Journal of epilepsy research最新文献

Anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis is characterised by the antibodies decreasing the NMDAR surface density and synaptic localization by selective antibody-mediated capping and internalization of surface NMDAR. Ketamine is a NMDAR antagonist which can produce dose-dependent, self-limiting side effects such as hypersalivation, hyperreflexia, transient clonus, dizziness, nausea, vomiting, tachycardia, hypertension, and detrusor muscle overactivity. A 22-year-old girl presented with recent onset behavioral change, progressive movement disorder and later lapsed into super refractory status epilepticus (SRSE). She was treated with at least four antiseizure medications, immunomodulation as well as anesthetic drugs with only partial relief in status. Ketamine was added for SRSE and led to a significant worsening of clinical symptoms with abatement after stopping. She finally responded to thiopentone infusion. Ketamine has been found to be beneficial in SRSE in encephalitis including anti-NMDAR encephalitis. This is seemingly counterintuitive given how the action of the drug mimics the pathophysiology of the disease. This report highlights the risk of paroxysmal sympathetic hyperactivity and aggravation of clinical and electrographic features of anti-NMDAR encephalitis with ketamine. Hence, ketamine and similar medications acting on the NMDAR should be used with caution in anti-NMDAR encephalitis.

Background and purpose: Epilepsy is a common indication for medicinal cannabis (MC) prescription in Australia. Despite legal MC products being available for 8 years, some individuals continue to rely on illicit cannabis. Here, we conducted a survey of Australian persons/people with epilepsy (PWE) and caregivers of a PWE to assess whether the current legal framework supports PWE and/or their caregivers to access prescribed MC.

Methods: The cross-sectional survey consisted of five sections examining sociodemographics, medical history, history of MC use, attitudes towards MC, and barriers to accessing MC.

Results: Of the 126 respondents included in these analyses, 102 were PWE (mean age, 40.9±12.3 years) and 24 were caregivers of a PWE (mean age of PWE, 14.1±8.9 years). Among PWE, 27.5% (28/102) had only used illicit MC products, 27.5% (28/102) had transitioned to prescribed MC products, and 16.7% (17/102) used both. Most caregivers 70.8% (17/24) had only accessed prescribed MC products. Most respondents 77.0% (97/126) reported using MC as an adjunct to conventional anti-seizure medications. Caregivers were more likely to administer prescribed high-cannabidiol products to children using oral routes of administration (p<0.001). In contrast, PWE often used inhaled cannabis (p<0.001). Overall, 67.0% (83/124) of respondents reported that MC "improved" or "greatly improved" their epilepsy, irrespective of MC type. The main barrier to accessing prescribed MC was "cost" (69.0%, 87/126), while tetrahydrocannabinol (THC)-related driving restrictions were also a significant concern for PWE.

Conclusions: The current regulatory framework in Australia supports MC access for PWE and their caregivers, primarily through cannabis clinics. However, cost remains a significant concern. The prevalent use of Δ9-THC-containing and inhaled MC products, either illicit or prescribed, highlights the urgent need to further investigate their safety and efficacy in epilepsy.

Brivaracetam, a high-affinity synaptic vesicle 2A (SV2A) ligand and propyl analog of levetiracetam, has been approved as an adjunctive and monotherapy option for focal onset seizures in various age groups. This review synthesizes data from both clinical trials and real-world studies to evaluate brivaracetam's efficacy, safety, and tolerability profile. Notably, brivaracetam's rapid penetration across the blood-brain barrier, selective SV2A binding, and favorable pharmacokinetic properties contribute to its robust seizure control capabilities, setting it apart from other antiseizure medications. Studies have shown that brivaracetam consistently achieves significant seizure frequency reductions and high responder rates, demonstrating strong efficacy and an overall favorable safety profile. Importantly, brivaracetam also demonstrates effectiveness in special populations, including older individuals and patients with post-stroke epilepsy, maintaining good tolerability and favorable outcomes and achieving high rates of seizure freedom. Future research should further investigate brivaracetam's utility in broader patient groups to better understand its long-term safety and expand its therapeutic reach. With its unique pharmacological properties, clinical flexibility, and promising safety profile, brivaracetam stands as a valuable addition to current epilepsy treatment options, addressing several unmet needs in seizure management.

In this case report, we discuss the oldest patient, at 47-year-old, on record to receive anakinra in treatment for febrile infection-related epilepsy syndrome (FIRES). Additionally, our patient was treated with a shorter course compared to that seen in the current literature. FIRES is rarely seen in adults and remains an area of investigation for best treatment practices due to the refractory and often devastating clinical course. Anakinra is a recombinant interleukin-1 receptor antagonist that effectively targets central nervous system inflammation implicated in the pathogenesis of FIRES. The current literature regarding anakinra use in FIRES mostly represents the pediatric population with dose schedules continued into the chronic phase of the disease. There is a dearth of information regarding the response to anakinra in adult FIRES patients as well as the appropriate treatment duration. This patient presented in focal status epilepticus after 1 week of febrile illness. Focal status epilepticus remained refractory despite these of multiple anti-seizure medications, anesthetics, and steroids. An extensive workup yielded no clear underlying etiology to account for his presentation. Anakinra was started 9 days after seizure onset and continued for 2 weeks. After anakinra initiation, sedative medications were fully weaned within 8 days and all epileptiform activity on electroencephalogram resolved within 2 weeks. The patient eventually returned to his prior cognitive baseline and achieved approximately 1 year of seizure freedom. These outcomes support the use of anakinra in treating adults with FIRES. Further studies with a focus on determining the underlying mechanism that accounts for variability in patient response to anakinra are essential. Such studies may aid in the development of ideal dosing and therapy duration of anakinra in FIRES.

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.