for adult LEMS in 2018 and for pediatric LEMS in 2022. Nineteen pediatric LEMS cases were identified in the literature. Compared with adult LEMS, the rate of malignancy is low as expected and the rate of dysautonomia is also low in pediatric LEMS. Unexpected finding is two cases of pediatric LEMS following antecedent infection. Amifampridine can be safely used as long the daily dose is less than 80 mg a day for adult LEMS patients and less than 30 mg a day for pediatric LEMS patients. Amifampridines can be supplemented with a liberal amount of pyridostigmine for long term usage. Amifampridine was used as symptomatic treatment in eight (42%) of 19 pediatric LEMS patients: 3,4-DAP in six and 3,4-DAPP in two patients. The most common practice of 3,4-DAP was a combination with pyridostigmine in four patients. With 3,4-DAP, normal activity was reported in 3 cases and mild to moderate-improvement in other 3 cases. In two patients with 3,4-DAPP, significant improvement in one and no improvement in one. Amifampridines are proven to be effective and safe drugs for the symptomatic treatment without serious side reaction in adults as well as in children as long as the dosage is properly adhered.

An Observational Study on Factors Causing Variable Response to Therapeutic Doses of Intravenous Immunoglobulin in Acute Immune-Mediated Polyneuropathy: A Tertiary Care Center Experience

Context: Immune-mediated neuropathies, notably Guillain–Barré syndrome (GBS) and its subtypes, exhibit diverse clinical presentations. Intravenous immunoglobulin (IVIG) is a standard treatment for these conditions, but the variable clinical course complicates outcome prediction. Despite standard IVIG dosing, some patients continue to deteriorate. Aim: The aim of this study was to assess the factors responsible for variable response to therapeutic doses of immunoglobulins in acute immune-mediated polyneuropathy. Settings and Design: This was a prospective, observational study. Materials and Methods: Acute immune-mediated polyneuropathy cases within 14 days of symptom onset and receiving IVIG were recruited. Hughes disability score (HDS), modified Erasmus GBS outcome score (mEGOS), and modified Rankin score (mRS) were assessed before IVIG, immediately after IVIG, and 4 weeks thereafter. Statistical Analysis Used: Categorical variables are expressed as percentages, and continuous variables are presented as mean ± standard deviation. Relationships between parameters were assessed using analysis of variance and multivariate analysis of covariance. Correlations were measured using Pearson’s correlation, and proportions were compared using the Chi-square test or Fisher’s exact test. Results: Among 60 GBS patients, the median age was 38 years, with upper respiratory tract infection as the most common antecedent infection (60%). The most common variant was pure motor type, while demyelinating (88.33%) was the most common electrophysiological subtype. Outcome correlated with post-IVIG scores of HDS and mRS. Linear regression analysis showed a positive correlation between onset-to-treatment duration and HDS and mEGOS scores after 4 weeks of IVIG, while mRS showed minimal correlation. No significant correlations were found between antecedent infection, gender, nerve conduction study pattern, GBS variant, and scores of HDS, mEGOS, and mRS. Conclusions: The study highlights the significance of onset-to-treatment duration. Factors with unfavorable course were age, delay in receiving IVIG, respiratory involvement, dysautonomia, cranial nerve involvement, and those with higher mEGOS scores at the 7th day of admission. Seasonal trends should not be overlooked. Serial evaluation of disability scores can predict the varying response to IVIG.

Rapid formation of aortoesophageal fistula complicated by mycotic thoracic aortic aneurysm secondary to infective endocarditis.

A 73-year-old man was admitted with four weeks of intermittent fever. He had a history of total aortic arch replacement for aortic arch aneurysm four years prior. CT scans showed no abnormalities before admission. Repeated blood cultures yielded Streptococcus anginosus and Prevotella melaninogenica, suggesting infective endocarditis (IE). Transesophageal echocardiography revealed a vegetation on the aortic valve, confirming IE. He suddenly presented with massive hematemesis and hypotension. Endoscopy revealed an elevated lesion with a laceration but no active bleeding in the esophagus. CT scans showed a thoracic aneurysm involving the esophagus. A diagnosis of aortoesophageal fistula (AEF) complicated by mycotic thoracic aortic aneurysm (MTAA) was made, and he underwent stent graft interpolation followed by minimally invasive esophagectomy. MTAAs are more prone to rupture than arteriosclerotic aneurysms as they are usually not true but pseudoaneurysms. Antecedent infection, including endocarditis, sepsis, predisposes to MTAA. AEF is a rare but life-threatening cause of gastrointestinal bleeding characterized by Chiari's triad. There have been no reports of such rapid formation of AEF after the graft replacement, as shown here. A recent article reported a rapid formation (16 days) of AEF after thoracic endovascular aortic repair, emphasizing prosthetic infection as the most important risk factor. Our case underscores the importance of suspecting AEF and conducting repeated appropriate examinations even if initial examinations do not reveal any aneurysms.

Guillain-Barré Syndrome.

This article summarizes the clinical features, diagnostic criteria, differential diagnosis, pathogenesis, and prognosis of Guillain-Barré syndrome (GBS), with insights into the current and future diagnostic and therapeutic interventions for this neuromuscular syndrome. GBS is an acute, inflammatory, immune-mediated polyradiculoneuropathy that encompasses many clinical variants and divergent pathogenic mechanisms that lead to axonal, demyelinating, or mixed findings on electrodiagnostic studies. The type of antecedent infection, the development of pathogenic cross-reactive antibodies via molecular mimicry, and the location of the target gangliosides affect the subtype and severity of the illness. The data from the International GBS Outcome Study have highlighted regional variances, provided new and internationally validated prognosis tools that are beneficial for counseling, and introduced a platform for discussion of GBS-related open questions. New research has been undertaken, including research on novel diagnostic and therapeutic biomarkers, which may lead to new therapies. GBS is among the most frequent life-threatening neuromuscular emergencies in the world. At least 20% of patients with GBS have a poor prognosis and significant residual deficits despite receiving available treatments. Research is ongoing to further understand the pathogenesis of the disorder, find new biomarkers, and develop more effective and specific treatments.

Guillain–Barré syndrome after COVID‐19 vaccination with suspected concurrent cytomegalovirus reactivation

AbstractBackgroundGuillain–Barré syndrome (GBS) is an immune‐mediated polyneuropathy most commonly associated with antecedent infections, such as Campylobacter jejuni or viral infections, but vaccination is also known as an antecedent event. In recent years, due to the worldwide pandemic, vaccination against coronavirus disease 2019 (COVID‐19) has widely spread throughout the world. Although surveillance of vaccination‐associated adverse effects is still ongoing, multiple cases of GBS after COVID‐19 vaccination have been reported. However, the exact pathophysiology of COVID‐19 vaccination causing GBS has not been clarified. Not only new‐onset infection, but also reactivation of dormant viral agents, is known to trigger GBS. This reactivation might occur after COVID‐19 vaccination; however, there are no reports of this phenomenon in association with post‐vaccination GBS.Case PresentationA 44‐year‐old immunocompetent woman presented with acute onset weakness in all four limbs 6 days after COVID‐19 vaccination. Neurological examination showed dysphagia, symmetrical weakness, absent tendon reflexes and distal dominant sensory disturbance in all four extremities. Nerve conduction studies were compatible with demyelinating neuropathy, and serum was positive for anti‐GD1a‐IgG antibody. A diagnosis of acute inflammatory demyelinating neuropathy was made. Serological examination on admission suggested concomitant cytomegalovirus reactivation. Plasma exchange rapidly improved her symptoms, including weakness and sensory disturbance, and she was discharged without residual symptoms.ConclusionsThe present case suggests that cytomegalovirus reactivation might play a role in the pathogenesis of GBS associated with COVID‐19 vaccination. Accounting for cytomegalovirus reactivation might reveal unidentified pathophysiology of GBS after COVID‐19 vaccination.

C57BL/6J mice exposed to perfluorooctanoic acid demonstrate altered immune responses and increased seizures after Theiler's murine encephalomyelitis virus infection.

Neurological diseases can stem from environmental influences such as antecedent viral infections or exposure to potential toxicants, some of which can trigger immune responses leading to neurological symptoms. Theiler's murine encephalomyelitis virus (TMEV) is used to model human neurological conditions associated with prior viral infections, with outcomes partly attributable to improper induction and regulation of the immune response. Perfluorooctanoic acid (PFOA) can alter pathologies known to influence neurological disease such as inflammatory responses, cytokine expression, and glial activation. Co-exposure to TMEV and PFOA was used to test the hypothesis that early life exposure to the potential immunotoxicant PFOA would affect immune responses so as to render TMEV-resistant C57BL/6J (B6) mice susceptible to viral-induced neurological disease. Neonate B6 mice were exposed to different treatments: non-injected, sham-infected with PBS, and TMEV-infected, with the drinking water of each group including either 70 ppt PFOA or filtered water. The effects of PFOA were evaluated by comparing neurological symptoms and changes in immune-related cytokine and chemokine production induced by viral infection. Immune responses of 23 cytokines and chemokines were measured before and after infection to determine the effects of PFOA exposure on immune response. Prior to infection, an imbalance between Th1, Th2, and Treg cytokines was observed in PFOA-exposed mice, suppressing IL-4 and IL-13 production. However, the balance was restored and characterized by an increase in pro-inflammatory cytokines in the non-infected group, and a decrease in IL-10 in the PFOA + TMEV group. Furthermore, the PFOA + TMEV group experienced an increase in seizure frequency and severity. Overall, these findings provide insight into the complex roles of immune responses in the pathogenesis of virus-associated neurological diseases influenced by co-exposures to viruses and immunotoxic compounds.

Antecedent Infections and Hospital Outcomes in Demyelinating and Axonal type of Guillain-Barre Syndrome among adult Patients

Background: Many differences are observed with respect to hospital outcome among demyelinating and axonal type of Guillain-Barre Syndrome. Objective: Aims of this study was to compare the hospital outcome at discharge among demyelinating and axonal type of Guillain-Barre syndrome. Methodology: This prospective observational study was conducted from Oct, 2017 to September, 2018 at the National Institute of Neurosciences and Hospital, Dhaka, Bangladesh. All demyelinating and axonal variant of GBS, presented within 2 weeks were included in this study. The clinical parameters were taken for baseline analysis. Outcomes were measured at discharge by MRC score, length of hospital and ICU stay, recovery and death. Results: Out of 108 GBS cases 55 (50.9%) demyelinating &amp; 53 (49.1%) were axonal subtype. Mean age; 40.20 ± 16.26 vs. 32.43 ± 14.93 years in demyelinating vs. axonal cases (p=0.011). Symptom onset and nadir of weakness are quicker in axonal than demyelinating cases (8.47±4.97 vs. 12.36±7.94) (p=0.017) and (6.7 ± 4.5 vs. 8.36 ± 4.59) (p=0.022)] days. Baseline MRC score were (18.04±9.85 vs 12.94±9.91) (p=0.009) demyelinating vs. axonal cases. At discharge, Mean MRC score, length of hospital stay, length of ICU stay were, (22.15±9.38 vs 17.39±9.46; p=0.013); (15.13±17.60 vs 23.58±23.55) days (p=0.038)} and (28.69±23.28 vs 37.0±20.01) days respectively. Conclusion: Hospital outcome of GBS patients is favorable to demyelinating subtype in respect of MRC score, length of hospital stay, length of ICU stay and also death and survivability. Bangladesh Journal of Medical Microbiology, January 2022;16(1):19-24

Guillain-Barré Syndrome in Children: A Narrative Review

Among the most typical reason of sudden, acquired weakness is Guillain-Barré Syndrome characterized by symmetrical ascending paralysis which is frequently sparked by an earlier illness. “Acute Inflammatory Demyelinating Polyneuropathy,” one of the prevailing kinds of Guillian-Barre Syndrome, is divided into four subtypes: acute inflammatory demyelinating polyradiculoneuropathy, Miller Fisher syndrome, acute motor sensory axonal neuropathy, and acute motor axonal neuropathy. Guillain-Barré syndrome can happen at any age, but it is frequently seen in persons over 40 years. More often, males are impacted. This syndrome lasts for 4 weeks, but if it is prolonged than that, it can be categorized as subacute, and if it lasts longer than 8 weeks, it can be classified as chronic. An antecedent infection is present in 70% of patients. The most frequent trigger for Guillain-Barré Syndrome is Campylobacter jejuni, along with the Cytomegalovirus, Zika virus, mycoplasma, hepatitis A and B, and human immunodeficiency virus. The antecedent infection generally occurs 1 to 3 weeks before. The pathogenesis of the disease solely based on the molecular mimicry. In some circumstances, collapse of respiratory system and autonomic dysfunction might make Guillain-Barré Syndrome more difficult to treat.

Guillain-Barré Syndrome in Adults in a Decade: The Largest, Single-Center, Cross-Sectional Study From the Kingdom of Saudi Arabia.

Guillain-Barré Syndrome (GBS) is the most common cause of acute, usually post-infectious, peripheral neuropathy resulting in a symmetrical, ascending paralysis. We evaluated the clinical and neurophysiological features, treatment, and outcomes of patients with GBS in our center. A retrospective chart review on patients with GBS admitted to King Abdulaziz Medical City, Riyadh, Saudi Arabia, from January 2011 to December 2020. Data were analyzed using JMP statistical software version 15 pro. A total of 86 patients who met the criteria were included, 55 (64%) were males, with a mean age of 49.5+/-17.5 years. Antecedent infection was reported in 53 (61.6%), 51 (62.2%) presented within one week of symptoms onset.Ascending weakness was seen in 55 (70.5%), while 70 (81.4%) had areflexia. Acute motor axonal neuropathy (AMAN) was the commonest electrophysiological type of GBS in 41 (51.9%) patients.Albuminocytologic dissociation was seen in 48 (57%) who had lumbar puncture.Nearly half, 41 (47.7%) were admitted to the intensive care unit (ICU). Seventy (81.3%) were treated with intravenous immunoglobulin.There was no significant difference in the clinical presentation, management, ICU requirement, and discharge disposition between males and females.Females were more likely to have a higher disability at discharge (p=0.01).Patients younger than 60 years were more likely to require ICU admission (p=<0.01). Our patients with GBS were slightly older than previously reported from the region.AMAN was the commonest type of GBS.Younger patients were more likely to need ICU admission, whereas females were more likely to have a more severe disability.

Impact of infections on the incidence of acute inflammatory demyelinating polyneuropathy in children

AbstractObjectivesAcute inflammatory demyelinating polyneuropathy (AIDP) is the leading cause of acute flaccid paralysis in children and hypothesized to be triggered by antecedent infection. We sought to determine the association between AIDP and commonly acquired community infections in children. We utilized the reduction in these infections due to measures during coronavirus disease 2019 (COVID‐19) to serve as a natural experiment and determine their contribution to AIDP.MethodsThis cross‐sectional study used administrative and billing data from children's hospitals contributing to the Pediatric Health Information System. We included hospitalizations of children with a diagnosis of AIDP from (January 2017 through February 2021). Encounters for infection‐ (including respiratory, gastrointestinal, and COVID‐19) related diagnoses were measured as a marker of community incidence.ResultsA total of 1111 index encounters for AIDP were included. Pre‐COVID‐19, AIDP was not associated with respiratory or gastrointestinal infections, specifically, influenza or campylobacter. During the COVID‐19 period from March 2020 to February 2021, respiratory, gastrointestinal, and influenza infections decreased compared to expected (for the same time of year pre‐COVID‐19) by 59.6%–90.1%, 51.5%–68.9%, and 54.5%–97.9%, respectively. In contrast, AIDP hospitalizations and all hospitalizations only decreased by 11.5%–39.3% and 14.2%–25%, respectively. COVID‐19 was not positively associated with AIDP overall or at individual hospitals.InterpretationCommon community‐acquired infections including COVID‐19 were not strongly associated with hospitalizations for AIDP in children. AIDP persisted despite the dramatic reduction in infection‐related encounters during the pandemic. These results suggest that recent antecedent community‐acquired infections were not the primary driver of AIDP and that alternative triggers should be explored.

Clinical Manifestation, Auxiliary Examination Features and Prognosis of GFAP Autoimmunity: a Chinese Cohort Study (P5-5.029)

<h3>Objective:</h3> To report the clinical manifestation and auxiliary examination features of 15 Chinese patients with glial fibrillary acidic protein (GFAP) autoimmunity. <h3>Background:</h3> GFAP astrocytopathy is a corticosteroid responsive inflammatory central nervous system (CNS) disorder spectrum predominantly affecting meninges, brain, spinal cord, and optic nerve mimics infectious meningoencephalitis. IgG antibodies binding to GFAP are specific biomarkers with diagnostic significance. <h3>Design/Methods:</h3> From June 2016 to December 2019, patients with suspected neurological autoimmune disease were scanned for autoantibodies by immunohistochemistry. Samples showed a characteristic immunoreactive pattern reminiscent of GFAP of astrocytes were selected and confirmed by cell-based assay. <h3>Results:</h3> Fifteen patients (8 male, 7 female) with a median age at onset of 53 years were identified as GFAP-IgG positive. Eleven of 15 patients presented with an acute monophasic course, of which 10 had antecedent flu-like symptoms. The predominant phenotype was meningoencephalitis (46.7%), followed by meningoencephalomyelitis in 40% of cases. Magnetic resonance imaging revealed enhancement in 10 of 13 patients. Inflammatory CSF showed mild lymphocyte-predominant pleocytosis with a median of 51/μl, elevated protein with a median of 87.5mg/dl. Five of six patients tested for CSF specific oligoclonal bands were positive. Five patients had coexisting antibodies, including NMDAR-IgG in 3 patients, Yo and MOG-IgG in one each patient. One patient underwent stereotactic brain biopsy and neuropathology diagnosis was diffuse large B-cell lymphoma with normal reactive fibrillary astrocytes. One patient had ovarian teratoma. Eleven of 15 patients received both intravenous immunoglobulin and steroids. Among them, 3 patients also received immunosuppressive agents later. During 2-year follow-up, nine of 15 patients achieved complete clinical remission. <h3>Conclusions:</h3> The clinical presentation of GFAP astrocytopathy is heterogeneous, including acute monophasic course or chronic relapsing courses. Patients with an acute monophasic course with antecedent infection symptoms responded well to immunotherapy. In case of primary central nervous system lymphoma, GFAP autoimmunity doesn’t equal to autoimmune GFAP astrocytopathy. <b>Disclosure:</b> Dr. Liu has nothing to disclose. Dr. Zhang has nothing to disclose.