ethosuximide / Generic mfg. - LARVOL DELTA

Spike-wave discharges are uncoupled with cognitive outcome in aged Long-Evans rats. (PubMed, Psychopharmacology (Berl)) - "Together these findings provide evidence that the disrupted excitatory/inhibitory balance associated with individual differences in cognitive aging is unrelated to the SWD increase seen in aged rats."

Journal • Preclinical • Alzheimer's Disease • CNS Disorders

Evaluation of ASM with new mechanisms of action in the GAERS model of absence seizure (AES 2025) - "Indeed, SWD in GAERS are inhibited by the anti-seizure medications (ASM) used to treat absence seizures, such as ethosuximide, valproate and lamotrigine. But SWD in GAERS are also aggravated by ASM or other drugs known for worsening absence in patients, e.g. carbamazepine or vigabatrin.Some recent compounds with antiseizure properties and specific mechanisms of action have never been evaluated in the GAERS...Retigabine (4-8 mg/kg PO) produced a significant aggravation of SWD... Evaluation of new mechanisms of action in the GAERS may provide fresh hypothesis for the development of new compounds addressing absence seizures. Our study indicates that targeting potassium channels may be at risk of aggravating SWD, whereas targeting the 5-HT system may produce an interesting effect on absence seizures."

Absence Seizure Disorder • Anesthesia • CNS Disorders • Epilepsy

Epilepsy and Neurodevelopmental Disorders Associated with ASH1L mutations: A Natural History Study of Genotype-Phenotype Correlation (AES 2025) - "Seizures responded to broad spectrum anti-seizure medications (ASMs), mostly with mono- or dual therapy, including Ethosuximide, Valproate, Lamotrigine, Levetiracetam, Clobazam. Our study has demonstrated a higher prevalence and worse phenotypic severity of epilepsy, in female patients with ASH1L mutations, which may implicate sex-specific epigenetic mechanisms of ASH1L in epileptogenesis."

Late-breaking abstract • Neurodevelopmental • Absence Seizure Disorder • ADHD (Impulsive Aggression) • Attention Deficit Hyperactivity Disorder • Autism Spectrum Disorder • CNS Disorders • Developmental Disorders • Epilepsy • Genetic Disorders • Mental Retardation • Psychiatry • Tic Disorders • Tourette Syndrome • ASH1L

Clinical Signature and Longitudinal Seizure Pattern of SLC6A1-Related Neurodevelopmental Disorder (AES 2025) - "The most common ASMs used were valproic acid (59%, 13/22), levetiracetam (59%, 13/22), lamotrigine (50%, 11/22), ethosuximide (45%, 10/22), and clobazam (41%, 9/22). This study represents a large longitudinal investigation of SLC6A1-related epilepsy. The natural history of seizures seen in this cohort demonstrates a unique pattern of seizure onset that contrasts with patterns previously described in STXBP1, SYNGAP1, and SCN8A (McKee et al. 2024, medRxiv)."

Clinical • Neurodevelopmental • Autism Spectrum Disorder • CNS Disorders • Developmental Disorders • Epilepsy • Gastrointestinal Disorder • Genetic Disorders • Mental Retardation • Movement Disorders • Psychiatry • SCN8A • XBP1

From the patient to the mouse: how clinically-validated anti-seizure medications can perform in a pre-clinical mouse model of MTLE. (AES 2025) - "We selected gabapentine and lacosamide, approved in clinic for focal-onset seizures, ethosuximide, approved for absence epilepsy only, and fenfluramine, approved for the treatment of seizures associated with Dravet and Lennox–Gastaut syndromes...The voltage-dependent calcium channel ligand gabapentin (10-300 mg/kg IP) induced a dose-dependent reduction of HPD, accompanied by a significant reduction of the duration of remaining events... Our study better characterizes the translational predictivity of the MTLE mouse model for the development of pharmacological tools addressing focal seizures."

Preclinical • Absence Seizure Disorder • CNS Disorders • Epilepsy

Epilepsy Course And Treatment Response In Individuals With CACNA1A Haploinsufficiency: A Multicenter And Automated Literature-Based Analysis (AES 2025) - "ASMs with broad mechanism of action (valproate, topiramate, zonisamide) resulted in seizure decrease (20/29 use cases, 69%), whereas ethosuximide only led to seizure decrease in 2/7 (28.6%) cases. Epilepsy in LoF CACNA1A variants presents with marked phenotypic variability and predominantly generalized phenotypes with typical and atypical absence seizures that are not responsive to ethosuximide. Earlier seizure onset and higher degree of intellectual disability may be associated with worse seizure control in this population. The presence of EA, though often delayed, along with developmental delay, can prompt considering genetic testing in individuals with history of generalized epilepsy."

Clinical • Absence Seizure Disorder • Ataxia • CNS Disorders • Developmental Disorders • Epilepsy • Mental Retardation • Movement Disorders • CACNA1A

Pseudohypoparathyroidism Spuriously Masking as Juvenile Myoclonic Epilepsy - An Argument for Early Genetic Testing (AES 2025) - "Despite trial of high doses of Keppra, Depakote, Zonisamide, and Ethosuximide, he continued to have frequent daily absence seizures, impairing his ability to attend school. Though this patient met all clinical and electrographic criteria for a diagnosis of JME, his genetic testing revealed he unexpectedly had pseudohypoparathyroidism, a treatable endocrine disorder. Pseudohypoparathyroidism can present with multiple seizure semiologies and generalized interictal discharges on EEG, similarly to JME, however, it is treated very differently. This case illustrates the value and importance of considering early genetic testing in JME patients, especially if medically intractable."

Absence Seizure Disorder • CNS Disorders • Endocrine Disorders • Epilepsy • Metabolic Disorders

EPIPOP: Population Pharmacokinetics of Antiepileptic in Pediatrics (clinicaltrials.gov) - P=N/A | N=753 | Completed | Sponsor: Assistance Publique - Hôpitaux de Paris | Recruiting ➔ Completed

Trial completion • CNS Disorders • Epilepsy • Pediatrics

Oral ethosuximide treatment in a child with short bowel syndrome. (PubMed, Pediatr Int) - No abstract available

Journal • Absence Seizure Disorder • CNS Disorders • Epilepsy • Gastrointestinal Disorder • Short Bowel Syndrome

Ethosuximide ameliorates clinical and pathological phenotypes in a mouse model of SCA42 (Neuroscience 2025) - "Using the knock-in mouse model of SCA42, we demonstrated that ETX suppresses Purkinje cell degeneration and improves ataxic symptoms by modulating the voltage dependence of the variant Cav3.1. These findings suggest that ETX is a promising candidate for disease-modifying therapy in SCA42."

Preclinical • Ataxia • CNS Disorders • Movement Disorders • CAV3

Spatial metabolic profiles of brain sections from mouse models of absence epilepsy (Neuroscience 2025) - "Ethosuximide, the first line treatment for absence epilepsy, fails 50% of patients, thereby motivating the need to identify more effective treatment options...This is achieved using Spatial Augmented Multiomics Interface (SAMI), a computational workflow designed for MALDI-TOF imaging analysis. Overall, our study provides a preliminary spatial map of neuronal metabolic features associated with SWDs and serves as a steppingstone to determine if targeting metabolic processes may serve as a treatment option for patients with absence epilepsy."

Preclinical • Absence Seizure Disorder • CNS Disorders • Epilepsy

Characterization of spike-and-wave discharges modulation by ethosuximide in the GAERS model of absence seizures with extended homecage EEG monitoring (Neuroscience 2025) - "The epileptic activity is notably different from evaluations in forced wakefulness, and it can better match the spontaneous SWDs in humans. The assessment of pharmacological effects over 24 hours can allow a better pharmacodynamic characterization of antiabsence compounds in preclinical studies."

Late-breaking abstract • Absence Seizure Disorder • CNS Disorders • Epilepsy

Case Report: Adenylosuccinate lyase deficiency type I caused by splicing disruption due to a novel missense variant in the ADSL gene. (PubMed, Front Genet) - "Seizures were temporarily controlled with ethosuximide and vigabatrin, though neurodegeneration progressed. This case highlights splicing disruption as a novel pathogenic mechanism in ALD and expands the mutational spectrum associated with the disease. This case also underscores the importance of integrating RNA analysis with genomic data to uncover cryptic splicing defects, especially when protein-level predictions suggest benignity."

Journal • CNS Disorders • Developmental Disorders • Epilepsy • Metabolic Disorders

Site-selective addition of succinimide motif through nitro-assisted C-H functionalization of (hetero) arenes under rhodium catalysis. (PubMed, Arch Pharm Res) - "Succinimide motifs are recognized as privileged cores in anticonvulsants and antipsychotics such as phensuximide, ethosuximide, and lurasidone. The versatility of the developed protocol is demonstrated through nitro-group reduction, reductive cyclization of the synthesized products, and selective modifications of the succinimide framework. Mechanistic studies, including deuterium-labeling and kinetic isotope effect experiments, helped elucidate a plausible reaction mechanism."

Journal • CNS Disorders

Clinical and genetic landscape of epilepsies with absence seizures and single-gene etiology. (PubMed, Epilepsia) - "Absence seizures are a common manifestation of different monogenic epilepsies, often associated with early onset, atypical clinical and/or EEG features, developmental delay or drug resistance. Classification models should incorporate genetic data alongside electroclinical features, especially as next-generation sequencing is increasingly used."

Clinical • Journal • Absence Seizure Disorder • CNS Disorders • Developmental Disorders • Epilepsy • Mental Retardation • CHD2 • SLC2A1 • STARD9

Autosomal Dominant DHDDS-Related Movement Disorder Treated with Primidone (MDS Congress 2025) - "The patient's seizures were well-controlled on carbamazepine currently, but had previously been on ethosuximide and topiramate. We describe a patient with DHDDS heterozygous pathogenic variant and movement phenotype consistent with prior case series reported in the literature.[1][2][3][4] To our knowledge, this is the first report to demonstrate clinical benefit of DHDDS-associated cortical myoclonus with primidone. We suggest that primidone may improve cortical myoclonus through modulation of GABA-related circuitries, as shown in other forms of cortical myoclonus.[5][6][7] Given limited therapies for myoclonus in this patient population, we propose consideration of primidone for symptomatic benefit, though larger studies are needed to validate our case report. [figure1] [figure2]"

Ataxia • CNS Disorders • Epilepsy • Essential Tremor • Mental Retardation • Movement Disorders • Parkinson's Disease • Psychiatry

CLOBAZAM INDUCED ABSENCE STATUS: A CASE REPORT (WCN 2025) - "IGE: Juvenile absence epilepsy in absence status most probably induced by clobazam. . Absence seizures may be aggravated by CBZ, GABAergic medications"

Case report • Clinical • Absence Seizure Disorder • CNS Disorders • Epilepsy

Clinical response to brivaracetam in two cases of drug-resistant juvenile absence epilepsy (PubMed, An Sist Sanit Navar) - "For absence seizures, only valproic acid and ethosuximide are approved by both the U.S. Food and Drug administration and the European Medicines Agency, with lamotrigine additionally approved by the European Medicines Agency. Evidence supporting the use of levetiracetam in this seizure type is limited, and brivaracetam has not yet been approved for generalized epilepsy. We report two cases of juvenile absence epilepsy in women with incomplete response and intolerance to first-line treatments, who demonstrated marked clinical improvement following treatment with brivaracetam."

Journal • Absence Seizure Disorder • CNS Disorders • Epilepsy

Identification of CNKSR2 Pathogenic Variant and Detection of Strong XCI in a Female Patient With Severe DEE-SWAS and Phenotype Expansion in Male Patients. (PubMed, Clin Genet) - "Her clinical symptoms were more severe than those of previously reported female patients, but they improved with ethosuximide and sulthiame treatment. The strength of the X-chromosome inactivation skewing may be related to severity. These novel pathogenic CNKSR2 variants have expanded the phenotypic spectrum of this disease."

Journal • CNS Disorders • Developmental Disorders • Epilepsy • Genetic Disorders • Mental Retardation

Absence seizures and sleep-wake abnormalities in a rat model of GRIN2B neurodevelopmental disorder. (PubMed, Epilepsia) - "Our data show that the new rat Grin2b haploinsufficiency model exhibits clinically relevant phenotypes and highlights two potential therapeutic options for GRIN2B-related epilepsy."

Journal • Preclinical • Absence Seizure Disorder • Autism Spectrum Disorder • CNS Disorders • Developmental Disorders • Epilepsy • Genetic Disorders • Mental Retardation • Psychiatry • GRIN2B

Ictal eructation in a case of idiopathic generalized epilepsy. (PubMed, Epilepsy Behav Rep) - "She was seizure free for many years on ethosuximide, but her absence seizures recurred as her dose was gradually decreased...Several episodes of unsteadiness were noted, at times the patient falling backwards into her bed, but never falling to the ground from a standing position; none of these episodes associated with ictal EEG correlate. In summary, our patient demonstrated and absence seizures with ictal eructation or belching, which has not been reported as an ictal symptom of generalized seizures, and episodes of unsteadiness of unknown characterization."

Journal • Absence Seizure Disorder • CNS Disorders • Epilepsy

Investigate the Clinical Responses of Ethosuximide in Patients With Treatment-Resistant Depression. (clinicaltrials.gov) - P1 | N=16 | Completed | Sponsor: Zhejiang University | Terminated ➔ Completed

Trial completion • CNS Disorders • Depression • Mood Disorders • Psychiatry

Calcium-iron crosstalk in epileptogenesis: Unraveling mechanisms and therapeutic opportunities. (PubMed, Neurobiol Dis) - "Clinically, antiepileptic drugs targeting Ca2+ channels (e.g., ethosuximide, zonisamide) and emerging ferroptosis inhibitors (e.g., deferoxamine, RTA 408) underscore the therapeutic potential of modulating these pathways. Future research must prioritize cross-model validation, advanced neuroimaging biomarkers, and multidisciplinary frameworks to translate mechanistic insights into precision therapies. This comprehensive analysis positions Ca2+-Fe2+ crosstalk as a pivotal frontier in epilepsy research, bridging molecular pathophysiology with innovative treatment paradigms."

Journal • Review • CNS Disorders • Epilepsy • Hematological Disorders • Metabolic Disorders • CAV3 • TRPC6

Case Report: Hematopoietic Stem Cell Transplantation to Treat Severe Acquired Aplastic Anemia in a Pediatric Kidney Transplant Recipient. (PubMed, Pediatr Transplant) - "Despite reported favorable outcomes in children who received allogeneic HSCT after kidney transplantation, there is a lack of evidence on how to overcome numerous challenges in these ultrarare cases."

Journal • Absence Seizure Disorder • Acute Graft versus Host Disease • Anemia • Aplastic Anemia • Atypical Hemolytic Uremic Syndrome • Bone Marrow Transplantation • Cardiovascular • Chronic Kidney Disease • CNS Disorders • Epilepsy • Epstein-Barr Virus Infections • Graft versus Host Disease • Hematological Disorders • Hypertension • Immunology • Infectious Disease • Nephrology • Pediatrics • Pulmonary Arterial Hypertension • Solid Organ Transplantation • Transplant Rejection • Transplantation

Ethosuximide Induced Raynaud's Phenomenon in a Child With Childhood Absence Epilepsy. (PubMed, J Child Neurol) - No abstract available

Journal • Absence Seizure Disorder • Cardiovascular • CNS Disorders • Epilepsy • Rheumatology