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On 10 May 2024, a severe geomagnetic storm coinciding with Mother's Day in Mexico lasted over 40 hr and produced polar auroras observable at low latitudes. This storm, the most intense since 2003, resulted from a series of solar flares and coronal mass ejections from active region 3664. The event was significant for space weather studies in Mexico, marking a milestone by enabling comprehensive measurements of its effects. The Mexico Space Weather Service (SCIESMEX) and the National Space Weather Laboratory (LANCE) had prepared for such an event since their inception. LANCE's instrument networks recorded solar chromospheric images, solar radio bursts, geomagnetic variations, Schumann resonances, ionospheric disturbances, and energetic particle flows. They also monitored Geomagnetically Induced Currents (GICs) in three strategic substations of the national electrical system. This provided unprecedented insights into the dynamics of severe space weather events at the North‐American low‐latitude environment. Citizen science efforts documented auroras and regional responses, capturing variations in geomagnetic indices, ionospheric disturbances, cosmic ray fluxes, GICs, and technological impacts. SCIESMEX worked with the National Civil Protection System (SINAPROC) to issue warnings, ensuring public awareness and preparedness. This coordination underscores the importance of effective communication and collaboration in mitigating impacts. The May 2024 geomagnetic storm demonstrated the critical role of preparedness, research, and public education in reducing the effects of future space weather events in Mexico. Plain Language Summary: On 10 May 2024, during the celebration of Mother's Day in Mexico, a powerful geomagnetic storm occurred, lasting over 40 hr and creating polar auroras visible in low‐latitude areas. The Mother's Day geomagnetic storm, the strongest since 2003, was triggered by a series of solar flares and eruptions from the sun. For researchers in Mexico, this event was significant. The storm produced northern lights that were seen in 20 of the 32 states in Mexico, and it gave us the chance to measure the storm's effects with our instruments for the first time. Over the past 10 years, the Mexico Space Weather Service (SCIESMEX) and the National Space Weather Laboratory (LANCE) have been preparing for such an event. During the storm, LANCE's network of instruments recorded various solar and space phenomena, such as solar images, radio bursts, geomagnetic and ionospheric disturbances, energetic particles, and currents in the national electrical system. This comprehensive data collection helps us understand the impact of severe space weather in Mexico. We also gathered observations from the public about the aurora and measured the regional effects on geomagnetic and ionospheric conditions, cosmic rays, and technological systems. Key Points: The May 2024 geomagnetic storm offered insights into space weather effects at low latitudes, impacting North AmericaObservations showed geomagnetic storm impacts on Mexico's power grid and atmospheric conditions, highlighting vulnerabilitiesPolar auroras in Mexico reveal solar weather's reach, stressing the need for awareness in areas rarely affected by such events [ABSTRACT FROM AUTHOR]

Background: Alternating hemiplegia of childhood (AHC) is a rare disorder with both neurologic and cardiac manifestations. The ATP1A3-D801N variant is associated with a pathologically short QT interval and risk of ventricular arrhythmia following bradycardia; however, the mechanism of this remains unknown. We investigated the relationship between heart rate (HR), QT, and QTc, hypothesizing that individuals with ATP1A3-D801N have abnormal, impaired shortening of QT and QTc at lower HR leading to arrhythmia predisposition., Methods: We performed a retrospective observational study of individuals who underwent clinical evaluation, Holter monitoring, and genetic testing for AHC at Duke University Hospitals. We also compiled a group of healthy individuals as a control cohort. A larger, worldwide cohort of individuals with ATP1A3 -related phenotypes was compiled to investigate sinus node dysfunction. Linear regression analysis was then performed., Results: The cohort consisted of 44 individuals with ATP1A3 -related phenotypes with 81 Holter recordings (52.27% female; mean age at first Holter 8.04 years, range 0.58 - 33 years), compared to 36 healthy individuals with 57 Holter recordings (52.78% female; mean age at first Holter 9.84 years, range 0.08 - 38 years). Individuals with ATP1A3-D801N had reduced prolongation of QT at lower HR, manifest as a significantly lower slope for HR vs QT compared to healthy (P<0.0001). This resulted in a significantly higher slope of the relationship for HR vs QTc compared to healthy (P<0.0001). Individuals with ATP1A3 -related phenotypes and baseline QTc <350 milliseconds (ms) had increased shortening of QT and QTc at lower HR compared to those with normal QTc (P=0.003; P=0.001). Among worldwide cases, 3 out of 131 individuals with ATP1A3 -related phenotypes required device implantation and/or had sinus pauses >4 seconds., Conclusions: Individuals with the ATP1A3-D801N variant exhibit paradoxical shortening of QT and QTc at lower HR, which contributes to an increased risk of arrhythmias during bradycardia. This is exacerbated by an underlying risk of sinus node dysfunction., Clinical Perspective: What is Known:Individuals with ATP1A3-D801N have a short baseline QTc.Two individuals with AHC experienced ventricular fibrillation following bradycardia.What the Study Adds:The QT and QTc shorten to a greater extent at lower heart rate in individuals with ATP1A3-D801N than in healthy individuals. Individuals with ATP1A3 -related phenotypes and QTc <350ms show greater impairment of QT and QTc dynamics than those with normal QTc. There is low prevalence of device implantation and significant sinus pauses in individuals with ATP1A3 -related phenotypes, with a relatively greater prevalence in those with ATP1A3-D801N.

Developmental and epileptic encephalopathies (DEEs) are severe forms of epilepsy characterized by seizure onset in infancy or childhood. The seizures are typically drug-resistant and often accompanied by significant alterations in the electroencephalogram (EEG). DEEs are associated with neurodevelopmental impairment, which can arise from both the epileptic activity itself and the underlying etiology, which is most often genetic in origin. We present the clinical and molecular features of two patients with DEE associated with a pathogenic variant in the UGDH gene. This gene encodes a protein that converts uridine diphosphate (UDP)-glucose into UDP-glucuronate, which plays a crucial role in the biosynthesis of glycosaminoglycans, essential components of the connective tissue and extracellular matrix. Both patients started with epileptic spasms associated with a pattern of hypsarrhythmia in the EEG at 4 months of age. Both developed global developmental delay and the physical examination revealed hypotonia and mildly dysmorphic features. In both families, there was another affected sibling with a similar clinical presentation, although genetic studies were not performed in one of these children. A homozygous pathogenic variant in the UGDH gene, NM&#95;003359.4:c.131C>T - p.(Ala44Val), previously reported to be associated with the described phenotype, was identified., (© 2024 International League Against Epilepsy.)

Background: Severe combined immunodeficiency secondary to adenosine deaminase deficiency is rare. The deficiency of this enzyme results in the accumulation of substrates in the tissues, including the brain. Clinical signs of neurological involvement may include seizures, neurodevelopmental disorders, hypotonia, and sensorineural hearing loss. Hematopoietic stem cell transplantation corrects the failure of the immune system but not the neurological involvement., Objectives: To describe the spectrum of neurological complications identified in a series of children with severe combined immunodeficiency due to adenosine deaminase deficiency. Additionally, we propose a neurological approach including electrophysiological, radiological, and neurocognitive studies to address this group of children in an efficient and timely manner., Methods: A descriptive, observational, retro-, and prospective analysis of patients with a confirmed immunological diagnosis seen between 1996 and 2021 and referred to the Department of Neurology for neurological evaluation was conducted., Results: Ten patients met the inclusion criteria. The median age at diagnosis was 4 months (range, 1-36 months). All patients had neurodevelopmental delay with hypotonia in six, language delay in three, sensorineural hearing loss in four, and spastic paraparesis in one patient. Two children developed an epileptic syndrome, consisting of generalized epilepsy in one and focal epilepsy in the other. Neuroimaging showed brain calcifications in the basal ganglia and/or centrum semiovale in four patients and enlarged subarachnoid spaces in two other patients., Conclusion: In this pediatric series, the rate of neurological involvement associated with abnormalities on neuroimaging was high. Although this involvement could be related to accumulation of adenosine metabolites in the central nervous system, the possibility of associated chronic infections should be ruled out. Given the neurological manifestations, it is important to involve the pediatric neurologist in the multidisciplinary follow-up team., Competing Interests: Declaration of competing interest The authors declare that there are no competing interests., (Copyright © 2024 Elsevier Inc. All rights reserved.)

Aim: To determine clinical, electroencephalographic, therapeutic and evolutive characteristics of a series of oncopediatric patients with acute symptomatic seizures., Patients and Methods: We performed a retrospective and prospective descriptive analysis of clinical records of oncopediatric children evaluated by neurology at the comprehensive outpatient Center for Hemato-Oncological Patients during 2017-2021. We included children aged one month to 17 years with intracranial and extracranial tumors who presented with acute symptomatic seizure (ASC). We defined acute symptomatic seizure according to the 2010 International League Against Epilepsy. We classified seizures according to 2017 International League Against Epilepsy classification. We excluded any patient with a diagnosis of previous epilepsy and non-epileptic paroxysmal episodes., Results: We analyzed 44 cases with a median of 4 years (range: 1 month-17 years) and mean of 5.75 months (range: 1 month-11 months) and 8.33 years (2-17 years). The main etiologies were neurotoxicity and post-surgical context. Four patients presented dysnatremias and two associated with endocranial hypertension. Forty-one electroencephalograms were performed with intercritical results with abnormalities in the baseline rhythm, but without foci or paroxysms. There were no critical recordings. Focal seizures were 25 (56.8%) and generalized seizures 19 (43.18%). Levetiracetam was the most commonly used drug for acute management., Conclusions: Our cohort shows that ASC, in this population, do not show considerable differences between focal motor and generalized seizures and occur mostly in neurotoxic and post-surgical contexts. Dysnatremias and endocranial hypertension associated with ASC were also recorded. Postcrisis electroencephalograms were without foci or paroxysms and good seizure evolution.

Objective: The aim of this study was to assess efficacy, safety, and tolerability of highly purified cannabidiol oil (CBD) as add-on therapy for the treatment of a series of patients with infantile epileptic spasms syndrome (IESS) who were resistant to antiseizure medications and ketogenic dietary therapy., Material and Methods: We conducted a retrospective analysis of the medical records of 28 infants with treatment-resistant IESS aged 6 to 21 months who received highly purified CBD between July 2021 and June 2023. Data were collected on neurological examinations, EEG, Video-EEG and polygraphic recordings, imaging studies, laboratory testing, and seizure frequency, type, and duration, and adverse effects. As the primary outcome, a reduction of frequency of epileptic spasms (ES) was assessed. ES freedom was considered after a minimal time of 1 month without ES., Results: Sixteen male and 12 female patients, aged 6-21 months, who received CBD for treatment-resistant IESS were included. The etiology was structural in 10, Down syndrome in seven, genetic in nine, and unknown in two. Initial CBD dose was 2 mg/kg/day, which was uptitrated to a median dose of 25 mg/kg/day (range, 2-50). Prior to CBD initiation, patients had a median of 69 ES in clusters per day (range, 41-75) and of 10 focal seizures per week (range, 7-13). After a mean and median follow-up of 15 and 12.5 months (range, 6-26 months), seven patients were ES free and 12 had a >50 % ES reduction. Five of seven patients (71 %) with Down syndrome and 3/5 (60 %) with cerebral palsy responded well. Adverse effects were mild. EEG improvements correlated with ES reductions., Conclusion: In this study evaluating the use of CBD in children with IESS, 19/28 (67.8 %) had a more than 50 % ES reduction with good tolerability., Competing Interests: Declaration of competing interest The authors declare they have no conflict of interest regarding this study., (Copyright © 2024 British Epilepsy Association. Published by Elsevier Ltd. All rights reserved.)

Dravet syndrome is currently considered as an developmental and epileptic encephalopathy and, recently, mandatory, alert, and exclusionary criteria have been proposed. Here, we describe three patients with Dravet syndrome with the typical early presentation including febrile and afebrile alternating hemiclonic seizures due to loss-of-function SCN1A variants. Subsequently, they developed episodes of febrile focal status epilepticus (SE) associated with hemiparesis and cerebral hemiatrophy with posterior focal seizures, as a consequence of Dravet syndrome. This sequence of events has been previously published in patients with Dravet syndrome and does not contradict the recent classification by the International League Against Epilepsy (ILAE). The ILAE guidance identifies "Focal neurological findings" as alert criteria and "MRI showing a causal focal lesion" as exclusionary criteria for making an initial diagnosis of Dravet syndrome at presentation. Our three patients would correspond to a severe phenotype, similar to the well-known presentation of generalized atrophy following prolonged status epilepticus. Common genetic findings in cases of diffuse and unilateral brain involvement may help explain these clinical presentations. Further genotype-phenotype studies may provide additional insights into this electroclinical behavior., (© 2023 International League Against Epilepsy.)

Importance: Pudendal nerve block has been frequently used as a pain management modality for vaginal prolapse surgery. However, studies investigating its efficacy and the type of anesthetic used have had conflicting results., Objective: This study aimed to evaluate the effect of intraoperative pudendal nerve block with liposomal bupivacaine on postoperative pain after sacrospinous ligament suspension surgery., Study Design: In this single-blinded randomized controlled trial, 83 women undergoing sacrospinous ligament suspension were randomized to receive either intraoperative pudendal nerve block with liposomal bupivacaine or no block. Participants recorded their pain level on postoperative days (PODs) 1-3 and 7, the number of pain medication pills consumed, and the quality of their recovery using a validated questionnaire., Results: There were no significant differences in pain scores between the groups on POD 1 and POD 3: 5 (2-6.25) versus 5.5 (4-8; P = 0.058) and 4 (1-6) versus 5 (2-7; P = 0.146), respectively. On POD 2, the overall pain score was statistically different between the groups, with the block group having lower interquartile ranges. This difference, however, was not clinically significant: 5 (1.75-5) versus 5 (3.25-7.75; P = 0.023). In the subset of participants who underwent a concomitant midurethral sling procedure, POD 2 and POD3 pain scores were significantly lower in those who received the block: 3 (1-5) versus 6 (5-8; P = 0.006) for POD 2 and 3 (1-5) versus 5 (3.250-7; P = 0.042) for POD 3. There were no significant differences in pain medication consumption or the quality of recovery scores., Conclusion: Pudendal nerve block with liposomal bupivacaine did not result in significant differences in postoperative pain after sacrospinous ligament suspension, except in those participants who underwent a concomitant midurethral sling procedure., (Copyright © 2023 American Urogynecologic Society. All rights reserved.)

The identification of factors that affect cannabidiol (CBD) systemic exposure may aid in optimizing treatment efficacy and safety in clinical practice. In this study, we aimed to correlate CBD plasma concentrations at a steady state to demographic, clinical, and pharmacological characteristics as well as seizure frequency after the administration of a purified CBD oil solution in a real-world setting of children with drug-resistant developmental and epileptic encephalopathies (DEEs). Patients receiving oral CBD pharmaceutical products at maintenance were enrolled. Venous blood samples were drawn before the CBD morning dose, 12 h apart from the last evening dose (C0 or CBD trough concentration). A linear mixed-effect analysis was implemented to assess the correlation between C0 and clinical, laboratory, pharmacological, and lifestyle factors. Fifteen females and seven males with a median age of 12.8 years (ranging between 4.7 and 17.2) were included. The median CBD dose was 8.8 mg/kg/day (ranging between 2.6 and 22.5), and the CBD C0 median (range) was 48.2 ng/mL (3.5-366.3). The multivariate model showed a 109.6% increase in CBD C0 in patients with concomitant levothyroxine (β = 0.74 ± 0.1649, p < 0.001), 56.8% with food (β = 0.45 ± 0.1550, p < 0.01), and 116.0% after intake of a ketogenic diet (β = 0.77 ± 0.3141, p < 0.05). All patients included were responders without evidence of an association between C0 and response status. In children with DEEs, systemic concentrations of CBD may be significantly increased when co-administered with levothyroxine, food, or a ketogenic diet.

Purpose: This retrospective study aimed to evaluate the efficacy and tolerability of sulthiame (STM) as an add-on treatment in 49 patients with non-self-limited focal epilepsies of childhood (non-SeLFE) resistant to other antiseizure medications (ASM) and/or non-pharmacological treatment., Methods: Patients with non-SeLFE who had failed to respond to at least five previous ASM, alone or in combination, were included in the study. All patients underwent neurological examination, brain magnetic resonance imaging repeated prolonged electroencephalography (EEG) or video-EEG studies, and neurometabolic studies. School achievements and/or performance on neuropsychological tests were also assessed. Sulthiame was added in doses ranging from 10 to 40 mg/kg/day. Efficacy was measured by comparing seizure frequency before and after initiating STM therapy., Results: Twenty-nine of 49 patients (59.1%) who received STM as add-on therapy had a greater than 50% decrease in seizures after a mean follow-up of 35 months. One patient (2%) became seizure-free. Fourteen patients (40%) had a 25-50% seizure reduction. The mean time of response was 5 months (range, 3.5 to 6 months). No differences were found either between patients with a response of more or less than 50% or between the response of the focal seizure types (motor or non-motor, with or without consciousness impairment)., Conclusion: In our study, STM was found to be effective and well-tolerated in children and adolescents with non-SeLFE. In the patients who responded, improvement in the EEG was seen., Competing Interests: Declaration of Competing Interest The authors declare that they have no known competing financial interests or personal relationships that could have appeared to influence the work reported in this paper., (Copyright © 2023 Elsevier Inc. All rights reserved.)

Objective: The study was conducted to analyze the possible diagnostic value of the electroclinical semiology of the epileptic seizures., Methods: We evaluated the medical records of 17 females and 5 males with CDKL5 deficiency disorder (CDD) considering the long-term evolution, including the polygraphic video-EEG recordings., Results: We recognized three disease phases. We found that the seizure semiology was already recognizable in the first phase of the syndrome. In the short-term evolution, all patients had focal motor and 12/21 hypermotor seizures. Both epileptic spasms and myoclonic seizures were already present in more than half of the cases in the first 2 months after onset. In the second phase, the intermediate period, the polymorphic pattern was maintained, but in eight patients the electroclinical pattern of epileptic encephalopathy with hypsarrhythmia appeared. In the long-term period, the seizure polymorphism continued but myoclonic and epileptic spasms diminished. Tonic seizures appeared in the last 2 phases. Progressively, with the aggravation of seizures and paroxysmal EEG abnormalities impairment of the neurocognitive status was observed. Severe behavioral disturbances were seen in eight and autistic-like features in 14., Conclusion: CDD is a true developmental and epileptic encephalopathy with a specific etiology characterized by the early appearance of epileptic seizures that quickly become polymorphic and drug resistant in infants that are most often female and already have neurological impairment. Polygraphic video-EEG recordings are important to recognize ictal events of the association of hypermotor seizures, epileptic spasms in clusters, and massive myoclonic jerks, already present at onset., (Copyright © 2023 Elsevier B.V. All rights reserved.)

Objective: The aim of the study was to determine whether an easy-to-read patient education card given at the preoperative visit can increase patient recall of the planned surgery., Methods: This was a randomized controlled trial. Patients scheduled to undergo pelvic reconstructive surgery were recruited during their preoperative visits. All participants received standard surgical counseling, whereas the intervention group also received a 4 × 6 inch card highlighting the anticipated procedure. The primary outcome was correct recall of the planned surgery as measured by a preoperative questionnaire. Secondary outcomes were correct recall of the surgery postoperatively and patient satisfaction with the information provided., Results: One hundred twenty-eight patients were enrolled with 64 participants in each arm. One hundred twenty-seven participants were analyzed because 1 patient was lost to follow-up. No difference was found between patient demographics, including types of surgical procedures performed. There was a statistically significant improvement in preoperative recall: 30 of 63 participants (47.6%) in the intervention group answered all questions correctly versus 18 of 64 patients (28.6%) patients in the standard counseling group (P = 0.021). There was no difference in the postoperative scores between the 2 groups: 48.3% (28 of 58) and 52.5% (32 of 61) of the participants answered all questions correctly in the intervention and standard counseling only groups, respectively (P = 0.648). There was no difference in satisfaction scores, with a median score of 20 out of 20 for either group (interquartile range = 19-20)., Conclusions: A concise and easy-to-use education card enhanced patient preoperative recall of the proposed surgery. This difference was not sustained postoperatively. High satisfaction with the information provided was reported regardless of counseling method., Competing Interests: The authors have declared they have no conflicts of interest., (Copyright © 2021 American Urogynecologic Society. All rights reserved.)

Introduction: Initiation of ketogenic diet therapies (KDT) for pediatric epilepsy is usually done on an inpatient basis and the diet is managed during clinical appointments following a protocol of visits and routine tests. Because of the 2019 coronavirus disease (COVID-19) pandemic and the associated lock-down measures, we switched from outpatient to telemedicine-based KDT initiation., Objective: To explore the feasibility, effectiveness, and safety of online KDT initiation and follow-up by comparing a group of children with drug-resistant epilepsy that was managed by telemedicine compared to a group that was treated on an outpatient basis., Materials and Methods: An observational study was conducted in two groups of patients with drug-resistant epilepsy who initiated KDT and were followed up with an online versus an outpatient modality by the interdisciplinary KDT team of Hospital Pediatria JP Garrahan in Buenos Aires, Argentina. Dietary compliance, ketosis, retention rate, adverse effects, number of contacts, and clinical outcome were evaluated at 1, 3, and 6 months on the diet., Results: Overall, 37 patients were included, of whom 18 started the KD by telemedicine and 19 on an outpatient basis. Minimum follow-up of the patients was 6 months. All patients received the classic ketogenic diet. No statistical differences between the two groups regarding efficacy and safety of the diet were found., Conclusions: Our results support the feasibility and safety of initiating and management of KDT by telemedicine. Patients and their families should be carefully selected in order to guarantee a good outcome., (Copyright © 2022. Published by Elsevier Ltd.)

Sialic acid (SA) is a monosaccharide usually linked to the terminus of glycan chains on the cell surface. It plays a crucial role in many biological processes, and hypersialylation is a common feature in cancer. Lectins are widely used to analyze the cell surface expression of SA. However, these protein molecules are usually expensive and easily denatured, which calls for the development of alternative glycan-specific receptors and cell imaging technologies. In this study, SA-imprinted fluorescent core-shell molecularly imprinted polymer particles (SA-MIPs) were employed to recognize SA on the cell surface of cancer cell lines. The SA-MIPs improved suspensibility and scattering properties compared with previously used core-shell SA-MIPs. Although SA-imprinting was performed using SA without preference for the α2,3- and α2,6-SA forms, we screened the cancer cell lines analyzed using the lectins Maackia Amurensis Lectin I (MAL I, α2,3-SA) and Sambucus Nigra Lectin (SNA, α2,6-SA). Our results show that the selected cancer cell lines in this study presented a varied binding behavior with the SA-MIPs. The binding pattern of the lectins was also demonstrated. Moreover, two different pentavalent SA conjugates were used to inhibit the binding of the SA-MIPs to breast, skin, and lung cancer cell lines, demonstrating the specificity of the SA-MIPs in both flow cytometry and confocal fluorescence microscopy. We concluded that the synthesized SA-MIPs might be a powerful future tool in the diagnostic analysis of various cancer cells.

Introduction: Noticing health warnings on cigarette packages has been associated with thinking about quitting. This study examined sociodemographic characteristics associated with awareness of health warnings on cigarette packages and thinking about quitting because of health warning labels among adults who currently smoked tobacco., Methods: We analyzed data from the 2017 Zambia WHO STEPS survey (STEPwise approach to surveillance) for noncommunicable disease risk factors. Descriptive analyses and logistic regression were performed to assess the association of select sociodemographic characteristics with awareness of health warnings and thinking about quitting because of health warnings., Results: Adults who currently smoked tobacco who were aged 30-44 years, of Chewa ethnicity, or with a formal education, were more likely to be aware of health warnings than those aged 18-29 years (adjusted prevalence ratio, APR=1.26; 95% CI: 1.02-1.54), of Bemba ethnicity (APR=1.43; 95% CI: 1.17-1.74), or with no formal education (APR: 2.61-5.95). Among all adults who currently smoked, those of Chewa ethnicity (APR=1.55; 95% CI: 1.03-2.35), or with a formal education (APR:1.80-4.38), were more likely to report thinking about quitting because of health warnings than those who were of Bemba ethnicity or with less than primary school education level. Women who currently smoked were 49% less likely (APR=0.51; 95% CI: 0.23-0.84) to report thinking about quitting than men. Among a subset of adults who currently smoked who were aware of health warning labels, no sociodemographic characteristics were significantly associated with thinking about quitting in unadjusted or adjusted models., Conclusions: Sociodemographic characteristics such as sex, ethnicity, and education level were significantly associated with awareness of cigarette health warnings. Among cigarette smokers aware of health warnings, no sociodemographic differences in thinking about quitting were found. Tobacco control campaigns may need to target people of ethnicities with the highest smoking prevalence in the country., Competing Interests: The authors have completed and submitted the ICMJE Form for Disclosure of Potential Conflicts of Interest and none was reported., (© Phiri M. M. et al.)

Objective: We report our findings regarding effectiveness, safety, and tolerability of cannabidiol (CBD)-enriched medical cannabis as add-on therapy in children with drug-resistant epileptic encephalopathies (DEEs) after a median follow-up of 20 months., Methods: A prospective cohort study was conducted to assess effectiveness, safety, and tolerability of CBD-enriched medical cannabis oil added to standard antiseizure medications in children with drug-resistant DEE seen at a single center., Results: Between October 2018 and March 2020, 59 patients were enrolled. Mean age at enrollment was 10.5 years (range, 2-17 years). Median treatment duration was 20 months (range, 12-32). Median age at first seizure was 8 months (range, 1 day - 10 years). At the end of follow-up, 78% of the children had a ≥ 50% decrease in seizure frequency and 47.5% had a > 75% decrease. Seven patients (11.9%) were seizure free. The number of seizures was reduced from a median of 305/month to 90/month, amounting to a mean reduction of 57% and a median reduction of 71% (p < 0.0001). Adverse effects were mostly mild or moderate. CBD was discontinued in 17 patients (28.8%) due to lack of response to treatment, increased seizure frequency, intolerance to the drug, or poor compliance., Conclusion: In children with drug-resistant DEEs, long-term treatment of CBD-enriched medical cannabis as an adjuvant therapy to antiseizure therapy was found to be safe, well tolerated, and effective. Sustained reductions in seizure frequency and improvement of aspects of daily living were observed compared to our preliminary findings., (Copyright © 2022 British Epilepsy Association. Published by Elsevier Ltd. All rights reserved.)

Coxsackievirus A24 variant (CVA24v) is responsible for several outbreaks and two pandemics of the highly contagious eye infection acute hemorrhagic conjunctivitis (AHC). Currently, neither prevention (vaccines) nor treatments (antivirals) are available for combating this disease. CVA24v attaches to cells by binding Neu5Ac-containing glycans on the surface of cells which facilitates entry. Previously, we have demonstrated that pentavalent Neu5Ac conjugates attenuate CVA24v infection of human corneal epithelial (HCE) cells. In this study, we report on the structure-based design of three classes of divalent Neu5Ac conjugates, with varying spacer lengths, and their effect on CVA24v transduction in HCE cells. In relative terms, the most efficient class of divalent Neu5Ac conjugates are more efficient than the pentavalent Neu5Ac conjugates previously reported., Competing Interests: There are no conflicts to declare., (This journal is © The Royal Society of Chemistry.)

Objective: We studied cases with long-lasting epileptic spasms (ES) considered as a spasm status analyzing type of epilepsy, epileptic syndrome, etiology, treatment, and outcome in 21 patients., Methods: We evaluated the charts of 21 patients seen between June 2006 and July 2017 who met the electroclinical diagnostic criteria of a spasm status. The spasm status was defined as continuous ES lasting 30 min or longer., Results: The type of ES was mixed in nine patients, flexion in seven, and extension in five. Epileptic spasms were asymmetric in three patients and unilateral in two. They occurred on awakening in all patients, while during sleep they decreased in all and disappeared in three cases. The duration of the spasm status ranged from 40 min to 15 days according to the seizure diaries of patients and video-EEG recordings. Two well-defined subgroups of patients were recognized; the first included patients with West syndrome (WS) and the second other types of severe non-West epilepsy syndromes. The spasm status responded well to oral vigabatrin (VGB) in four patients, oral topiramate (TPM) in three, oral corticosteroids in one, and cannabidiol in another patient. A good response was observed with benzodiazepines in six patients, with phenytoin (PH) in two, and with phenobarbital (PB) in one. Adrenocorticotropic hormone (ACTH) was effective in one patient and the ketogenic diet in two. Prognosis depends on the etiology., Conclusion: In this study we identified patients with WS and other types of severe non-West epilepsy syndromes who had a particular type of long-lasting ES that, in spite of its long duration does not strictly meet the criteria of the International League against Epilepsy (ILAE) classification of status epilepticus, may be considered a spasm status., Competing Interests: Declaration of Competing Interest The authors declare that they have no known competing financial interests or personal relationships that could have appeared to influence the work reported in this paper., (Copyright © 2021 Elsevier Inc. All rights reserved.)

Background: Hypertension and diabetes are highly prevalent conditions in Chilean adults., Aim: To describe the demographic and clinical profiles, risk factors and complications associated with arterial hypertension (AH) and diabetes mellitus (DM) in patients ascribed to a cardiovascular health program at a public primary health care center in Santiago., Material and Methods: Review of medical records of 583 patients aged 37 to 95 years (56% women). Gender, age, smoking habits, blood pressure, glycated hemoglobin levels, LDL cholesterol and triglycerides levels, nutritional status in the last control carried out, and associated cardiovascular complications were recorded., Results: Thirty four percent (201 participants) and 36% (210 participants) had a decompensated DM and AH, respectively. Dyslipidemia was the main associated cardiovascular risk factor. The prevalence of obesity was 43% (249 participants). Twenty percent had chronic kidney disease and 13% had diabetic retinopathy., Conclusions: These patients have a high frequency of obesity, dyslipidemia, and chronic kidney disease.

Introduction: Ketogenic diet therapy (KDT) is a metabolic treatment with proven effectiveness for the treatment of drug-resistant epilepsy in children. Although previously not used in infants under 2 years of age, recent studies have shown KDT to be highly effective and well tolerated in infants with epilepsy, especially those with epileptic encephalopathies. Here, we describe the effectiveness and tolerability of the diet in infants up to 2 years of age., Material and Methods: A prospective study was conducted in a cohort of infants younger than 2 years of age with drug-resistant epilepsy who received the classic ketogenic diet using a specific protocol at a single center in Argentina., Results: 56 infants with treatment-refractory epilepsy were evaluated. The etiology was genetic in 21.4%, structural in 28.6%, unknown in 44.7%, and metabolic in 5.4%. At 3 months, a > 50% decrease in seizure frequency was observed in 35 patients (62.4%), of whom 11 (19.6%) became seizure free. At 6 months, 34 patients (60.7%) had a decrease in seizure frequency of > 50%, of whom 10 (17.8%) were seizure free. At the one-year follow-up, 27 patients (48.2%) had a > 50% decrease in seizure frequency, of whom six (10.7%) were seizure free. At two years, 14 patients (25%) had a > 50% seizure control, of whom four (7.1%) were seizure free. The most common early adverse effects were hypoglycemia and vomiting, while after 1 month and beyond metabolic acidosis, vomiting, and constipation more commonly found. A trend towards a higher rate of acute adverse events in infants younger than 1 year was observed., Conclusions: CKD showed to be a useful option in infants with treatment-resistant epilepsy. Adverse effects were common, but not a reason to discontinue the diet. Further studies are necessary to evaluate in which epilepsy syndromes and etiologies KDT is most effective., (Copyright © 2021 Elsevier B.V. All rights reserved.)