Giant cell arteritis (GCA) as a risk factor for seizures: a cohort study.

OBJECTIVES: The objective of this study was to assess the risk of seizures in Giant Cell Arteritis (GCA) patients in a large cohort of Israeli subjects, in comparison to matched controls. METHODS: Patients diagnosed with GCA between 2002 and 2017 were included. Controls were matched based on sex, age, socioeconomic status, country of birth, diabetes mellitus, and hypertension in a 4:1 ratio. Patients with seizure records prior to the study period were excluded. Hazard ratios for seizures was obtained by cox regression models. RESULTS: The study cohort was composed by 8,103 GCA patients and 32,412 matched controls. The GCA group included 5,535 women (68%), 2,644 patients born in Israel (33%), and 2,888 patients with low socioeconomic status (36%). The median age of this group was 71. During the followed cumulative person-years of 54,641 and 222,537 in the GCA and control group, respectively, 15.92 cases per 10,000 person-years was found in the GCA group, compared to 9.62 per 10,000 person-years in the controls. GCA was associated with seizures in the unadjusted (HR = 1.66, 95% CI [1.29 to 2.13]) and adjusted (HR = 1.67, 95% CI [1.3 to 2.14]) models. GCA was also associated with seizures after controlling for strokes (HR = 1.55, 95% CI [1.16 to 2.07]). CONCLUSION: According to this study, individuals with GCA are at a higher risk of developing seizures when compared to the general population. This increased risk is independent of their predisposition for stroke. One proposed mechanism is that the GCA pro-inflammatory state may decrease the neuronal threshold for depolarization.

Etiological Profile of First Episode Seizures in Paediatric Patients at a Tertiary Care Centre: A Descriptive Cross-sectional Study.

INTRODUCTION: Seizures are the most common neurological emergency and one of the most common reasons for paediatrics hospital admissions. This study aimed to identify the etiology, clinical profile, and immediate outcome of children with the first episode of seizure in Eastern Nepal. METHODS: This was a prospective descriptive study carried out in the Tertiary Care Centre from September 2022 to August 2023. Ethical clearance was taken from the Institutional Review Committee (Ref no:654/2022). Convenience sampling was done to include 170 children presenting with the first episode of seizure at age 6 months to 15 years. Variables collected were demographics, clinical presentations, family history, trauma history, laboratory tests, neuroimaging, EEG, final diagnosis, and immediate outcome. RESULTS: A total of 170 patients were admitted with the first episode of seizure with 123 (72.36%) males and 47 (27.64%) females. The mean age of the patients was 5.13±2.95 years with 104 (61.18%) patients under 5 years of age. The most common seizure was generalized tonic-clonic type in 132 (77.64%) patients. The most common associated symptom was fever in 150 (88.23%) children. Neuroimaging was abnormal in 52 (30.59%) patients, with neurocysticercosis seen in 27 (15.88%). The most common etiology was febrile seizure in 92 (54.17%) patients, neurocysticercosis in 27 (15.88%), and meningitis in 12 (7.05%). CONCLUSIONS: Febrile seizures, neurocysticercosis, infection, and trauma were the major causes of seizures in children. When simple febrile seizures were unlikely, lumbar puncture, neuroimaging, and laboratory tests were useful tools for diagnosing etiologies of seizures.

Unveiling post-COVID-19 Rasmussen's encephalitis: a case report of rare neurological complication.

Introduction and importance: Rasmussen encephalitis (RE) is a rare autoimmune disorder that causes unilateral inflammation of the cerebral cortex and can lead to drug-resistant epilepsy and progressive neurological decline. Although the emergence of RE following COVID-19 has not been well documented, it emphasizes the need to understand the impact of COVID-19 on neurological health. This case emphasizes the importance of early recognition and intervention to prevent adverse outcomes related to post-COVID-19 neurological complications. Case presentation: A 30-year-old woman, recently diagnosed with COVID-19, experienced recurrent seizures that primarily affected the left side of her body. Despite medical management, signs of progressive weakness and altered consciousness were observed. Neurological examination, imaging, and electroencephalography confirmed a diagnosis of post-COVID-19 RE. Despite conservative management, the patient's condition continued to deteriorate, ultimately resulting in fatal outcomes. Clinical discussion: The relationship between COVID-19 and autoimmune responses, which can lead to neurological complications, such as RE, is a matter of concern. Accurate diagnosis of RE depends on imaging and EEG studies; however, a definitive diagnosis often requires histopathological examination. The management of RE involves the use of anti-seizure medications and surgical interventions to control symptoms and improve outcomes. However, the unusual presentation of this case, along with challenges in diagnosis and treatment, underscores the need for increased awareness and extensive research on the neurological consequences of COVID-19. Conclusion: This case underscores the severe neurological consequences that can emerge after COVID-19, emphasizing the need for prompt identification and intervention. Additional research is essential to improve the comprehension and management of the neurological aftermath of COVID-19 with the ultimate goal of enhancing patient outcomes.

Vascular Anomaly Presenting as Neurological Crisis: A Case Report of Dolichoectasia-Induced Seizures.

A rare brain vasculature illness called dolichoectasia (DE) causes the blood vessels to enlarge and elongate. DE describes an irregular enlargement and dilatation of arteries; this condition primarily affects big blood vessels such as the internal carotid and vertebrobasilar arteries. A range of neurological symptoms may result from this illness because of the compression of nearby structures or changed hemodynamics. Although it is documented more commonly in the vertebrobasilar circulation, rarely it can be seen in the anterior circulation, as in our case. The brainstem structures and cranial nerves are compressed in DE, resulting in turbulent blood flow that causes vertigo and tinnitus and frequently damages the brain's small blood vessels. One risk factor for ischemic stroke is DE. The occurrence of seizures in DE is not so common. Here, we report a case of a 20-year-old female, bioengineering UG with generalized tonic-clonic seizure diagnosed to have DE on MRI.

Efficacy and safety of stiripentol in the prevention and cessation of status epilepticus: A systematic review.

Status epilepticus (SE) is a life-threatening emergency with high morbidity and mortality. In people with epilepsy, the management of SE is focused on early medical treatment. Stiripentol is a third-generation antiseizure medication (ASM) approved for refractory generalized tonic-clonic seizures in Dravet syndrome. The aim of this systematic review was to evaluate the effectiveness and safety of stiripentol in reducing the incidence of SE in patients with Dravet syndrome or any epilepsy characterized by recurrent SE. The PubMed and Cochrane databases were systematically searched, and gray literature was hand-searched. Search results were screened by title and abstract; studies with data on the effect of stiripentol on SE outcomes, including the cessation of SE, reduction in number of SE episodes, or reduction in hospitalizations, were included. Of 66 records identified, 17 studies were eligible for inclusion, of which 15 were human studies (n = 474; aged 1.1-78 years), and two were animal experiments. Results of retrospective or prospective observational studies showed that stiripentol as add-on therapy to ASMs such as clobazam or valproate reduced the incidence of SE in patients with Dravet syndrome or other developmental and epileptic encephalopathies (DEEs). A mean of 68% of patients (range 41%-100%) had a ≥50% reduction in SE episodes from baseline, and 26%-100% of patients (mean 77%) became SE-free after stiripentol initiation. Moreover, this review found stiripentol, used as acute treatment, may also be effective for the cessation of super-refractory SE, but data are limited to three retrospective case series. Stiripentol was generally well-tolerated. In conclusion, stiripentol reduces the incidence of SE episodes in patients with Dravet syndrome and potentially other DEEs, and it promotes cessation of super-refractory SE in patients with and without a history of seizures. PLAIN LANGUAGE SUMMARY: Status epilepticus (SE) is a life-threatening, long-lasting seizure occurring in patients with/without epilepsy. This article analyzed 15 published studies that investigated the effects and safety of the anti-seizure medication stiripentol for preventing SE in epilepsy patients (prevention) or stopping an SE episode (cessation), and two animal studies that investigated how stiripentol works. In epilepsy patients, stiripentol halved the number of SE episodes in 41-100% of patients, 26-100% of patients became SE-free, and stiripentol was considered to be well tolerated. In patients with/without epilepsy, stiripentol may stop the SE episode after other drugs like anesthetics have not worked.

Assessment of Adaptive Functioning and the Impact of Seizures in KBG Syndrome.

This study aimed to examine the adaptive functioning status and the impact of epileptic seizures on neurocognitive outcomes in KBG syndrome, a rare genetic neurodevelopmental disorder characterized by pathogenic variants in ANKRD11. A single clinician interviewed individuals and families with genetically confirmed cases of KBG syndrome. Trained professionals also conducted assessments using the Vineland-3 Adaptive Behavior Scales. The assessment covered the domains of communication, daily living skills, socialization, and maladaptive behaviors, and then compared individuals with and without epilepsy. Further comparisons were made with data from interviews and participants' medical records. Thirty-nine individuals (22 males, 17 females) with KBG syndrome, confirmed through genetic analysis, were interviewed via videoconferencing, followed by Vineland-3 assessment by trained raters. Individuals with KBG syndrome came from 36 unique families spanning 11 countries. While the KBG cohort displayed lower overall adaptive behavior composite scores compared with the average population, several members displayed standard scores at or higher than average, as well as higher scores compared with those with the neurodevelopmental disorder Ogden syndrome. Within the KBG cohort, males consistently scored lower than females across all domains, but none of these categories reached statistical significance. While the group with epilepsy exhibited overall lower scores than the nonseizure group in every category, statistical significance was only reached in the written communication subdomain. Our research provides insights that can aid in epilepsy screening and inform assessment strategies for neurocognitive functioning in those with this condition. The cohort performed overall higher than expected, with outliers existing in both directions. Although our results suggest that seizures might influence the trajectory of KBG syndrome, the approaching but overall absence of statistical significance between study groups underscores the need for a more extensive cohort to discern subtle variations in functioning.

Novel loss-of-function variants in WDR26 cause Skraban-Deardorff syndrome in two Chinese patients.

Introduction: Mutations in the protein WD repeat structural domain 26 (WDR26, MIM 617424) have been identified as the cause of autosomal dominant Skraban-Deardorff syndrome, a rare genetic disorder characterized by intellectual disability (ID), developmental delay (DD), hypotonia, epilepsy, infant feeding difficulties, gait abnormalities and distinctive facial features. The objective of this study is to investigate the genetic factors that may contribute to the development of Skraban-Deardorff syndrome in affected individuals. Methods: In this study, we used whole-exome sequencing (WES) to analyze pathogenic and likely pathogenic variants in two unrelated Chinese patients with DD and ID. We confirmed the origin of the variants by conducting Sanger sequencing and classified them according to ACMG/AMP guidelines. Results: Here, two novel de novo variants (c.1797delC(p.His599fs*11) and c.1414C>T(p.Gln472*)) in the WDR26 gene have been identified in two Chinese patients with Skraban-Deardorff syndrome. These patients exhibit a range of symptoms, including varying degrees of ID, DD, speech delay, an abnormal wide-foot and/or stiff-legged gait, facial dysmorphism, behavioural abnormalities, with or without seizures. Conclusions: In this study, We report two unrelated Chinese patients with Skraban-Deardorff syndrome caused by novel de novo pathogenic variants of the WDR26 gene. These patients showed a clinical phenotype similar to that of patients with the WDR26 variant. Compared to reported cases with WDR26 pathogenic variants, patient 2 presented a novel complication of severe behavioural problems, including hyperactivity, social anxiety, self-mutilation, impulsivity and violent behaviour. This research broadens the range of genetic and clinical features of Skraban-Deardorff syndrome. In addition, the symptoms may become more pronounced as the patient ages. Furthermore, our report highlights the clinical diversity of Skraban-Deardorff syndrome. The findings may assist healthcare professionals in providing more accurate genetic testing and counselling to affected families and improving the overall management of the condition.

A Comparison of Treatment Practices for Newborn Seizure Management Across Level II and III Neonatal Intensive Care Units in the United States.

BACKGROUND: Neonatal seizures (NS) represent an important clinical manifestation among critically ill infants and are often the first sign of underlying brain injury. Early recognition and treatment are essential to reduce morbidity and mortality. The present study investigated the NS management and treatment approaches employed by level II/III neonatal intensive care units (NICUs) across the United States to identify areas of consensus and variability. METHODS: Personnel associated with level II/III NICUs were directly surveyed with an electronic questionnaire. Access to neurology specialists, on-site electroencephalography (EEG) monitoring, and use of antiseizure medications was directly queried. A total of 51 NICUs participated in this survey. RESULTS: Twenty-five percent of the surveyed NICUs reported having an established clinical practice pathway available for treating NS. Twenty-four percent endorsed having written guidelines that provided a formal definition for the concept of "neonatal seizures." Although the majority of NICUs reported having phenobarbital available for rapid seizure management, most NICUs lacked access to additional antiseizure medications for treatment escalation. Twenty-four percent of the surveyed NICUs had no access to EEG monitoring available to them on-site. Daytime and overnight access to neurology consultants was limited and variable. CONCLUSIONS: Findings were consistent with a lack of equitable access for NS treatment. Areas of potential improvement include development and implementation of a protocol for rapidly treating NS that emphasizes enhanced access to EEG and rapid neurology consultation, acknowledging and improving upon resource limitations. These developments may eventually provide earlier detection, evaluation, and treatment of seizures in newborns, contributing to improved long-term outcomes.

Role of Nrf2 in Epilepsy Treatment.

Oxidative stress is a consequence of the disruption of the balance between the generation of reactive nitrogen and oxygen species and the biological system's ability to neutralize those reactive products. Oxidative stress is involved in the generation of many disorders, including epilepsy, which is a prevalent chronic neurological disease that affects the lives of millions of people around the world. Epilepsy is characterized by unforeseeable and repeated seizures that can be very disturbing. Studies have reported that oxidative stress occurs before and after seizures. A transcription factor named Nuclear factor erythroid-derived 2-related factor 2 (Nrf2) controls genes related to the induction of oxidative stress and defends cells against oxidative stress. The Nrf2 protein has seven different domains, ranging from Neh1 to Neh7. Each domain is responsible for a distinctive function of this protein. Keap1 binds to Nrf2, but during oxidative stress, Nrf2 detaches from the Keap1 protein, moves to the nucleus, and binds to DNA. The result of this translocation and binding is the initiation of transcription of detoxifying genes to control the harmful effects of oxidative stress. There is some evidence of oxidative stress involvement in epilepsy. In this review, we have listed potential Nrf2-related therapeutic targets for treating and controlling epilepsy, such as Berberis alkaloids, pentoxifylline, lovastatin, progesterone, and chrysin nanoparticles. These activators were tested in animals (in vivo) and cells (in vitro), and most of these experiments showed promising results in different epilepsy models. Finally, the results have suggested that the activation of Nrf2 can be an option for controlling epilepsy.

Youth and Family Perspectives on Diagnosis Communication About Pediatric Functional Seizures: A Qualitative Study.

BACKGROUND: This study aims to understand diagnosis communication experiences and preferences of youths with functional seizures and their parents. METHODS: Semistructured interviews with youths and their parents from a tertiary care children's hospital were conducted separately. We confirmed the diagnosis of functional seizures with the youth's treating providers. All interviews were audio recorded and professionally transcribed. Two coders performed thematic analysis and determined themes. RESULTS: Thirteen youths (aged 12 to 18) and 13 parents completed interviews separately. Themes included the following: (1) both parents and youths reported substantial fear about their symptoms, diagnosis, prognosis, and treatment options; (2) good communication skills highlighted included providing reassurance, listening, and allowing time for questions, with different preferences between youths and parents in the mode of communication; (3) poor communication for both youths and parents included use of medical jargon, brevity of communication especially with the youth, minimization of youth's symptoms, lack of apparent compassion, and lack of written materials to supplement discussions; (4) perspectives on the role of mental health in functional seizures were heterogeneous, yet concrete treatment plans were desired including access to behavioral therapy; and (5) youths and parents wanted practical guidance including plans for school and how to respond to functional seizures in all settings. CONCLUSIONS: Youths and parents provided concrete recommendations for optimal diagnosis communication. Study findings will inform the future development of an educational intervention to improve communication and create a supportive environment for youths with functional seizures.

Microglial TREM2 promotes phagocytic clearance of damaged neurons after status epilepticus.

In the central nervous system, triggering receptor expressed on myeloid cells 2 (TREM2) is exclusively expressed by microglia and is critical for microglial proliferation, migration, and phagocytosis. Microglial TREM2 plays an important role in neurodegenerative diseases, such as Alzheimer's disease and amyotrophic lateral sclerosis. However, little is known about how TREM2 affects microglial function within epileptogenesis. To investigate this, we utilized male TREM2 knockout (KO) mice within the murine intra-amygdala kainic acid seizure model. Electroencephalographic analysis, immunocytochemistry, and RNA sequencing revealed that TREM2 deficiency significantly promoted seizure-induced pathology. We found that TREM2 KO increased both the severity of acute status epilepticus and the number of spontaneous recurrent seizures characteristic of chronic focal epilepsy. Phagocytic clearance of damaged neurons by microglia was also impaired by TREM2 KO and reduced phagocytic activity correlated with increased spontaneous seizures. Analysis of human tissue from patients who underwent surgical resection for drug resistant temporal lobe epilepsy also showed a negative correlation between expression of the microglial phagocytic marker CD68 and focal to bilateral tonic-clonic generalized seizure history. These results indicate that microglial TREM2 and phagocytic activity are important to epileptogenic pathology.

A case of acute encephalopathy with hyperperfusion detected by arterial spin labelling: Extending spectrum of acute encephalopathy with biphasic seizures and late reduced diffusion.

BACKGROUND: Acute encephalopathy with biphasic seizures and late reduced diffusion (AESD) is the most common encephalopathy syndrome among Japanese children. We report, for the first time, a case of AESD, in which magnetic resonance imaging (MRI) showed no diffusion abnormalities, but hyperperfusion was detected by arterial spin labelling (ASL). CASE REPORT: A previously healthy Japanese 1-year and 5-month-old boy was transferred to our hospital due to a consciousness disorder after >60 min of status epilepticus on the first day of fever. Brain MRI on the first day revealed no abnormal findings. On the fourth day, focal seizures of the left upper and lower limbs were observed. Thereafter, the patient's condition progressed without seizures. Diffusion-weighted imaging (DWI) on day 6 showed no abnormal findings, including a bright tree appearance. However, ASL showed hyperperfusion in the frontoparietal lobes. MRI scans on days 19 and 39 revealed that the hyperperfusion lesions on day 6 had transitioned to hypoperfusion on ASL and displayed high signal intensity on T2-weighted and fluid-attenuated inversion recovery imaging. Cerebral atrophy was also observed. Based on the clinical course and imaging findings during the chronic phase, a diagnosis of AESD was made. CONCLUSION: ASL may be more sensitive than DWI for detecting AESD lesions and should be performed in children with suspected AESD.

A single exposure to brivaracetam or perampanel does not cause cell death in neonatal rats.

Introduction: Exposure to a range of anti-seizure medications (ASMs) during early brain development adversely impacts neurodevelopmental outcomes in both animal models and in clinical studies. Many ASMs, including phenobarbital, phenytoin, valproate (VPA), and benzodiazepines, are associated with acute neurotoxicity (cell death), impaired synaptic development, and long-term behavioral changes following gestational or neonatal exposure in animals. This is mirrored in clinical studies which show lasting neurodevelopmental deficits following early-life or gestational exposure to these drugs. Brivaracetam (BRV) and perampanel (PER) are two newer generation anti-seizure medications and are of interest based on their mechanisms of action (SV2A modulator, AMPA antagonist, respectively), as other drugs with these mechanisms of action do not trigger acute neurotoxicity. Both BRV and PER show anti-seizure efficacy in developing animals, but potential neurotoxicity of these drugs is unexplored. Methods: To address this gap, we treated postnatal day (P)7 Sprague-Dawley rats with BRV (20, 40, 80 mg/kg) and PER (0.1, 0.9, 2.7 mg/kg), and assessed the induction of cell death across a range of vulnerable brain regions 24 h after exposure. Cell death was assessed using pathogreen staining. Results: In each of the regions examined (dorsal striatum, nucleus accumbens, motor cortex, cingulate cortex, lateral thalamus, septum, hippocampus), VPA, which served as a positive control, significantly increased cell death as measured by the numer of pathogreen positive cells. By contrast, neither BRV nor PER increased the number of pathogreen positive cells in any region examined. Discussion: Our results suggest that BRV and PER may have a positive safety profile-at least with respect to acute induction of cell death - and therefore may offer a safer option for the treatment of early life seizures.

Functional seizure semiology and classification in a public and private hospital.

PURPOSE: Our understanding of potential differences in seizure semiology among patients with functional seizures (FS), also known as psychogenic non-epileptic seizures (PNES), across socioeconomic contexts is currently limited. By examining the differences in seizure manifestations between different socioeconomic groups, we aim to enhance the understanding of how socioeconomic factors may influence FS presentation. This study aimed to describe FS semiology in patients from a private and public epilepsy monitoring units (EMUs) in Cape Town, South Africa. METHODS: The study included patients with FS confirmed through video-electroencephalography (video-EEG) and without comorbid epilepsy. For this retrospective case-control study, data on seizure semiology was gathered from digital patient records, beginning with the earliest available record for each hospital. RESULTS: A total of 305 patients from a private hospital and 67 patients from a public hospital were eligible for the study (N = 372). The private hospital tended to report more akinetic and subjective seizure types when compared to the public hospital. Additionally, patients at the public hospital had higher odds of reporting emotional seizure triggers (aOR=2.57, 95% CI [1.03, 6.37]), loss of consciousness or awareness (aOR=2.58, 95% CI [1.07, 6.24]), and rapid post-event recovery (aOR=6.01, 95% CI [2.52, 14.34]). At the same time, they were less likely to report both short (<30 s) (aOR=0.21, 95% CI [0.08, 0.55]) and long (>5 min) seizures (aOR=0.73, 95% CI [0.13, 0.56]), amnesia for the event (aOR=0.19, 95% CI [0.09, 0.43]), ictal aphasia (aOR=0.33, 95% CI [0.14, 0.76]) or falls and drop attacks (aOR=0.43, 95% CI [0.18, 0.996]), when compared to the private hospital patients. CONCLUSION: While the seizure manifestations were largely consistent across the two socioeconomic cohorts of patients with FS, some subtle differences were observed and warrant further investigation.

Locus coeruleus co-activation patterns at rest show higher state persistence in patients with dissociative seizures: A Pilot Study.

OBJECTIVE: Dissociative seizures are paroxysmal disruptions of awareness and behavioral control in the context of affective arousal. Alterations in stress-related endocrine function have been demonstrated, but the timescale of dissociation suggests that the central locus coeruleus (LC) noradrenergic system is likely pivotal. Here, we investigate whether LC activation at rest is associated with altered brain network dynamics. METHODS: A preliminary co-activation pattern (CAP) analysis of resting-state functional magnetic resonance imaging (fMRI) in 14 patients with dissociative seizures and 14 healthy controls was performed by using the LC as a seeding region. The red nucleus served as a control condition. Entry rates, durations, and state transition probabilities of identified CAPs were calculated. Analyses were corrected for demographic, technical, and clinical confounders including depression and anxiety. RESULTS: Three LC-related CAPs were identified, with the dominant two showing inverse activations and deactivations of the default mode network and the attention networks, respectively. Analysis of transition probabilities between and within the three CAPs revealed higher state persistence in patients compared to healthy controls for both CAP2LC (Cohen's d = -0.55; p = 0.01) and CAP3LC (Cohen's d = -0.57; p = 0.01). The control analysis using the red nucleus as a seed yielded similar CAPs, but no significant between-group differences in transition probabilities. SIGNIFICANCE: Higher state persistence of LC-CAPs in patients with dissociative seizures generates the novel hypothesis that arousal-related impairments of network switching might be a candidate neural mechanism of dissociation. PLAIN LANGUAGE SUMMARY: Dissociative seizures often arise during high affective arousal. The locus coeruleus is a brain structure involved in managing such acute arousal states. We investigated whether the activity of the locus coeruleus correlates with activity in other regions of the brain (which we refer to as "brain states"), and whether those brain states were different between patients with dissociative seizures and healthy controls. We found that patients tended to stay in certain locus coeruleus-dependent brain states instead of switching between them. This might be related to the loss of awareness and disruptions of brain functions ("dissociation") that patients experience during seizures.

Glioma tectal pediátrico con hidrocefalia y ventriculomegalia. Dos casos clínicos / Pediatric tectal glioma presented with acute hydrocephalus and ventriculomegaly. Two case reports

Los gliomas tectales representan un subtipo de tumores de bajo grado que se desarrollan en la región tectal, en la parte superior del tronco encefálico. Los síntomas incluyen los causados por el aumento de la presión intracraneal por hidrocefalia obstructiva. Son comunes la cefalea, la visión borrosa o doble, las náuseas y los vómitos. El tratamiento de la hidrocefalia es la ventriculostomía endoscópica del tercer ventrículo o la derivación ventrículo-peritoneal. Los gliomas tectales se diagnostican habitualmente en la infancia, pero son frecuentes también en adultos. En general son benignos y de progresión lenta; es suficiente el seguimiento ambulatorio clínico y radiológico. Se presentan dos pacientes pediátricos con tumores de la placa tectal mesencefálica. Un niño de 11 años y una niña de 15 años concurrieron al Departamento de Emergencias con diferentes síntomas. El niño fue tratado con derivación ventrículo-peritoneal por hidrocefalia aguda.

Tectal gliomas represent a subset of low-grade tumors that arise in the tectal region at the roof of the brainstem. Symptoms of tectal glioma include those caused by increased intracranial pressure due to obstructive hydrocephalus. Headache, blurred vision, double vision, nausea and vomiting are common symptoms. In the treatment, ETV (endoscopic third ventriculostomy) or VP-shunt (ventriculoperitoneal) can be applied to treat hydrocephalus. Tectal gliomas are usually diagnosed in childhood and often occur in adults. They are often benign, slowly progressing lesions; outpatient clinical and radiological followup is sufficient. We present two cases of pediatric patients with mesencephalic tectal plate tumors. An 11-year-old boy and a 15-year-old girl applied to the Emergency Department with different complaints. The 11 year-old-boy was treated with VP-shunt due to acute hydrocephalus.

Role of comorbidities in epilepsy surgery outcomes of older adults.

We lack knowledge about prognostic factors of resective epilepsy surgery (RES) in older adults (≥60 years), especially the role of comorbidities, which are a major consideration in managing the care of people with epilepsy (PWE). We analyzed a single-center cohort of 94 older adults (median age = 63.5 years, 52% females) who underwent RES between 2000 and 2021 with at least 6 months of postsurgical follow-up. Three fourths of the study cohort had lesional magnetic resonance imaging and underwent temporal lobectomy. Fifty-four (57%) PWE remained seizure-free during a median follow-up of 3.5 years. Cox proportional hazard multivariable analysis showed that aura (hazard ratio [HR] = .52, 95% confidence interval [CI] = .27-1.00), single ictal electroencephalographic pattern (HR = .33, 95% CI = .17-.660), and Elixhauser Comorbidity Index (HR = 1.05, 95% CI = 1.00-1.10) were independently associated with seizure recurrence at last follow-up. A sensitivity analysis using the Charlson Combined Score (HR = 1.38, 95% CI = 1.03-1.84, p = .027) confirmed the association of comorbidities with worse seizure outcome. Our findings provide a framework for a better informed discussion about RES prognosis in older adults. More extensive, multicenter cohort studies are needed to validate our findings and reduce hesitancy in pursuing RES in suitable older adults.

Characteristics of motion signal profiles of tonic-clonic, tonic, hyperkinetic, and motor seizures extracted from nocturnal video recordings.

OBJECTIVE: In this study, characteristics of signal profiles formed by motion, oscillation, and sound signals were analyzed to evaluate generalizability and variability in a single patient setting (intra-patient variability) and between patients (inter-patient variability). As a secondary objective, the effect of brivaracetam intervention on signal profiles was explored. METHODS: Patient data included 13 hyperkinetic seizures, 65 tonic seizures, 13 tonic-clonic seizures, and 138 motor seizures from 11 patients. All patients underwent an 8-week monitoring, and after a 3-week baseline, brivaracetam was initiated. Motion, oscillation, and sound features extracted from the video were used to form signal profiles. Variance of signals was calculated, and combined median and quartile visualizations were used to visualize the results. Similarly, the effect of intervention was visualized. RESULTS: Hyperkinetic motion signals showed a rapid increase in motion and sound signals without oscillations and achieved low intra-patient variance. Tonic component created a recognizable peak in motion signal typical for tonic and tonic-clonic seizures. For tonic seizures, inter-patient variance was low. Motor signal profiles were varying, and they did not form a generalizable signal profile. Visually recognizable changes were observed in the signal profiles of two patients. SIGNIFICANCE: Video-based motion signal analysis enabled the extraction of motion features characteristic for different motor seizure types which might be useful in further development of this system. Tonic component formed a recognizable seizure signature in the motion signal. Hyperkinetic and motor seizures may have not only significantly different motion signal amplitude but also overlapping signal profile characteristics which might hamper their automatic differentiation. Motion signals might be useful in the assessment of movement intensity changes to evaluate the treatment effect. Further research is needed to test generalizability and to increase reliability of the results.

Perampanel for the treatment of Asian people with epilepsy: Real-world evidence from the PERMIT extension study.

This post-hoc analysis of the PERMIT Extension study compared the effectiveness and safety/tolerability of perampanel (PER) between Asian and non-Asian participants. Retention rates, adverse events (AEs), seizure frequency, responder rate (≥50% seizure frequency reduction), and seizure freedom rate (no seizures since at least the prior visit) were assessed. Retention was assessed after 3, 6 and 12 months. Effectiveness assessments were evaluated at 3, 6 and 12 months and the last visit by seizure type (total, focal and generalised). PERMIT Extension included 730 Asian and 1662 non-Asian individuals. Significant differences in demographic/baseline characteristics were reported for the Asian versus non-Asian subgroups including higher median age at epilepsy onset, longer median duration of epilepsy, higher mean number of previous and concomitant ASMs and lower mean monthly seizure frequency (total, focal and generalised). Retention rates were similar between the two subgroups at 3 and 12 months, but significantly lower in the Asian versus non-Asian subgroup at 6 months (65.6% vs. 71.8%; p = 0.004). At last visit, seizure freedom rate was significantly higher in the Asian versus non-Asian for total (35.9% vs. 25.4%; p = 0.001) and focal seizures (32.4% vs. 18.9%; p = 0.001) as was responder rate for both total (63.9% vs. 52.3%; p = 0.001) and focal seizures (62.2% vs. 44.7%; p < 0.001). Seizure freedom and responder rates for generalised seizures were similar between the two subgroups at the last visit. Rates of AEs were similar between the two subgroups (Asian, 47.6%; non-Asian, 45.4%). PER was effective and generally well-tolerated in Asian and non-Asian individuals.

Pathomechanism of infantile traumatic brain injury with a biphasic clinical course and late reduced diffusion evaluated by MR spectroscopy.

BACKGROUND: Infantile traumatic brain injury (TBI) with a biphasic clinical course and late reduced diffusion (TBIRD) has recently been reported as a distinct type of TBI in infancy. However, the pathological and prognostic factors of TBIRD remain unknown. We aimed to compare patients with and without TBIRD and evaluate the pathomechanism of TBIRD using magnetic resonance spectroscopy (MRS). METHODS: Ten Japanese patients with TBI were admitted to our hospital and underwent MRS between September 2015 and September 2022 (age range, 3-15 months; median age, 8.5 months). TBIRD was diagnosed in six patients. MRS data were compared among patients with TBIRD, patients without TBIRD, and controls. Neurological prognosis was classified into grades 1 (normal) to 3 (severe). RESULTS: In patients with TBIRD, MRS revealed an increase in the glutamine (Gln) level on days 3-29, which subsequently became close to normal. The degree of Gln elevation in the non-TBIRD group was smaller (117-158 % of controls) than that in the TBIRD group (210-337 %) within 14 days. MRS in the TBIRD group showed decreased N-acetyl aspartate (NAA) concentrations. The degree of NAA decrease was more prominent in grade 3 than in grades 1 and 2. NAA levels in the non-TBIRD group were almost normal. CONCLUSIONS: Patients with TBI and markedly elevated Gln levels on MRS may develop TBIRD. Neuro-excitotoxicity is a possible pathological mechanism of TBIRD. Decreased NAA levels may be useful for predicting the prognosis of patients with TBIRD.