Epilepsia最新文献

Objective: Previous retrospective studies have reported vigabatrin-associated brain abnormalities on magnetic resonance imaging (VABAM), although clinical impact is unknown. We evaluated the association between vigabatrin and predefined brain magnetic resonance imaging (MRI) changes in a large homogenous tuberous sclerosis complex (TSC) cohort and assessed to what extent VABAM-related symptoms were reported in TSC infants.

Methods: The Dutch TSC Registry and the EPISTOP cohort provided retrospective and prospective data from 80 TSC patients treated with vigabatrin (VGB) before the age of 2 years and 23 TSC patients without VGB. Twenty-nine age-matched non-TSC epilepsy patients not receiving VGB were included as controls. VABAM, specified as T2/fluid-attenuated inversion recovery hyperintensity or diffusion restriction in predefined brain areas, were examined on brain MRI before, during, and after VGB, and once in the controls (at approximately age 2 years). Additionally, the presence of VABAM accompanying symptoms was evaluated.

Results: Prevalence of VABAM in VGB-treated TSC patients was 35.5%. VABAM-like abnormalities were observed in 13.5% of all patients without VGB. VGB was significantly associated with VABAM (risk ratio [RR] = 3.57, 95% confidence interval [CI] = 1.43-6.39), whereas TSC and refractory epilepsy were not. In all 13 VGB-treated patients with VABAM for whom posttreatment MRIs were available, VABAM entirely resolved after VGB discontinuation. The prevalence of symptoms was 11.7% in patients with VABAM or VABAM-like MRI abnormalities and 4.3% in those without, implicating no significant association (RR = 2.76, 95% CI = .68-8.77).

Significance: VABAM are common in VGB-treated TSC infants; however, VABAM-like abnormalities also occurred in children without either VGB or TSC. The cause of these MRI changes is unknown. Possible contributing factors are abnormal myelination, underlying etiology, recurrent seizures, and other antiseizure medication. Furthermore, the presence of VABAM (or VABAM-like abnormalities) did not appear to be associated with clinical symptoms. This study confirms that the well-known antiseizure effects of VGB outweigh the risk of VABAM and related symptoms.

Objective: Intracranial single-pulse electrical stimulation (SPES) can elicit cortico-cortical evoked potentials. Their investigation with intracranial EEG is biased by the limited number and selected location of electrodes, which could be circumvented by simultaneous non-invasive whole-scalp recording. This study aimed at investigating the ability of magnetoencephalography (MEG) to characterize cortico-cortical evoked fields (CCEFs) and effective connectivity between the epileptogenic zone (EZ) and non-epileptogenic zone (i.e., non-involved [NIZ]).

Methods: A total of 301 SPES trains (at 0.9 Hz during 120 s) were performed in 10 patients with refractory focal epilepsy. MEG signals were denoised, epoched, averaged, and decomposed using independent component analysis. Significant response deflections and significant source generators were detected. Peak latency/amplitude were compared between each different cortical/subcortical structure of the NIZ containing more than five SPES, and then between the EZ and corresponding brain structures in the NIZ.

Results: MEG detected and localized polymorphic/polyphasic CCEFs, including one to eight significant consecutive deflections. The latency and amplitude of CCEFs within the NIZ differed significantly depending on the stimulated brain structure. Compared with the corresponding NIZ, SPES within the extratemporal EZ demonstrated delayed CCEF latency, whereas SPES within the temporal EZ showed decreased CCEF amplitude. SPES within the EZ elicited a significantly higher rate of CCEFs within the stimulated lobe compared with those within the NIZ.

Significance: This study reveals polymorphic CCEFs with complex spatiotemporal dynamics both within the NIZ and EZ. It highlights significant differences in effective connectivity of the epileptogenic network. These cortico-cortical evoked responses could thus contribute to increasing the yield of intracranial recordings.

Objective: Magnetic resonance imaging (MRI) is a crucial tool for identifying brain abnormalities in a wide range of neurological disorders. In focal epilepsy, MRI is used to identify structural cerebral abnormalities. For covert lesions, machine learning and artificial intelligence (AI) algorithms may improve lesion detection if abnormalities are not evident on visual inspection. The success of this approach depends on the volume and quality of training data.

Methods: Herein, we release an open-source data set of pre-processed MRI scans from 442 individuals with drug-refractory focal epilepsy who had neurosurgical resections and detailed demographic information. We also share scans from 100 healthy controls acquired on the same scanners. The MRI scan data include the preoperative three-dimensional (3D) T1 and, where available, 3D fluid-attenuated inversion recovery (FLAIR), as well as a manually inspected complete surface reconstruction and volumetric parcellations. Demographic information includes age, sex, age a onset of epilepsy, location of surgery, histopathology of resected specimen, occurrence and frequency of focal seizures with and without impairment of awareness, focal to bilateral tonic-clonic seizures, number of anti-seizure medications (ASMs) at time of surgery, and a total of 1764 patient years of post-surgical followup. Crucially, we also include resection masks delineated from post-surgical imaging.

Results: To demonstrate the veracity of our data, we successfully replicated previous studies showing long-term outcomes of seizure freedom in the range of ~50%. Our imaging data replicate findings of group-level atrophy in patients compared to controls. Resection locations in the cohort were predominantly in the temporal and frontal lobes.

Significance: We envisage that our data set, shared openly with the community, will catalyze the development and application of computational methods in clinical neurology.

Up to 80% of the world's population with epilepsy lives in low and middle-income countries. Around one-third of these patients will have drug-resistant epilepsy, for which epilepsy surgery is an option. Unfortunately, many of these regions, as well as some more developed nations, lack sufficient epilepsy surgery units and trained neurosurgeons. With this in mind, the International League Against Epilepsy (ILAE) formed the Epilepsy Surgery Education Taskforce to address the shortage of further educational opportunities for surgeons and neurologists and to promote the creation of more epilepsy surgery units around the world. In this article, we publish our findings from a web-based international survey, in which we investigated the global distribution and experience of neurosurgeons who perform epilepsy surgery, their educational paths, and opinions on the further need for epilepsy surgery education, as well as the resources available to them. We report a detailed analysis of the 202 survey replies received from 35 different countries across six continents. The lack of adequate numbers of epilepsy surgery units in the Southern Hemisphere is notable, and the aim of this task force with other ILAE committees, is to improve access to epilepsy surgery for patients and to enhance training for their health care providers.

Objective: Area tempestas, a functionally defined region in the anterior piriform cortex, was identified as a crucial ictogenic trigger zone in the rat brain in the 1980s. However, whether the primate piriform cortex can trigger seizures remains unknown. Here, in a nonhuman primate model, we aimed to localize a similar trigger zone in the piriform cortex and, subsequently, evaluated the ability of focal inhibition of the substantia nigra pars reticulata (SNpr) to suppress the evoked seizures.

Methods: Focal microinjection of the γ-aminobutyric acid type A (GABA_A) antagonist bicuculline methiodide into the piriform cortex was performed, in macaque monkeys, on a within-subject basis to map the ictogenic regions within this area. Glutamate antagonists were used to characterize the local circuit pharmacology. Focal inhibition of the substantia nigra by infusion of the GABA_A agonist muscimol suppressed seizures evoked from piriform cortex.

Results: We documented a well-defined region highly susceptible to bicuculline-induced seizures in the piriform cortex, just posterior to the junction of the frontal and temporal lobes, indicating that a functional homolog to the rodent area tempestas is present in the primate brain. Focal infusion of glutamate receptor antagonists into the area tempestas revealed that α-amino-3-hydroxy-5-methyl-4-isoxazolepropionic acid receptor-mediated, but not N-methyl-D-aspartate-mediated, neurotransmission was necessary for the expression of seizures. Pharmacological inhibition of the SNpr robustly suppressed area tempestas-evoked seizures.

Significance: Together, these data point to the area tempestas as a potent ictogenic zone in the primate brain and underscore the antiseizure effects of inhibition of the SNpr. Building on decades of studies in rodents, our present findings emphasize the relevance of these targets to the primate brain and provide further rationale for exploring these targets for clinical use.

Objective: Epilepsy surgery in the operculoinsular cortex is challenging due to the difficult delineation of the epileptogenic zone and the high risk of postoperative deficits.

Methods: Pre- and postsurgical data from 30 pediatric patients who underwent operculoinsular cortex surgery at the Motol Epilepsy Center Prague from 2010 to 2022 were analyzed.

Results: Focal cortical dysplasia (FCD; n = 15, 50%) was the predominant cause of epilepsy, followed by epilepsy-associated tumors (n = 5, 17%) and tuberous sclerosis complex (n = 2, 7%). In eight patients where FCD was the most likely etiology, the histology was negative. Seven patients (23%) displayed normal magnetic resonance imaging results. Seizures exhibited diverse semiology and propagation patterns (frontal, perisylvian, and temporal). The ictal and interictal electroencephalographic (EEG) findings were mostly extensive. Multimodal imaging and advanced postprocessing were frequently used. Stereo-EEG was used for localizing the epileptogenic zone and eloquent cortex in 23 patients (77%). Oblique electrodes were used as guides for better neurosurgeon orientation. The epileptogenic zone was in the dominant hemisphere in 16 patients. At the 2-year follow-up, 22 patients (73%) were completely seizure-free, and eight (27%) experienced a seizure frequency reduction of >50% (International League Against Epilepsy class 3 and 4). Fourteen patients (47%) underwent antiseizure medication tapering; treatment was completely withdrawn in two (7%). Nineteen patients (63%) remained seizure-free following the definitive outcome assessment (median = 6 years 5 months, range = 2 years to 13 years 5 months postsurgery). Six patients (20%) experienced corona radiata or basal ganglia ischemia; four (13%) improved to mild and one (3%) to moderate hemiparesis. Two patients (7%) operated on in the anterior insula along with frontotemporal resection experienced major complications: pontine ischemia and postoperative brain edema.

Significance: Epilepsy surgery in the operculoinsular cortex can lead to excellent patient outcomes. A comprehensive diagnostic approach is crucial for surgical success. Rehabilitation brings a great chance for significant recovery of postoperative deficits.

Objective: White matter abnormalities in patients with temporal lobe epilepsy (TLE) and hippocampal sclerosis (HS) are well known. Peak width of skeletonized mean diffusivity (PSMD) is a novel marker for quantifying white matter integrity that may reflect small vessel disease. In this study, we aimed to quantify the extent of white matter damage in patients with TLE and HS by using PSMD.

Methods: We enrolled 52 patients with TLE with HS and 54 age- and sex-matched healthy controls. Diffusion tensor imaging (DTI) was performed using a 3-T magnetic resonance imaging scanner. We measured PSMD using DTI findings and compared PSMD between patients with TLE with HS and healthy controls. We also evaluated the correlation between PSMD and clinical factors in patients with TLE and HS.

Results: PSMD differed significantly between healthy controls and patients with TLE and HS, and it was higher in the patients (2.375 × 10^-4 mm²/s vs. 2.108 × 10^-4 mm²/s, p < .001). Furthermore, PSMD in the ipsilateral hemisphere of the HS was higher than in the contralateral hemisphere of the HS (2.472 × 10^-4 mm²/s vs. 2.258 × 10^-4 mm²/s, p = .040). PSMD was positively correlated with age (r = .512, p < .001) and age at seizure onset (r = .423, p = .002) in patients with TLE and HS.

Significance: Patients with TLE and HS had higher PSMD values than healthy controls, and PSMD was positively correlated with age. These findings provide evidence of white matter damage probably due to small vessel disease in patients with TLE and HS and support the feasibility of PSMD as a promising imaging marker for epileptic disorders.

Joseph Conrad's epilepsy is well documented but has received little attention as he had convulsive seizures only in childhood and adolescence. The type of epilepsy has never been discussed. His biography reveals that his condition was decidedly neuropsychiatric with depression, a suicidal attempt, and prominent signs of frontal lobe dysfunction, as is seen typically in juvenile myoclonic epilepsy. This diagnosis is supported by a congruent family history and probable lifelong myoclonic seizures including reflex myocloni that were misunderstood as nervosity. It is impressive to see how he disciplined himself to become a great writer against the odds of neuropsychological impairment.