Febrile Encephalopathy Research Articles

Clinical profile, complications, outcome and predictors of intensive care for paediatric scrub typhus: a tertiary hospital-based study from Southern Rajasthan, India

Background: Scrub typhus is an endemic acute febrile zoonotic disease and one of the most neglected tropical illnesses. In 2019, its outbreak was reported from Mewar region of Rajasthan, India. It is caused by Orientia tsutsugamushi which is intra cellular gram-negative coccobacilli. Methods: It is retrospective descriptive study conducted on children diagnosed positive for scrub typhus by IgM enzyme-linked immunosorbent assay (ELISA) admitted in ward and paediatric intensive care unit at tertiary care centre during 1 year of study period from April 2023 to March 2024. Clinical profile, laboratory parameters, course of illness and outcome data were collected in predesigned case records proforma for analysis. Results: Mean age of presentation was 10.5±3.2 years with female preponderance (F:M 1.21:1). 78.5% belong to rural areas. Environmental exposure was living close to forests, bushes in 38%, crop fields in 26% and cattle shed with pets in 30%. All cases had fever (100%, n=42) followed by vomiting (62%), cough-cold (42.8%), myalgia (40.4%), altered sensorium (35.7%), breathlessness (31%), oliguria (16.6%), conjunctival redness (11.9%). Most common signs were temperature >101’F (90.4%), hepatomegaly (62%), hypotension (21.4%), edema (23.8%), meningeal signs (14.2%). Tachypnoea (n=14) (p value <0.0001), CRT>3 seconds (n=13) (p value <0.0003), altered sensorium (n=15) (p value=0.0003) and SpO2 <90% (n=7) (p value=0.004) were found significant predictors of pediatric intensive care unit (PICU) admission. Eschar and rash seen in 3 cases (7.1%). 16 (38%) children requiring intensive care unit admission had statistically significant anaemia (85.7%) (p=0.013), thrombocytopenia (78.5%) (p=0.042) and raised C-reactive protein (CRP) (50%) (p<0.0001). Conclusions: Scrub typhus is common in Rajasthan and should be suspected in any child with acute febrile encephalopathy, signs of shock, breathlessness, hepatomegaly, anaemia and thrombocytopenia in the monsoon season with the history of exposure. Early diagnosis and prompt treatment with doxycycline can prevent mortality.

Magnetic resonance imaging findings in acute febrile encephalopathy in children

Magnetic resonance imaging findings in acute febrile encephalopathy in children

Clinicoetiological profile and outcome of patients of acute febrile encephalopathy in a tertiary care hospital in New Delhi - A prospective observational study

Clinicoetiological profile and outcome of patients of acute febrile encephalopathy in a tertiary care hospital in New Delhi - A prospective observational study

Acute leukoencephalopathy with restricted diffusion – Case Series

Acute leukoencephalopathy with restricted diffusion (ALERD) is a clinicopathological diagnosis that is characterized by severe encephalopathy and seizures with extensive areas of restricted diffusion in cerebral parenchyma. Case 1: Apparently healthy 13-month-old male child presented with acute febrile encephalopathy with a flurry of seizures with raised intracranial pressure. Investigations were consistent with multiorgan dysfunction along with severe metabolic acidosis, ketosis, and hyperammonemia. Initial magnetic resonance imaging (MRI) Brain was normal. However subsequent MRI brain suggested features of diffuse ALERD. With immunotherapy and supportive management, he improved and was discharged without sequelae. Case 2: A 9-month-old male child who was premorbidly normal presented with fever, status epilepticus, and encephalopathy. MRI Brain was suggestive of central sparing ALERD. He was discharged with sequelae with the advice of rehabilitation. Unfortunately, he got succumbed due to aspiration. Case 3: A 10-day-old neonate presented with the refusal of feeds, multiple seizures, and encephalopathy. MRI Brain revealed central sparing ALERD. Improved with immunotherapy and was developmentally normal at five months of age. ALERD is a clinic-radiological syndrome characterized by acute encephalopathy with restricted areas of diffusion in subcortical white matter on MRI. Reported two categories are Diffuse and Central sparing ALERD. Diffuse ALERD has severe manifestations. However, Case 1 had a good outcome, suggesting a variable prognosis. Central sparing ALERD is a milder form, but when basal ganglia are involved, it may have a worsened outcome, as seen in Case 2. Case 3 had central sparing ALERD, and had a good prognosis as described in literature. This study highlights the varied signs and symptoms of ALERD, including neonatal age of onset. Diagnosis is based on the restricted diffusion in white matter on diffusion-weighted imaging and the apparent diffusion coefficient in MRI of the brain. MRI may be normal in the initial course; hence, it’s important to repeat if strongly suspected. Prompt immunotherapy and supportive care are emphasised.

Clinical profile of febrile encephalopathy patients at a tertiary care hospital in India: A retrospective study

Objective: To investigate demography, etiology, and clinical profile and to analyze the outcomes of patients presenting with febrile encephalopathy at a tertiary care center in Eastern India. Methods: This retrospective, observational study included a total of 50 patients (>18 years) who presented with fever and encephalopathy. All patients underwent blood analysis; culture test for blood, urine, sputum, cerebrospinal fluid, and other body fluids; chest and abdominal ultrasonography; neuroimaging; polymerase chain reaction test, and other relevant tests as and when applicable. Results: The mean age of all enrolled patients was (58.0±16.0) years with male to female ratio of 1.27:1. Viral encephalitis was diagnosed in 16 (32%) patients, sepsis-associated encephalitis in 14 (28%), bacterial meningoencephalitis in 8 (16%) and tuberculosis meningoencephalitis in 5 (10%). The mean hospital stay was 10 (4, 17) days. At 6-month follow-up, 10 (20%) patients died, 10 (20%)patients recovered with sequelae (cognitive impairment, critical careneuropathy, etc.), and 30 (60%) patients recovered without sequelae. A statistically significant association was noted between GlasgowComa Scale (<8) and mortality rate (P=0.02). Conclusions: Primary central nervous system infection is the mostfrequent etiology of febrile encephalopathy and viral encephalitisis the most common etiological cause. Accurate, systematic, timelydiagnosis and management are prime factors to reduce mortality andmorbidity.

Clinical profile and outcome of acute febrile encephalopathy in children

Background: This prospective observational study aims at studying clinical profile of patients with acute febrile encephalopathy. Methods: This study was conducted in patients of acute febrile encephalopathy admitted in PICU at tertiary care centre during November 2019 to May 2021 in age group one month to 18 years. 35 patients satisfied the inclusion criteria. Results: 51.4% children belonged to age group less than 60 months and 40% children belonged to age group 61-120 months. Other than fever and altered sensorium (diagnostic criteria), seizures (74.3%), vomiting (60%), headache (22.9%) and refusal to feed (17.1%), were the major complaints. Raised ICT was found in 20 (57.1%). 20 (57.1%) patients had hyponatremia (Na+ <135). MRI brain abnormalities were found in 13 patients with majority finding being edema 8 (61.5%). 15 (62.5 %) patients had CSF cell count between 5-100/µl. 16 (45.7%) children presented with GCS score 10-12. In the present study, most common diagnosis was rickettsia encephalitis in 10 patients (28.6%) followed by Dengue encephalitis 6 (17.1%) and probable Viral encephalitis 6 (17.1%). There was no mortality in the present study. Conclusions: Seizure, headache and vomiting were the other common presenting features in a case of acute febrile encephalopathy. This indicates raised intracranial tension. Rickettsia followed by Dengue and probable Viral encephalitis were the common etiological diagnosis. Many cases are viral in nature and since no particular or highly effective therapy is available, early diagnosis, emergent treatment of raised intracranial pressure and hyponatremia and implementation of aggressive supportive care may reduce mortality and morbidity as in present study.

Biallelic variants in ribonuclease inhibitor (RNH1), an inflammasome modulator, are associated with a distinctive subtype of acute, necrotizing encephalopathy

PurposeMendelian etiologies for acute encephalopathies in previously healthy children are poorly understood, with the exception of RAN binding protein 2 (RANBP2)–associated acute necrotizing encephalopathy subtype 1 (ANE1). We provide clinical, genetic, and neuroradiological evidence that biallelic variants in ribonuclease inhibitor (RNH1) confer susceptibility to a distinctive ANE subtype. MethodsThis study aimed to evaluate clinical data, neuroradiological studies, genomic sequencing, and protein immunoblotting results in 8 children from 4 families who experienced acute febrile encephalopathy. ResultsAll 8 healthy children became acutely encephalopathic during a viral/febrile illness and received a variety of immune modulation treatments. Long-term outcomes varied from death to severe neurologic deficits to normal outcomes. The neuroradiological findings overlapped with ANE but had distinguishing features. All affected children had biallelic predicted damaging variants in RNH1: a subset that was studied had undetectable RNH1 protein. Incomplete penetrance of the RNH1 variants was evident in 1 family. ConclusionBiallelic variants in RNH1 confer susceptibility to a subtype of ANE (ANE2) in previously healthy children. Intensive immunological treatments may alter outcomes. Genomic sequencing in children with unexplained acute febrile encephalopathy can detect underlying genetic etiologies, such as RNH1, and improve outcomes in the probands and at-risk siblings.

Changing Clinical Profile and Predictors of Mortality in Patients of Acute Febrile Encephalopathy from North India.

Acute encephalitis syndrome (AES) or acute febrile encephalopathy is a clinical condition characterized by altered mental status occurring after or along with a short febrile illness. In developing countries, infections are the predominant cause of AES. Prominent infections known to cause AES include viruses (such as herpes simplex virus [HSV], Japanese Encephalitis [JE] virus, dengue, enteroviruses [EVs]), bacteria, fungus, and parasites. In the present study, we aim to analyze the etiology, clinical features, and predictors of mortality in patients presenting with acute febrile encephalopathy or acute encephalitic syndrome. The present study was a prospective observational study conducted at Post Graduate Institute of Medical Education and Research a tertiary care center in Chandigarh, India. A total of 105 patients with ≥18 years of age with fever (body temperature >101° F for duration ≤14 days) and altered sensorium (Glasgow coma scale [GCS] score ≤10) lasting for more than 24 h, either accompanying the fever or following it were enrolled. Demographic and clinical details were recorded on pro forma. Cerebrospinal fluid (CSF) analysis was performed for all the enrolled patients at admission for cytology, CSF glucose to blood glucose ratio, protein levels, gram stain and culture sensitivity, adenosine deaminase levels, polymerase chain reaction for HSV/EV/mycobacterium tuberculosis (TB) and immunoglobulin M Enzyme-linked immune assay for JE. Computed tomography of the brain was done in all patients while magnetic resonance imaging (MRI) of the brain was carried out in 75 patients. Among the 105 patients, tubercular meningitis was seen in 27 (25.7%) patients followed by acute pyogenic meningitis in 18 (17.1%) patients. Probable viral encephalitis was present in 12 (11.4%) cases. Septic encephalopathy (n = 10) and scrub typhus encephalitis (n = 8), HSV encephalitis (n = 6), dengue encephalitis (n = 4), leptospirosis (n = 3) were the other infections causing acute febrile encephalitis in our study. In addition to fever and altered sensorium common symptoms observed were headache (52.4%), vomiting (35.2%), and seizures (29.5%). The factors predicting increased mortality were female gender, fever of more than 38°C at admission, GCS <7, MRI showing disease-related findings like altered signal intensity bilateral medial temporal and insular area in herpes simplex encephalitis, etc., changes, and the group of patients where a definite diagnosis could not be established during the hospital stay. Tubercular meningitis/central nervous system TB is the predominant cause of acute febrile encephalopathy in developing countries. Scrub and dengue encephalitis are emerging as an important cause of acute febrile encephalopathy and occur predominantly in postmonsoon seasons. Acute febrile encephalopathy remains an important cause of mortality in patients presenting to Emergency Department (ER). The strongest predictors of mortality are low GCS and undiagnosed cases of AES.

COVID-19 and Acute Neurologic Complications in Children.

Little is known about the epidemiology and outcomes of neurologic complications associated with coronavirus disease 2019 (COVID-19) in children. We performed a cross-sectional study of children 2 months to &lt;18 years of age with COVID-19 discharged from 52 children's hospitals from March 2020 to March 2022. Neurologic complications were defined as encephalopathy, encephalitis, aseptic meningitis, febrile seizure, nonfebrile seizure, brain abscess and bacterial meningitis, Reye's syndrome, and cerebral infarction. We assessed length of stay (LOS), ICU admission, 30 day readmissions, deaths, and hospital costs. We used multivariable logistic regression to identify factors associated with neurologic complications. Of 15 137 children hospitalized with COVID-19, 1060 (7.0%) had a concurrent diagnosis of a neurologic complication. The most frequent neurologic complications were febrile seizures (3.9%), nonfebrile seizures (2.3%), and encephalopathy (2.2%). Hospital LOS, ICU admission, ICU LOS, 30 day readmissions, deaths, and hospital costs were higher in children with neurologic complications compared with those without complications. Factors associated with lower odds of neurologic complications included: younger age (adjusted odds ratio [aOR]: 0.97; 95% confidence interval [CI]: 0.96-0.98), occurrence during delta variant predominant time period (aOR: 0.71; 95% CI: 0.57-0.87), presence of a nonneurologic complex chronic condition (aOR: 0.80; 95% CI: 0.69-0.94). The presence of a neurologic complex chronic condition was associated with higher odds of neurologic complication (aOR 4.14, 95% CI 3.48-4.92). Neurologic complications are common in children hospitalized with COVID-19 and are associated with worse hospital outcomes. Our findings emphasize the importance of COVID-19 immunization in children, especially in high-risk populations, such as those with neurologic comorbidity.

Outcome predictors in patients (older than 14 years) with acute febrile encephalopathy: Role of cerebrospinal fluid analysis

Background: Managing patients with acute febrile encephalopathy (AFE), characterized by fever and altered mental status, is one of the primary reasons for hospitalization and mortality. Understating the predictors of AFE outcome will assist the physician in providing early clues and subsequent inventions to tackle the problem.&#x0D; Aims and objectives: To evaluate the predictors of outcome in patients with AFE, focusing on the cerebrospinal fluid (CSF) analysis.&#x0D; Materials and Methods: A prospective observational study was performed on 507 patients with fever and altered mentation (above 14 years of age) in the Department of General Medicine between December 2017 and May 2019. CSF analysis was done to obtain protein, glucose, cell count, and adenosine deaminase (ADA). Computed tomography/magnetic resonance imaging and PS for malaria parasites were also performed.&#x0D; Results: AFE was more common in males (54.63%). Tuberculous meningitis (TM) (44.8% vs. 41.6%) followed by bacterial meningitis (BM) (25.7% vs. 25%) was the most common diagnosis in females and males respectively. Mortality was more common in TM (57.4%) followed by BM (21.3%) patients. Of the 211 patients with TM, those who died had CSF protein ≥90 (49.7%; P= 0.012), CSF glucose ≥35 (53.2%; P=0.002), CSF cells ≥60 (47%; p=0.012) and ADA ≥15 (31.5%; P&lt;0.001). Of the 127 patients with BM, those who died had CSF protein ≥90 (100%; P&lt;0.001), CSF glucose ≥35 (78.3%; P &lt;0.001) and CFS cells ≥60 (100%; P &lt;0.001).&#x0D; Conclusion: CSF analysis could be important for predicting the outcome and should be done as soon as possible after the clinical judgment of the AFE.

Phase III assessment of topotecan and cyclophosphamide and high-dose ifosfamide in rEECur: An international randomized controlled trial of chemotherapy for the treatment of recurrent and primary refractory Ewing sarcoma (RR-ES).

LBA2 Background: 5-year survival of RR-ES is about 15%. rEECur, the first randomized controlled trial in this setting, is defining standard care, balancing efficacy and toxicity. Methods: Patients aged 4-50 with RR-ES were randomly assigned to topotecan and cyclophosphamide (TC), irinotecan and temolozomide (IT), gemcitabine and docetaxel (GD), or high-dose ifosfamide (IFOS). Primary outcome was event-free survival (EFS) for the phase III comparison. Secondary outcomes included overall survival (OS), toxicity, and quality of life (QoL). A probability-based Bayesian approach was used with multiple pairwise comparisons. At the first and second interim assessments, patients allocated to GD and IT, respectively, had worse objective response (OR) and EFS than the other arms, halting recruitment to both. The final intent-to-treat assessment of the original four arms was a phase III evaluation of TC and IFOS. Results: 451 patients recruited between 18/12/14 and 31/08/21, were randomly assigned to TC (163 patients), IT (127 patients), GD (72 patients), and IFOS (83 patients). Median age was 19 years (range 4-49). Patients had: refractory disease (18%), first recurrence (66%), &gt; first recurrence (17%). Initial disease site was bone in 70%. Sites of progression were: primary site only (15%), pleuropulmonary metastases only (34%), and other metastatic (51%). Baseline renal function was similar in both. Median follow-up (reverse Kaplan-Meier method) was 40 months. For the phase III comparison between TC and IFOS (both, 73 patients), median EFS was 3.7 months (95% CI, 2.1-6.2) for TC and 5.7 months (95% CI, 3.8-7.0) for IFOS. Median OS was 10.4 months (95% CI, 7.5-15.5) for TC and 16.8 months (95% CI, 11.1-25.8) for IFOS. Given the observed data, the posterior probability that EFS and OS were better after IFOS than after TC (ie Pr [true hazard ratio &lt; 1 | data]) was 95% for both. A greater survival difference was observed for patients aged under 14 than those aged ≥ 14 for EFS and OS. Subgroup analyses favored IFOS for all minimization factors. The main grade 3/4 adverse events (% patients with an event) for TC (left-hand values) compared with IFOS were: febrile neutropenia (26% vs. 25%), infections (8% vs. 14%), vomiting (1% vs. 1%), nausea (0% vs. 3%), diarrhea (1% vs. 1%), encephalopathy (0% vs. 7%), and renal toxicity (0% vs. 8%). Descriptive statistics of quality of life scores appeared to favor the IFOS arm over the TC arm in children but not in adults. Conclusions: The first randomized trial in RR-ES has shown that high-dose ifosfamide is more effective in prolonging survival than TC, having previously beaten GD and IT, and should be considered as a control arm in future randomized phase II/III studies in RR-ES if combination with IFOS is logical. rEECur is the first study to provide comparative toxicity and survival data for the four most commonly used chemotherapy regimens in RR-ES. Clinical trial information: ISRCTN36453794.

Clinico-aetiological profile and outcome in children with acute febrile encephalopathy

Background: Acute febrile encephalopathy (AFE) is a medical emergency as well as diagnostic and therapeutic challenge in children. Objective of this study was to assess clinico-aetiological profile and outcome in children with AFE.Methods: This prospective observational study was carried out at tertiary care hospital Udaipur, from January 2020 to July 2021. Total 61 children aged 1 month to 18 years who were admitted in PICU with fever ≤2 weeks duration and altered sensorium either at onset or following fever were enrolled. Patient’s detailed history and physical examination including detailed neurological examinations were recorded on pre-structured performa. The investigations included CBC with PBF, ESR, malarial parasite, dengue, scrub typhus, typhi-dot, blood sugar, KFT, electrolytes, LFT, calcium, CSF examination, urine examination, X-ray chest. ABG, serum ammonia, blood culture for bacteriological studies and CT/MRI brain were performed whenever required.Results: 61 patients were admitted with fever and loss of sensorium. The most common clinical sign was the pallor (63.9%) and vomiting (55.7%) was the most common clinical symptom. Cerebral malaria was the commonest cause (31.1%) of AFE followed by suspected viral encephalitis (14.7%) and pyogenic meningitis 9 (14.7%). Out of total 61 patients, 45 (73.8%) patients were discharged and 16 (26.2%) patients were expired. Maximum mortalities were seen in Reye’s syndrome (5 out of 6 cases) and was most in age group &lt;5 years of age (26 cases).Conclusions: Cerebral malaria was the leading cause of AFE followed by suspected viral encephalitis and pyogenic meningitis. While determining the aetiology of AFE in a malarial endemic area, cerebral malaria should be considered in all patients. Reye’s syndrome should also be considered in patients of AFE should be evaluated to diagnose or rule out this entity.

Acute Febrile Encephalopathy in Children: Etiology, Clinical Presentation and Outcome

Background: Fever and altered sensorium constitute a medical emergency in children. Early recognition, accurate diagnosis and timely management may help in reducing the morbidity and mortality in acute febrile encephalopathy (AFE). AIM: To study etiology, clinical presentation and outcome in children presenting with acute febrile encephalopathy. Methods: This prospective observational cohort study was conducted in a tertiary care center over the duration of one year (2016 to 2017).consecutive patients (age group of one month to 12 years) presenting with fever of ≤ 2 weeks duration and altered mental status were enrolled. The clinical features were noted and patients were followed up through the duration of hospital stay (wards &amp; intensive care unit). Etiology and clinical features were listed as percentage of total cases. Risk factors for mortality were determined using chi-square test. Results: 120 children were enrolled. Besides fever and altered sensorium, convulsions (70%) and meningeal signs (38.3%) were common clinical features. Tubercular meningitis (33.3%) was the most common cause followed by viral encephalitis (24.2%), bacterial meningitis (9.2%) and cerebral malaria (5.8%). Mortality was 16.7% with neuro-morbidity seen in 45% of the cases. Longer duration of hospital stay (≥ 28 days), longer duration of PICU stay (&gt; 14 days), longer duration of mechanical ventilation (≥ 7 days) and low Glasgow coma score (GCS&lt; 8) were risk factors associated with higher mortality. Conclusion: Infectious causes were the commonest cause of AFE. AFE has a high rate of neuro-morbidity. Early identification of high risk factors may help to reduce the mortality. Corresponding Author : Milind S. Tullu, Professor Additional, Department of Pediatrics, Seth Gordhandas Sunderdas Medical College &amp; King Edward Memorial Hospital, Mumbai 400012, Maharashtra, India.

Viral aetiology in paediatric age group patients admitted with acute febrile encephalopathy in Western Rajasthan

PurposeIn this study, we are trying to find out viral aetiology in paediatric age group patients from 1 month to 15 years of age in Western Rajasthan region. MethodsA total of 105 patients from 1 month to 15 years were recruited into this study. CSF samples were collected and were processed by multiplex real-time PCR for detection of various predefined panels of viral agents. ELISA was also done for all samples for detection of dengue, JE, measles and mumps. ResultsA total of 32 samples out of 105 were tested positive for viral agents. Viral aetiology detected in this study were Adenovirus (n ​= ​2), EBV (n ​= ​1), HHV-1 (n ​= ​10), HHV-6 (N ​= ​5), Parechovirus (n ​= ​1), Parvovirus B19 (n ​= ​7), Dengue (n ​= ​2) and Measles (n ​= ​1). Mixed infections were also detected, HHV-1 and HHV-6 (n ​= ​2), HHV-1 and Parvovirus B19 (n ​= ​1). In 73 patients no viral aetiology could be detected. ConclusionsParvovirus B19 is sporadically prevalent in this geographical region. In this study, HHV-6 was also found which has not been reported earlier from India.

Scrub Typhus Meningoencephalitis: An Uncommon Cause of Acute Febrile Encephalopathy in the Elderly

Central nervous system infections are an uncommon cause of acute febrile encephalopathy (AFE) in the elderly. Scrub typhus meningoencephalitis is uncommon and often missed in the elderly. A 70-year-old male presented with complaints of intermittent fever, maculopapular rash, and altered sensorium. Physical examination revealed fever, tachycardia, tachypnea, maculopapular rash on the trunk, and all four limbs, and an eschar on the right thigh. Initial laboratory evaluation revealed anemia, thrombocytopenia, and azotemia. The cerebrospinal fluid revealed lymphocytic pleocytosis, elevated proteins with normal glucose, and adenosine deaminase. Weil–Felix test was positive. The diagnosis of scrub typhus meningoencephalitis with acute kidney injury and atypical pneumonia was established. A prompt treatment with injection doxycycline and tablet rifampicin led to an uneventful recovery. We report the first case of scrub typhus meningoencephalitis as the cause of AFE in the elderly. This case highlights the importance of clinical examination in any febrile encephalopathy presenting in endemic areas or travelers returning from such places.

Fever, Seizures and Encephalopathy: From Bush Fires to Firestorms.

Fever-associated seizures and febrile encephalopathy are common neurological problems in children. Infections of the nervous system are responsible for the majority of cases. However, there is a spectrum of infection-associated and inflammatory conditions associated with the triad of fever, seizures, and encephalopathy. Apart from complex febrile seizures and febrile status epilepticus, fever infection-related epilepsy syndrome of childhood (FIRES), infantile hemiconvulsion hemiplegia epilepsy syndrome (IHHE), acute encephalopathy with delayed diffusion restriction (AESD), acute necrotizing encephalopathy of childhood (ANE), and reversible splenial lesion syndrome (RESLES) are age-related clinical phenotypes of fever-related epilepsy and encephalopathy. Awareness of these entities is important for appropriate diagnosis and the prompt use of immunomodulatory/immunosuppressive therapies. In this review, we discuss the pathophysiology, clinical phenotypes, and management approaches of these fever-related seizure and encephalopathy states.

A Rare Cause of Acute Febrile Encephalopathy in a Four Year Old Boy-A Case Report with Review of Literature.

A Rare Cause of Acute Febrile Encephalopathy in a Four Year Old Boy-A Case Report with Review of Literature.

Vanishing White Matter Disease Presenting as Acute Febrile Encephalopathy: Case Report

AbstractA 3.5-year-old male child patient with mild developmental delay presented with history of acute onset fever, encephalopathy, and dyskinesia. The patient was investigated for common etiologies and was managed supportively. His neuroimaging was suggestive of vanishing white matter (VWM) disease which was confirmed by clinical exome sequencing. The child had an eventful hospital stay followed by near-total recovery after 4 weeks. The case attempts to sensitize readers about the current perspectives pertaining to VWM disease.

Influenza-Associated Neurologic Complications in Children

Research Article| October 01 2021 Influenza-Associated Neurologic Complications in Children AAP Grand Rounds (2021) 46 (4): 39. https://doi.org/10.1542/gr.46-4-39 Views Icon Views Article contents Figures & tables Video Audio Supplementary Data Peer Review Share Icon Share Twitter LinkedIn Tools Icon Tools Get Permissions Cite Icon Cite Search Site Citation Influenza-Associated Neurologic Complications in Children. AAP Grand Rounds October 2021; 46 (4): 39. https://doi.org/10.1542/gr.46-4-39 Download citation file: Ris (Zotero) Reference Manager EasyBib Bookends Mendeley Papers EndNote RefWorks BibTex toolbar search nav search search input Search input auto suggest search filter All PublicationsAll JournalsAAP Grand RoundsPediatricsHospital PediatricsPediatrics In ReviewNeoReviewsAAP NewsAll AAP Sites Search Advanced Search Topics: influenza, neurologic complications Source: Antoon JW, Hall M, Herndon A, et al Prevalence, risk factors, and outcomes of influenza-associated neurologic complications in children [published online ahead of print June 30, 2021]. J Pediatr. doi:10.1016/j.jpeds.2021.06.075 Investigators from multiple institutions conducted a retrospective study to estimate the prevalence of neurologic complications in children hospitalized with influenza, identify risk factors associated with these complications, and assess hospital-related outcomes. For the study, they abstracted data from the Pediatric Health Information System (PHIS) database, which includes information on admissions to 49 tertiary care children’s hospitals across the US. Participants for the current study were children 2 months through 17 years old hospitalized at a PHIS hospital during the influenza seasons from 2015 through 2020 with an ICD-10 diagnostic code for influenza. Information abstracted from the PHIS database on these patients included demographic data, geographic region, hospital length of stay (LOS), ICU admission, other ICD-10 diagnoses, presence of chronic neurologic conditions (defined using the pediatric complex chronic conditions classification system version 2), and hospital charges. The primary study outcome was an ICD-10 code for a neurologic complication, including febrile seizure, non-febrile seizure, encephalopathy, encephalitis, aseptic meningitis, brain abscess and bacterial meningitis, Reye syndrome, and cerebral infarction. Multivariate regression analyses to identify risk factors for a neurologic complication, with controlling for confounders, were conducted. Predominant influenza type was included in the regression model and estimated from CDC influenza surveillance data in each patient’s census division during the week that they were hospitalized. Bivariate analyses were used to compare hospital-related outcomes including LOS, ICU admission, costs (estimated from charge data), and in-hospital death rates among patients with and without neurologic complications. Data were analyzed on 29,676 children hospitalized with influenza. The median age of the included patients was 4 years, and 56% were male. A total of 2,246 (7.6%) had a neurologic complication. The most common neurologic complications were febrile seizure (N = 1,447, 5.0% of influenza admissions), encephalopathy (514, 1.7%), and non-febrile seizure (364, 1.2%). Risk factors identified in the multivariate analyses included male sex (odds ratio [OR], 1.1; 95% confidence interval [CI], 1.0, 1.2), Asian race (OR, 1.7; 95% CI, 1.4, 2.1), other race (includes Pacific Islander, American Indian, multiracial, and other; OR, 1.2; 95% CI, 1.03, 1.4), and presence of a chronic neurologic condition (OR, 3.7; 95% CI, 3.1, 4.2). There was no statistical association between predominant influenza type and neurologic complication. Patients with neurologic complications had longer ICU LOS than those without (mean values 2.7 and 2.2 days, respectively; P <0.05), a higher rate of ICU admissions (24.5% vs 15.3%; P <0.001), a higher rate of in-hospital death (0.7% vs 0.2%; P <0.001), and higher costs (mean costs $6,013 vs $5,282; P <0.001). The authors conclude that neurologic complications were common among children hospitalized with influenza, particularly in those with chronic neurologic conditions. Dr Brady 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. For... You do not currently have access to this content.

P-PN028. Clinical and electrophysiological profile of arsenic neuropathy

Introduction . Arsenic toxicity though rare can affect multiple organs including peripheral nerves. We aim to describe the clinical and electrophysiological characteristics of two patients with arsenic neuropathy Methods . A retrospective chart review of two patients with neuropathy and markedly elevated arsenic levels in blood and hair. Results . Patient 1, a 33-years-old male, developed acute gastroenteritis after consuming locally made liquor, followed by febrile encephalopathy, which improved over one week. Two weeks later, he developed painful ascending paraesthesias involving legs up to knees and hands up to wrists. This was associated with difficulty in walking independently. The patient also used to smoke illicit imported cigarettes. He was provisionally diagnosed to have chronic inflammatory demyelinating polyradiculoneuropathy (CIDP). Examination during the fourth week of illness revealed Mee’s lines, hypotonia, and weakness of all limbs distally, global areflexia, and graded sensory loss to all modalities of sensation. Electrophysiological testing showed reduced motor conduction velocities and absent sensory potentials suggesting demyelinating neuropathy. The patient was treated with d-penicillamine. At three month follow up he improved significantly and could resume his job. Patient 2, a 35-year-old gentleman, painter by occupation, developed painful paraesthesias of distal extremities, imbalance while walking in the dark, and slippage of footwear of two months duration. He had a febrile illness 10 days prior to the onset of neurological symptoms. He was provisionally diagnosed to have immune-mediated neuropathy. Examination revealed Mee’s lines, generalised hyperpigmentation, raindrop pigmentation, and palmoplantar hyper-keratosis. He had normal power, but global areflexia, impaired kinesthetic sensation in all limbs and sensory ataxic gait. Electrophysiological testing showed reduced amplitude of sensory potentials suggesting sensory axonopathy Conclusion . Neuropathy arising from arsenic toxicity can have acute/subacute/chronic course and may be mistaken for other acquired inflammatory neuropathies. A high index of suspicion is required to make an early diagnosis and to initiate appropriate preventive measures.