Bifurcation dissecting aneurysm: tips and tricks to differentiate from saccular aneurysm.

Bifurcations are a common site for saccular aneurysms, but rarely can be a site for dissecting aneurysms. Identification of these aneurysms is extremely important because the management plan depends on it. We describe a rare case of a ruptured dissecting aneurysm at the right ICA bifurcation in a pre-teen child which posed a diagnostic dilemma but ultimately was successfully managed with flow diversion.

Estimating the active and lifetime prevalence and incidence of epilepsy in Asian Countries: A systematic review and meta-analysis.

OBJECTIVE: In the current era of the World Health Organization's Intersectoral Global Action Plan on Epilepsy and Other Neurological Disorders 2022-2031 (IGAP), precise and updated estimates of epilepsy burden are vital in formulating policies to improve the care of persons with epilepsy, especially in Asian countries with significant treatment gap. Hence, we aimed to consolidate the available data and quantify epilepsy prevalence and incidence estimates in Asian countries. METHODS: We systematically searched PubMed, Embase, Ovid, and Scopus databases from inception until March 2023 for studies reporting epilepsy prevalence and incidence in Asian countries. We applied random effects meta-analysis to generate the pooled prevalence and incidence using the Meta package in R. Additionally, we performed a subgroup meta-analysis to explore the potential sources of heterogeneity. A meta-regression analysis was conducted to examine the trend of epilepsy over time. RESULTS: A total of 99 studies with 100,654,124 participants were included in the meta-analysis. The pooled prevalence was 5.6 per 1000 (95 % confidence interval (CI) 4.4-6.8) for active epilepsy and 6.7 per 1000 (95 % CI 5.7-7.9) for lifetime epilepsy. The pooled incidence rate of epilepsy was 52.5 per 100,000 person-years (95 % CI 42.7-79.4). The subgroup analysis revealed a higher prevalence of active epilepsy (6.7/1000) and lifetime epilepsy (8.6/1000) in West Asia than in other regions. The funnel plot and Egger's test (p-value =<0.0001) revealed publication bias for active epilepsy. CONCLUSION: Our findings highlight a high prevalence of active and lifetime epilepsy in West Asia and emphasize the necessity of implementing and formulating specific strategies to tackle the epilepsy burden in this region. Furthermore, high-quality epidemiological studies incorporating economic burdens and comorbidities associated with epilepsy in Asia are still needed.

Predictive Ability of Amplitude Integrated Electroencephalography for Adverse Outcomes in Neonates with Sepsis-Associated Encephalopathy: A Cohort Study.

The authors examined the prevalence of abnormal amplitude integrated electroencephalography (aEEG) patterns in neonates diagnosed with sepsis-associated encephalopathy (SAE). They recorded 36626 min of aEEG in 75 study neonates. Encephalopathy was defined by the Brighton Collaboration Neonatal Encephalopathy criteria. Neonates with primary outcome [either non-survivors or survivors with abnormal neurological examination at discharge using Amiel-Tison assessment tool, n = 58, (77%)] were compared with 17 survivors having normal neurological examination at discharge. Severely abnormal aEEG patterns (isoelectric voltage, continuous low voltage, burst suppression) collectively represented 31% of total 36626 min aEEG tracings. Neonates experiencing primary outcome had significantly higher Burdjalov scores than survivors with normal neurological exam (p value 0.01). After adjusting for gestational age, birth weight, and invasive ventilation, severely abnormal aEEG (aOR 5.8, 95% CI 1.7-19.5, p value 0.005) and Burdjalov score (aOR 0.77, 95% CI 0.63-0.95, p value 0.01) were independently associated with death or abnormal neurological examination at discharge.

Electroclinical Landscape of Infantile Epileptic Spasms Syndrome.

OBJECTIVES: To elucidate the electroclinical characteristics of infantile epileptic spasms syndrome (IESS) and to determine any potential association among these with underlying etiologies and response to therapy. METHODS: Sixty-eight, treatment-naive children with IESS underwent long-term video electroencephalogram (EEG) recording, which was used to characterize the semiology, ictal, and inter-ictal EEG patterns. Children were further followed up to assess electroclinical predictors of etiologies and short-term therapeutic response. RESULTS: Of 68 children enrolled (69% boys), the median age at enrollment was 10.5 mo (IQR-8). Eighty-eight percent of children had flexor spasms, followed by mixed (7%) and extensor (4.4%). Asymmetrical spasms were noted in 17.6% children, and all of them had underlying structural etiology. Two children had the status of epileptic spasms. In the present cohort, authors recognized five distinct ictal EEG correlates of epileptic spasms; the frontocentral dominant slow wave was the most prevalent (32%), followed by the generalized slow-wave complex with superimposed fast rhythm in 29.4%. The occipital dominant slow wave complex was a peculiar pattern in 16%. The major underlying etiologies were hypoxic-ischemic brain injuries (36.7%) and neonatal hypoglycemic brain injuries (22%). Besides asymmetric spasms, authors could not identify any significant association among electroclinical characteristics, underlying etiologies and response to therapy in this study. CONCLUSIONS: The electroclinical landscape of IESS is peculiar and diverse in developing countries. The presence of asymmetrical spasms indicated underlying structural etiology.

Societal costs of illness for infantile epileptic spasms syndrome and evolutionary cost prediction in the era of WHO's IGAP.

BACKGROUND: To achieve the goal of improving the quality of life for persons with epilepsy within the framework of the WHO's Intersectoral Global Action Plan (IGAP), our study aimed to assess the societal financial burden linked to infantile epileptic spasms syndrome (IESS), ensuring that children afflicted with IESS receive high-quality healthcare without enduring substantial financial constraints. METHODS: Between August 2022 and March 2023, 92 children with IESS (male: female: 2:1), recently diagnosed or previously followed-up, were recruited. We gathered costs for drugs, tests, and medical services, along with legal guardians' monthly income. Total expenditure was determined by multiplying unit costs by the yearly service usage commencing from the onset. Time series analysis was utilised to forecast the financial burden from 2022 to 2032. RESULTS: Clinicians' first choice of treatment was ACTH (n = 60, 65·2%), prednisolone (n = 25, 27·2%), and vigabatrin (n = 7, 7·6%) and the median cost of treatment during the initial year was INR 39,010 [USD 479·2]. The median direct medical, direct non-medical, and indirect cost were INR 31,650 [USD 388·4], INR 6581 [USD 80·8], and INR 10,100 [USD 124·07], respectively. Families lost a median of 12 days of work annually. Drug costs and loss of wages were the key factors in the financial burden. The projected and adjusted figures exhibited an incremental growth rate of 2·6% tri-annually. INTERPRETATION: This pioneering study in developing countries, the first of its kind, evaluates the societal cost, financial hardship, and trajectory of incremental cost in IESS. The primary drivers of the financial burden were pharmacological treatment and family work adjustments. The government shoulders 62% of the financial burden, and projected a triannual growth of 2·6% from 2022 to 2032. Our results rationalize policymakers' focus on incorporating IESS into social security programs, particularly in developing countries.

A panel of blood-based circulatory miRNAs with diagnostic potential in patients with psoriasis.

Psoriasis is a chronic inflammatory skin disease with keratinocyte hyperproliferation and T cells as key mediators of lesional and systemic inflammatory changes. To date, no suitable differential biomarkers are available for the disease diagnosis. More recently, microRNAs have been identified as critical regulators of lesional and systemic immune changes in psoriasis with diagnostic potential. We have performed expression profiling of T cell-specific miRNAs in 38 plasma samples from psoriasis vulgaris patients and an equal number of age- and gender-matched healthy subjects. Our findings have identified a panel of five blood-based circulatory miRNAs with a significant change in their expression levels, comprising miR-215, miR-148a, miR-125b-5p, miR-223, and miR-142-3p, which can differentiate psoriasis vulgaris patients from healthy individuals. The receiver operating characteristic (ROC) curves for all five miRNAs individually and in combination exhibited a significant disease discriminatory area under the curve with an AUC of 0.762 and a p < 0.0001 for all the miRNAs together. Statistically, all five miRNAs in combination depicted the best-fit model in relation to disease severity (PASI) compared with individual miRNAs, with the highest R2 value of 0.94 and the lowest AIC score of 131.8. Each of the miRNAs also exhibited a significant association with at least one of the other miRNAs in the panel. Importantly, the five miRNAs in the panel regulate one or more immune-inflammation pathways based on target prediction, pathway network analysis, and validated roles in the literature. The miRNA panel provides a rationalized combination of biomarkers that can be tested further on an expanded cohort of patients for their diagnostic value.

Socio-cultural Adaptation and Validation of Ages and Stages Questionnaire (ASQ 3) in Indian Children Aged 2 to 24 Months.

OBJECTIVE: To socio-culturally adapt and validate a Hindi language version of ASQ-3 in Indian children aged 2-24 months. METHODS: This cross-sectional study was conducted at a tertiary-care center between March, 2017 and April, 2019. Children "at-risk" for developmental delay of either gender aged 2-24 months. Socio-cultural adaptation was done through interaction among 37 subject experts followed by validation. After piloting in 20 children, modified ASQ-3 was validated in 568 at-risk children (4 age-groups: 2-7, 7-13, 13-19 and 19-24 months). Validation was done against Development assessment scale for Indian infants (DASII). RESULTS: Results: After screening 654 children, 568 were enrolled. Among these, 420 had developmental delay on DASII while 18 failed to be identified on ASQ (4.3%). Overall sensitivity and specificity of Hindi language Indian-adaptation of ASQ-3 in detecting developmental delay were 95.9% (95%CI: 93.6%-97.5%) and 81.7% (95%CI: 74%-87.9%), respectively with a positive predictive value (PPV) of 94.6% (95%CI: 92%-96.5%) and negative predictive value (NPV) of 85.6% (95%CI: 78.2%-92.2%). The sensitivity and specificity for motor delay were 96.1% (93.8%-97.7%) and 92.4% (86.4%-96.3%) [PPV: 97.7% (95.8%-98.9%); NPV: 87.7% (81%-92.7%)]. Sensitivity and specificity for mental delay were 95.5% (93.1%-97.2%)and 95.3% (90.1%-98.3%) [PPV: 98.6% (97%-99.5%); NPV: 85.9% (79.1%-91.2%)]. CONCLUSION: The Hindi language Indian-adaptation of ASQ-3 had good psychometric properties with high sensitivity for developmental delay (95.9%), mental delay (95.5%), and motor delay (96.1%), suggesting it to be a good screening tool for neurodevelopmental delay.

Landscape of genetic infantile epileptic spasms syndrome-A multicenter cohort of 124 children from India.

OBJECTIVE: Literature on the genotypic spectrum of Infantile Epileptic Spasms Syndrome (IESS) in children is scarce in developing countries. This multicentre collaboration evaluated the genotypic and phenotypic landscape of genetic IESS in Indian children. METHODS: Between January 2021 and June 2022, this cross-sectional study was conducted at six centers in India. Children with genetically confirmed IESS, without definite structural-genetic and structural-metabolic etiology, were recruited and underwent detailed in-person assessment for phenotypic characterization. The multicentric data on the genotypic and phenotypic characteristics of genetic IESS were collated and analyzed. RESULTS: Of 124 probands (60% boys, history of consanguinity in 15%) with genetic IESS, 105 had single gene disorders (104 nuclear and one mitochondrial), including one with concurrent triple repeat disorder (fragile X syndrome), and 19 had chromosomal disorders. Of 105 single gene disorders, 51 individual genes (92 variants including 25 novel) were identified. Nearly 85% of children with monogenic nuclear disorders had autosomal inheritance (dominant-55.2%, recessive-14.2%), while the rest had X-linked inheritance. Underlying chromosomal disorders included trisomy 21 (n = 14), Xq28 duplication (n = 2), and others (n = 3). Trisomy 21 (n = 14), ALDH7A1 (n = 10), SCN2A (n = 7), CDKL5 (n = 6), ALG13 (n = 5), KCNQ2 (n = 4), STXBP1 (n = 4), SCN1A (n = 4), NTRK2 (n = 4), and WWOX (n = 4) were the dominant single gene causes of genetic IESS. The median age at the onset of epileptic spasms (ES) and establishment of genetic diagnosis was 5 and 12 months, respectively. Pre-existing developmental delay (94.3%), early age at onset of ES (<6 months; 86.2%), central hypotonia (81.4%), facial dysmorphism (70.1%), microcephaly (77.4%), movement disorders (45.9%) and autistic features (42.7%) were remarkable clinical findings. Seizures other than epileptic spasms were observed in 83 children (66.9%). Pre-existing epilepsy syndrome was identified in 21 (16.9%). Nearly 60% had an initial response to hormonal therapy. SIGNIFICANCE: Our study highlights a heterogenous genetic landscape and phenotypic pleiotropy in children with genetic IESS.

Care of Children with Infantile Epileptic Spasms Syndrome and Applicability of Telemedicine Amidst the COVID-19 Pandemic.

This ambispective, observational study evaluated the impact of the COVID-19 pandemic on managing children with Infantile epileptic spasms syndrome (IESS) and the feasibility of telemedicine-based management for IESS. Caregivers of children with IESS were telephonically interviewed using a structured questionnaire and various relevant indices were compared between the study population and a pre-pandemic cohort from the same center. There was a significant increase in diagnostic lag during the pandemic (p = 0.04). Adrenocorticotropic hormone was the first-line antiseizure medication of choice in both cohorts and the response to treatment was also similar. Telemedicine was utilized by around 80% of caregivers and satisfaction rates with telemedicine were high. However, caregivers continued to rate physical consultations higher in preference.

The landscape of infantile epileptic spasms syndrome in South Asia: peculiarities, challenges, and way forward.

Infantile Epileptic Spasms Syndrome (IESS), commonly known as West syndrome, is the most common cause of infantile-onset epileptic encephalopathy. There is a peculiar epidemiological profile of IESS in South Asia. Specific features identified were a preponderance of acquired structural aetiology, male gender dominance, a long treatment lag, limited availability of adrenocorticotropic hormone (ACTH) and vigabatrin, and use of carboxymethyl cellulose derivative of ACTH. Because of the significant disease burden and limited resources, there are distinctive challenges to the optimal care of children with IESS in the South Asian region. Also, there are unique opportunities to bridge these challenges and improve outcomes. This review provides an overview of the landscape of IESS in South Asia and highlights its peculiarities, various challenges, and way forward.

Mental health of health care workers during the hour of crisis - A study from tertiary care hospital in North India.

Background and Aims: This cross-sectional study aimed to analyze the psychosocial, behavioral, and sleep impact of coronavirus disease-19 (COVID-19) pandemic on healthcare workers (HCWs) at a tertiary-care hospital in Northern India. Materials and Methods: An online questionnaire including three psychological scales - peritraumatic distress inventory (PDI), insomnia Severity Index (ISI), and Depression anxiety stress scale was circulated among the HCWs at a tertiary-care hospital in Northern India. Results: Three hundred and ninety-six HCWs (Mean age standard deviation: 34.8 [8.1%] years; 181 females) responded. Place of posting was active COVID area (27.2%), reserve active COVID team (23.2%), trained reserve pool (29.5%), and non-COVID areas (19.9%). More than half of all the respondents (51%) had abnormal PDI score (>14) with a propensity to develop posttraumatic stress disorder. Furthermore, there was a significantly higher prevalence of abnormal PDI scores in those involved in active COVID care units (74%) versus non-COVID care unit (24%, P = 0.001). More than 60% of all the respondents had abnormal ISI score suggesting significant insomnia. Stress was seen in 71% of all the respondents while 82% were anxious and 77% participants had depressive symptoms. Hence, the psychological morbidity among the HCWs was high. Conclusion: Our study found a much higher prevalence of peritraumatic distress, insomnia, anxiety, stress, and depression among the HCWs, more so in those working in COVID areas. Factors indigenous to Indian population in terms of psychological health must be studied and addressed to reduce this psychological morbidity since the battle with COVID is long.

Taking action on climate change: Testimonials and position statement from the International League Against Epilepsy Climate Change Commission.

The release of the 2021 Intergovernmental Panel on Climate Change (IPCC) report makes clear that human activities have resulted in significant alterations in global climate. There is no doubt that climate change is upon us; chronic global warming has been punctuated by more frequent extreme weather events. Humanity will have to mitigate climate change and adapt to these changing conditions or face dire consequences. One under-appreciated aspect of this global crisis is its impact on healthcare, particularly people with epilepsy and temperature-sensitive seizures. As members of the inaugural International League Against Epilepsy (ILAE) Climate Change Commission, we recount the personal motivations that have led each team member to decide to take action, in the hope that our journeys as ordinary clinicians and scientists will help persuade others that they too can act to foster change within their spheres of influence.

Efficacy and Safety of Dietary Therapies for Childhood Drug-Resistant Epilepsy: A Systematic Review and Network Meta-analysis.

Importance: Despite advances in the understanding of dietary therapies in children with drug-resistant epilepsy, no quantitative comparison exists between different dietary interventions. Objective: To evaluate the comparative efficacy and safety of various dietary therapies in childhood drug-resistant epilepsy. Data Sources: Systematic review and network meta-analysis (frequentist) of studies in PubMed, Embase, Cochrane, and Ovid published from inception to April 2022 using the search terms ketogenic diet, medium chain triglyceride diet, modified Atkins diet, low glycemic index therapy, and refractory epilepsy. Study Selection: Randomized clinical trials comparing different dietary therapies (ketogenic diet, modified Atkins diet, and low glycemic index therapy) with each other or care as usual in childhood drug-resistant epilepsy were included. Abstract, title, and full text were screened independently by 2 reviewers. Data Extraction and Synthesis: Data extraction was conducted following Preferred Reporting Items for Systematic Reviews and Meta-analyses reporting guideline. Cochrane risk-of-bias tool was used to assess the study quality. Effect sizes were calculated as odds ratio with 95% CI using random-effects model. The hierarchy of competing interventions was defined using the surface under the cumulative ranking curve. Main Outcomes and Measures: Short-term (≤3 months) 50% or higher and 90% or higher reduction in seizure frequency and treatment withdrawal due to adverse events were the primary efficacy and safety outcomes. Results: Of 2158 citations, 12 randomized clinical trials (907 patients) qualified for inclusion. In the short term, all dietary interventions were more efficacious than care as usual for 50% or higher seizure reduction (low glycemic index therapy: odds ratio [OR], 24.7 [95% CI, 5.3-115.4]; modified Atkins diet: OR, 11.3 [95% CI, 5.1-25.1]; ketogenic diet: OR, 8.6 [95% CI, 3.7-20.0]), while ketogenic diet (OR, 6.5 [95% CI, 2.3-18.0]) and modified Atkins diet (OR, 5.1 [95% CI, 2.2-12.0]) were better than care as usual for seizure reduction of 90% or higher. However, adverse event-related discontinuation rates were significantly higher for ketogenic diet (OR, 8.6 [95% CI, 1.8-40.6]) and modified Atkins diet (OR, 6.5 [95% CI, 1.4-31.2]) compared with care as usual. Indirectly, there was no significant difference between dietary therapies in efficacy and safety outcomes. Conclusions and Relevance: This study found that all dietary therapies are effective in the short term. However, modified Atkins diet had better tolerability, higher probability for 50% or higher seizure reduction, and comparable probability for 90% or higher seizure reduction and may be a sounder option than ketogenic diet. Direct head-to-head comparison studies are needed to confirm these findings.

COVID-19 in Children with West Syndrome: An Ambispective Study.

OBJECTIVES: To study the course of West syndrome (WS) and coronavirus disease-19 (COVID-19) in children with WS who contracted SARS-CoV-2 infection. METHODS: This ambispective study was conducted at a tertiary-care center in North India between December 2020 and August 2021 after approval from the Institute Ethics Committee. Five children with WS, positive for COVID-19 based on RT-PCR, fulfilled the inclusion criteria. RESULTS: One child with COVID-19 during the first wave was retrospectively included while four children (of the 70 children screened) were prospectively enrolled. The median age at onset of epileptic spasms was 7 mo (2 boys), and that at presentation with COVID-19 was 18.5 mo. Three had underlying acquired structural etiology. Three were in remission following standard therapy, while two had ongoing spasms at the time of COVID-19 illness. During the illness, two of those in remission continued to be in remission while one child had a relapse. The children with ongoing epileptic spasms had variable course [one had persistent spasms and other had transient cessation lasting 3 wk from day 2 of COVID-19 illness, but electroencephalography (on day 8 of COVID-19 illness) continued to show hypsarrhythmia]. Fever was the most typical symptom (and sometimes the only symptom) of COVID-19, with a duration ranging from 1-8 d. Two children had moderate COVID-19 illness requiring hospitalization, while the rest had a mild illness. All the affected children had complete recovery from COVID-19. CONCLUSION: The severity of COVID-19 illness in children with WS is often mild, while the subsequent course of WS is variable.

Translation, Adaptation, and Validation of Hindi version of Quality of Life of the Infant (QUALIN) for Use in Infants and Toddlers.

OBJECTIVES: To translate Quality of Life of the Infant (QUALIN), cross-culturally adapt the Hindi version of QUALIN (Hi-QUALIN), and evaluate its psychometric properties in children. METHODS: This cross-sectional study was performed at the tertiary-care center in North India over 21 mo (April 2019 to January 2021). Healthy children (aged 3 to 36 mo) visiting the hospital for vaccination, minor ailments, routine health checkup, and accompanying an ill sibling were included. Children with infantile spasms in same age group were also included. Hindi translations were carried out by bilingual translators who could fluently communicate and write in Hindi and English. Standard Hindi was used to avoid the misinterpretation or misunderstanding. Discriminant and Construct validity was determined utilizing the known-groups method and factor analysis. Reliability was analysed as internal consistency and test-retest reliability. RESULTS: Four hundred and sixty-four children were recruited through opportunity sample selection method with statistically significant difference between healthy and unhealthy children in total score of Hi-QUALIN (3-12 mo) and (13-36 mo). Finally, Hi-QUALIN (3-12 mo and 13-36 mo) consisted of 29 and 30 items constituting the five extracted factors respectively. Overall internal consistency was excellent (α = 0.92 and 0.88, respectively). Intra-class correlation coefficients (ICC) were 0.84 (95% CI: 0.78-0.89; p <0.0001) and 0.94 (95% CI: 0.93-0.96; p <0.0001) indicating excellent test-retest reliability. CONCLUSIONS: Hi-QUALIN has good psychometric properties and can be used for health-related quality of life (HRQoL) measurement in young children.