This retrospective study assessed long-term effectiveness of add-on perampanel (PER) in patients with Lennox-Gastaut syndrome (LGS). Outcomes included time to PER failure and time to seizure relapse in responders. PER failure was defined as either discontinuation of PER or initiation of another treatment. Seizure relapse in responders was defined as occurrence of a seizure in seizure-free patients and increase of at least 50% in average monthly seizure frequency for those who were responders. Eighty-seven patients were included. Treatment failure occurred in 52 (59.8%) subjects at a median time of 12 months. Treatment failure was due to lack of efficacy in 27 (52.0%) patients, lack of tolerability in 14 (27.0%), and both reasons in 11 (21.0%). A slower titration was associated with a lower risk of PER failure compared to faster titration schedules, and the occurrence of adverse events increased the risk of treatment failure. Thirty-six patients (41.4%) were responders during a median follow-up of 11 months. Seizure relapse occurred in 13 of 36 (36.1%) patients after a median time of 21 months. The overall rate of seizure responders was 23 of 87 (26.4%) at the end of follow-up. This study provides real-world evidence on the effectiveness of PER as adjunctive treatment in LGS patients.
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http://dx.doi.org/10.1111/epi.17601 | DOI Listing |
Diseases
December 2024
Department of Pediatrics, Dokkyo Medical University, Tochigi 321-0293, Japan.
Background: Tuberous sclerosis complex (TSC) is an autosomal dominant genetic disorder characterized by mutations in the TSC1 and TSC2 genes, leading to the dysregulation of the mammalian target of rapamycin (mTOR) pathway. This dysregulation results in the development of benign tumors across multiple organ systems and poses significant neurodevelopmental challenges. The clinical manifestations of TSC vary widely and include subependymal giant cell astrocytomas (SEGAs), renal angiomyolipomas (AMLs), facial angiofibromas (FAs), and neuropsychiatric conditions such as autism spectrum disorder (ASD).
View Article and Find Full Text PDFEpilepsy Behav Rep
March 2025
Department of Neurology, Children's Hospital, Zhejiang University School of Medicine, Hangzhou 310052, China.
We presented a 7-year-old boy with refractory Epileptic Encephalopathy with Spike-and-Wave Activation in Sleep (EE-SWAS) successfully managed with a combination of propofol and midazolam. His seizures began at age 2, initially controlled by multiple antiseizure medications (ASMs) for almost three years. At age 5, seizures recurred with electroencephalography (EEG) showing electrical status epilepticus in sleep (ESES) and a spike-wave index (SWI) of 85 %.
View Article and Find Full Text PDFEpilepsy Curr
December 2024
Department of Neurology, Baylor College of Medicine, Houston, TX, USA.
Initiation and maintenance of antiseizure therapy can be relatively straightforward in most patients. Depending on epilepsy type, patients may be more or less likely to enter remission or a resolution of their epilepsy and the International League Against Epilepsy developed clinically guiding definitions in this regard. The mechanisms by which resolution or remission are achieved are poorly understood which complicates clinical decision making and risk estimate for future seizure relapse.
View Article and Find Full Text PDFFront Oncol
December 2024
Department of Neurosurgery, Zibo Central Hospital, Zibo, China.
Meningiomas are some of the most prevalent primary brain tumors in adults, and are typically non-neuroglial in nature. A variety of symptoms may be observed, including headaches, fluctuations in mental status, ataxia, muscle weakness, nausea and vomiting, seizures, visual changes, speech disorders, and sensory abnormalities. The World Health Organization (WHO) has a grading system for meningiomas based on histological criteria, which is as follows: Grade 1 meningiomas are considered benign; Grade 2 meningiomas have a moderately aggressive nature and usually present with histological atypia; and Grade 3 meningiomas exhibit aggressive malignant behavior.
View Article and Find Full Text PDFJ Vet Intern Med
December 2024
Clinical Science and Services, Royal Veterinary College, Hatfield, UK.
Background: Little is known regarding the comorbidities and prognostic factors associated with the long-term outcome of ischemic stroke in dogs. Although poststroke epilepsy is a well-recognized syndrome in people, it is unclear if this phenomenon also occurs in dogs.
Hypothesis/objective: Document comorbidities, long-term outcome (survival and stroke recurrence), and occurrence of epileptic seizures associated with ischemic stroke.
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