Journal of epilepsy research最新文献

Lance Adams syndrome (LAS) is characterized by chronic action or intention myoclonus resulting from cerebral hypoxia. Perampanel, a non-competitive antagonist of aamino-3-hydroxy-5methyl-4 isooxazoleproprionic acid glutamate receptor, has demonstrated some efficacy in myoclonic epilepsy and other types of myoclonus. We report significant benefit in a patient with LAS treated with add on perampanel and provide a review of the relevant literature. In our case, a male patient in his 30s was found pulseless with unknown down time. The patient developed post anoxic myoclonus within 1 week from cardiac arrest. Patient continued to suffer from intractable myoclonus despite being treated with brivaracetam, valproic acid, and clonazepam. Perampanel was added to his medication regimen and up-titrated to 12 mg daily over 1-2 weeks. This resulted in significant improvement in frequency and severity of myoclonus for about 6 months. Growing evidence exists for perampanel as an adjunctive treatment in patients with post hypoxic myoclonus or LAS. A review of the available literature, comprised of case reports and case series, and suggests a potential role for perampanel in patients with LAS. Further study is warranted including controlled trials of perampanel use in post hypoxic myoclonus.

Background and purpose: Epilepsy increases poor outcomes in patients with post-traumatic brain injury and brain tumor-related epilepsy, for whom early seizure control is essential. Perampanel (PER) was a known third-generation antiepileptic drug for treatment all types of seizures. The objective of the study is to compare clinical outcomes and safety of PER administration as monotherapy.

Methods: A prospective study of all 84 patients assigned to PER monotherapy (PER group, n=36) and other first-line antiepileptic drugs (n=48). Clinical outcomes parameters were measured by the prevalence of patients with a diminish in seizure frequency at 50% in 28 days. From November 1, 2020 to April 30, 2024, comparing the PER group with usual care. Clinical outcomes included adherence rate and seizure-free proportion at 28 days and 6 months. Adverse drug reactions were recorded in both groups.

Results: There was no difference in demographic data and incidence of adverse drug reactions between two groups. Median PER dosage was 4 mg (range, 2-12 mg). Compared to other antiepileptic drugs, the PER group had a prevalence of 50% responder rate at 28 days and 6 months significantly were 75%, 81%, 65%, and 51% respectively. Common adverse drug reactions were somnolence and dizziness.

Conclusions: PER administration as monotherapy demonstrated good efficacy and less adverse drug reactions. Low dosages helped to decrease adverse drug reactions and improved retention rate.

Absence status epilepticus may occur in persons diagnosed with idiopathic/genetic epilepsy as well as de novo in adult and elderly patients. Despite being a rare phenomenon, pregnant women with no previous history of epileptic seizures may be presented with new onset status epilepticus. In this report, we describe the case of a 22-year-old pregnant female with no prior history of seizures. The patient was admitted to our center with reduced spontaneous speech and perplexity. Electroencephalography showed continuous, generalized synchronous paroxysms of 3 Hz spike-wave complexes. The patient's clinical condition improved following the administration of diazepam and levetiracetam. To the best of our knowledge, we describe the first case of new onset absence status epilepticus during pregnancy.

Discontinuation of antiseizure medications (ASMs), primarily prompted by adverse effects, presents a formidable challenge in the management of epilepsy, and impacting up to 25% of patients. This article thoroughly explores the clinical spectrum of cutaneous adverse drug reactions (cADRs) associated with commonly prescribed ASMs. Ranging from mild maculopapular rashes to life-threatening conditions such as Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN), the diverse manifestations are meticulously detailed. Diagnostic strategies, incorporating red flags and testing methodologies, are elucidated to ensure precise identification. The classification of adverse drug reactions (ADRs), with a specific focus on cADRs and their association with type A or type B reactions, is presented. Critical risk factors, encompassing patient demographics, drug-related skin reactions, and genetic predispositions, are thoroughly explored. The article underscores the role of human leucocyte antigen (HLA), including HLA*15:02, in predicting susceptibility to severe reactions like SJS/TEN, particularly with aromatic ASMs prevalent in specific populations. Management strategies for varying cADR severities are discussed, placing emphasis on drug discontinuation, symptomatic relief, and potential desensitization. The article concludes by consolidating current knowledge, providing clinicians with a roadmap for navigating the complexities of diagnosis and management. The integration of personalized medicine principles and evidence-based approaches emerges as a crucial paradigm for the future of epilepsy management, aiming to minimize the impact of ADRs on patient outcomes.

Background and purpose: The timeline of alteration of vitamin D and calcium levels in those receiving anti-seizure medication (ASM) remains to be elucidated. To determine the changes in vitamin D levels over a period of 6 months among children receiving monotherapy with commonly used ASM.

Methods: The baseline serum levels of vitamin D, parathyroid hormone (PTH), calcium, alkaline phosphatase (ALP), phosphorus were measured in 32 children (median age 8 years) with newly diagnosed epilepsy. An appropriate ASM monotherapy was started. Those found to be deficient were treated with vitamin D supplementation. Children were reassessed after 90 days and 180 days for drug compliance and drug side-effects. All the baseline investigations were repeated.

Results: At baseline, 21.9% of children were vitamin D-deficient, with a median serum level of 19.8 ng/mL. For children who were not vitamin D-deficient (VDD) at baseline (n=25), the median (interquartile range [IQR]) vitamin D levels were found to be significantly lower than baseline after 90 days of ASM use (23.0 [18.0 to 28.9] vs. 22.0 [12.0 to 24.0]; p<0.001). After 90 days, ASMs caused notable decreases in vitamin D levels from baseline for children who were not VDD at baseline (n=25) (23.0 [18.0 to 28.9] vs. 22.0 [12.0 to 24.0]; p<0.001), alongside changes in calcium, phosphorus, PTH and ALP levels. Similarly, in children who were non-deficient at 90 days follow-up (n=20), median (IQR) vitamin D levels were found to be significantly lower at 180 days than at 90 days (24.5 [21.0 to 28.9] vs. 18.4 [13.6 to 20.6]; p<0.001).

Conclusions: The study noted vitamin D deficiency in children on ASM monotherapy for 3-6 months, emphasizing regular monitoring by clinicians.

Background and purpose: Alzheimer's disease (AD) and epileptic seizure are among the most common health problems in the elderly population. This study aimed to estimate the prevalence rate and predictors of seizures in sporadic AD patients.

Methods: The study was conducted by retrospectively for a period of 10 years examining the file records. Patients were selected among the patients diagnosed with probable sporadic late onset AD according to the National Institute of Neurological Communicative Disorders and Stroke AD and related disorders association criteria and the diagnostic and statistical manual of mental disorders (n=451). In our 213 sporadic AD patients who were followed up regularly and had a follow up examination in the last 6 months, the file records were examined, scanned and questioned for the presence of epileptic seizures.

Results: The prevalence of non provoked generalized tonic clonic seizures in sporadic AD was found to be 6.57% (n=14). Neuroleptic use, presence of diabetes mellitus (DM) and/or treatment, presence of ischemic heart disease (IHD) and/or treatment were found to be 2.99 times, 1.91 times and 3.09 times higher in our patients who had seizures, respectively. When the factors that can affect seizures were examined, the use of neuroleptics and the presence of IHD and/or treatment were found to be statistically significant in terms of the risk of seizure in AD.

Conclusions: The use of neuroleptics, the presence of IHD and DM and/or their medications could facilitate the development of unprovoked generalized tonic clonic seizures in sporadic AD. It is doubtful whether the seizures are primary or secondary generalized.

Background and purpose: The magnetic resonance images (MRIs) ability of lesion detection in epilepsy is crucial for a diagnosis and surgical outcome. Using automated artificial intelligence (AI)-based tools for measuring cortical thickness and brain volume originally developed for dementia, we aimed to identify whether it could lateralize epilepsy with normal MRIs.

Methods: Non-lesional 3-Tesla MRIs of 428 patients diagnosed with focal epilepsy, based on semiology and electroencephalography findings, were analyzed. AI-based segmentation/volumetry software measured the cortical thickness and the hippocampal volume. The laterality index (LI) was calculated.

Results: We classified into temporal lobe epilepsy (TLE, n=294), frontal lobe epilepsy (FLE, n=86), occipital lobe epilepsy (OLE, n=29), and parietal lobe epilepsy (PLE, n=22). Onset age and MRI age were 24.0±16.6 (0-84) and 35.6±14.8 (16-84) years old. In FLE, the LI of frontal thickness was significantly different between the left and right FLE groups, with LIs of the right FLE group being right-shifted and those of the left FLE group being left-shifted, indicating that the lesion side was thinner than the non-lesion side (p=0.01). The discriminable group, which included the patients with left FLE and a LI lower than minus one standard deviation, as well as the patients with right FLE and a LI higher than one standard deviation, showed a longer duration of epilepsy than the non-discriminable group (12.7±9.9 vs. 8.3±7.7 years; p=0.03). Specifically, the LI of individual regions of interest showed that the rostral middle frontal cortex was significantly different in FLE. However, the TLE, PLE, OLE, and LIs were not significantly different.

Conclusions: AI-based brain segmentation software can be helpful to decide the laterality of non-lesional FLE especially with longer duration of disease.

Managing epilepsy in the context of intellectual disability can be complicated as this population is known to have higher rates of drug resistance and sensitivity to side effects of antiseizure medications (ASMs). Perampanel is a novel ASM recently approved as an adjunctive treatment for drug resistant focal seizures. It carries a black-box warning for serious psychiatric and behavioral adverse reactions of aggression, irritability, et cetera. However, psychosis is a seldom reported side effect of perampanel. We herein describe a case of a 15-year-old girl with moderate intellectual disability who presented with refractory seizures managed successfully after using perampanel. Around 2 months later, she developed psychosis and aggression. The patient's history lacked any significant family or personal history of mental illness. Managing psychotic symptoms was difficult in this case; as perampanel was needed for proper seizure control, and both psychosis and seizures were severe and significantly endangering the patient and people around her. Thus, symptoms were addressed by adding a low-dose risperidone, an atypical antipsychotic. This paper highlights the importance of pre-treatment counselling and monitoring for the emergence of psychiatric side effects including the rarely occurring psychosis while using perampanel, particularly in highly sensitive patients, e.g., those with intellectual disability. We also emphasize on the importance of accurate weighing of risks and benefits while managing psychosis as an adverse event to ASMs in the background of drug-resistant epilepsy.

Gyratory seizures (GS) are a rare seizure type characterized by body rotation of ≥180° around its vertical axis. While GS have been documented in various epileptic syndromes, their occurrence in association with hypothalamic hamartomas (HH) has not been reported previously. This case report introduces the first documented instance of GS in a patient with a HH, a non-neoplastic tumor originating from the tuber cinereum. The patient, a 25-year-old female, with a history of recurrent seizures since childhood, initially presented with gelastic seizures, marked by inappropriate laughter, and subsequent evolution of symptoms including right oculocephalic version and gyratory seizures to the right side. Despite multiple antiepileptic medications, seizures persisted. Neuroimaging revealed a HH in the right hypothalamic region. The presence of polydactyly prompted consideration of Pallister Hall syndrome (PHS). PHS is an autosomal dominant condition linked to GLI3 gene mutations. While some features of PHS were absent in this case, the presence of both gelastic and gyratory seizures indicated the hypothalamus as the lesion site, despite inconclusive electroencephalogram findings. This report underscores the novel association of GS with HH and highlights the importance of considering PHS in patients with HH and polydactyly presenting with gelastic and gyratory seizures. Understanding GS in HH may offer insights into broader hypothalamic lesion-related epileptic phenomena.

Background and purpose: Medication errors are common in the inpatient setting. Epilepsy patients who miss doses of their antiseizure medications are at risk for breakthrough seizures and subsequent complications. The purpose of this study was to quantify and characterize anti-seizure medications reconciliation errors on discharge from the epilepsy monitoring unit (EMU).

Methods: Consecutive admissions to an academic medical center EMU were retrospectively reviewed. Medication reconciliation errors on discharge, including drug errors, dosing errors, and dose timing errors, were recorded. Associations between medication errors and clinical and demographic variables were analyzed using binary logistic regression for continuous variables and Fisher exact tests for categorical variables.

Results: One hundred and eleven admissions between January 1, 2021 and December 31, 2021 were identified. Fourteen anti-seizure medication reconciliation errors were recorded during 11 unique admissions (9.9% of admissions). The most common error type was dosing error (10/14 errors; 71.4%). Number of antiseizure medications on admission (p=0.004), total number of medications on admission (p=0.013), number of medication changes during admission (p=0.0007), and length of stay (p=0.0001) were associated with increased likelihood of errors.

Conclusions: Medication reconciliation errors upon discharge from the EMU occur during approximately 10% of admissions. A higher number of preadmission antiseizure medications, higher total number of preadmission medications, higher number of medication changes during admission, and longer length of stay are associated with increased risk of discharge medication reconciliation errors. Careful attention should be paid to patients with these risk factors.