Introduction: Focal epilepsy secondary to mesial temporal sclerosis (MTS) is one of the main causes of refractory epilepsy. It is typically associated with frontotemporal discharges in the electroencephalogram (EEG), a characteristic image in the magnetic resonance scan and a probability of post-operative remission above 70%.
Aims: To identify different patterns of ictal propagation in surface EEG recordings in patients with refractory epilepsy and MTS, and to analyse their relation with the post-operative outcome.
Patients And Methods: We conducted a retrospective review of the medical records of patients with refractory epilepsy secondary to MTS evaluated in the Epilepsy Surgery Programme of the Hospital de Clinicas, Montevideo (n = 30). The propagation of ictal rhythms was analysed in time windows of three seconds, and propagation maps were produced for each seizure.
Results: Six patterns were identified: ipsilateral temporal (type 1; 37%), bilateral frontotemporal with (type 2A; 22%) or without (type 2B; 17%) extension to suprasylvian regions, alternating temporal (type 3; 13%), unilateral suprasylvian (type 4; 7%) and bilateral at onset (type 5; 3%). The type 1 pattern was associated with classic clinical features and a favourable post-operative outcome. The clinical variants were associated with extratemporal EEG propagation. Patients with reflex seizures continued with post-operative seizures. Overall, no unambiguous relation was found between the ictal EEG pattern and the post-operative outcome.
Conclusions: The ictal EEG pattern does not allow for a surgical prognosis in patients with epilepsy secondary to MTS. The history of reflex seizures in these patients may be a red flag suggesting a less favourable surgical outcome.
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http://dx.doi.org/10.33588/rn.6901.2018328 | DOI Listing |
J Neurol
January 2025
IRCCS Stella Maris Foundation, Via Dei Giacinti 2, 56128, Pisa, Italy.
The neuronal ceroid lipofuscinoses (NCLs) are incurable pediatric neurodegenerative diseases characterized by accumulation of lysosomal material and dysregulation of autophagy. Given the promising results of treatment with trehalose, an autophagy inducer, in cell and animal models of NCL, we conducted an open-label, non-placebo-controlled, non-randomized 12-month prospective study in NCL patients receiving oral trehalose (4 g/day). All were treated with a commercially available formulation for 6 months, followed by a 6-month washout.
View Article and Find Full Text PDFChildren (Basel)
December 2024
Department of Pediatrics, National Cheng Kung University Hospital, College of Medicine, National Cheng Kung University, Tainan 704302, Taiwan.
Introduction: Polycythemia is a rare condition that can be either primary or secondary. We report a case of an adolescent with progressive hydronephrosis-induced polycythemia and low erythropoietin levels, along with a thorough literature review.
Report Of A Case: A 17-year-old girl with epilepsy had progressively elevated hemoglobin levels and low erythropoietin levels.
Orphanet J Rare Dis
January 2025
Division of Pediatric Epileptology, Department of Pediatrics I, Medical Faculty of Heidelberg, Heidelberg University, Heidelberg, Germany.
Background: Tuberous sclerosis complex (TSC) is an autosomal dominant genetic disorder affecting multiple organ systems, with a prevalence of 1:6,760-1:13,520 live births in Germany. On the molecular level, TSC is caused by heterozygous loss-of-function variants in either of the genes TSC1 or TSC2, encoding the Tuberin-Hamartin complex, which acts as a critical upstream suppressor of the mammalian target of rapamycin (mTOR), a key signaling pathway controlling cellular growth and metabolism. Despite the therapeutic success of mTOR inhibition in treating TSC-associated manifestations, studies with mTOR inhibitors in children with TSC above two years of age have failed to demonstrate beneficial effects on disease-related neuropsychological deficits.
View Article and Find Full Text PDFEpilepsy Behav
January 2025
Department of Epileptology, National Center Hospital, National Center of Neurology and Psychiatry, Tokyo, Japan.
Objective: This study aimed to evaluate the quality of life (QOL) in children with epilepsy and their caregivers, as well as the caregiver burden, through a secondary analysis of a cross-sectional online survey in Japan.
Methods: Eligible participants were caregivers of children (aged < 18 years) diagnosed with epilepsy. Children's QOL was measured using the daily living subset of the Japanese version of the Quality of Life in Children with Epilepsy (QOLCE-Js52).
J Craniofac Surg
January 2025
Department of Neurosurgery, Renji Hospital, Shanghai Jiao Tong University School of Medicine, Shanghai, China.
Objective: To confirm the incidence of subcutaneous effusion secondary to cerebrospinal fluid leakage after craniotomy, analyze the risk factors for cerebrospinal fluid leakage leading to subcutaneous effusion, summarize the underlying causes of its occurrence and explore the corresponding treatment strategies.
Methods: A retrospective analysis was conducted on 757 patients who underwent craniotomy at our hospital from January to December 2023. The authors documented the sex, age, surgical characteristics, and history of chronic diseases for all patients, including those who developed subcutaneous effusion secondary to cerebrospinal fluid leakage.
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