Epileptic Disorders最新文献

Objective: The present study provides normative data for adapted versions of the Rey Auditory Verbal Learning Test (RAVLT) and the Logical Memory subtest from the Wechsler Memory Scales - 3rd edition (WMS-III-LM), involving both recall and recognition procedures after a 2-week delay to assess accelerated long-term forgetting (ALF). The study also aims to achieve a clinical validation of these tests in a group of people with epilepsy (PWE).

Methods: A total of 124 (18-55 years old) healthy participants and 30 PWE undergoing presurgical monitoring for drug-resistant seizures completed these tasks. Associations between memory performance and sociodemographic, neuropsychological function, and testing factors were examined among healthy participants. Memory performance was compared between healthy and PWE groups, with special attention to forgetting rates over 2 weeks as a measure of long-term consolidation.

Results: Contrarily to raw recall and recognition performance, forgetting rates over 2 weeks were not significantly modulated by age or sex. As expected, higher forgetting rates and a greater prevalence of ALF were found among PWE compared with healthy participants on both tests.

Significance: This study offers useful normative data to assess ALF in PWE and provides clinical evidence that our adapted tests can identify long-term consolidation impairments in this population.

Objective: Despite pharmacological advances in epilepsy treatment, one-third of patients remain pharmacoresistant and may require surgery. Despite extensive literature on epilepsy surgery, studies with follow-ups longer than 5 years are rare. Our goal was to analyze the outcomes of patients undergoing epilepsy surgery at our center, with a minimum follow-up of 15 years.

Methods: This was a retrospective study of prospectively collected data. We used the Engel classification to assess seizure freedom, performed univariate descriptive analysis of the variables of interest, and applied appropriate correlation tests for nominal and categorical variables, with statistical significance set at 0.05.

Results: We included 160 patients with a minimum follow-up of 15 years. A total of 105 (70%) patients underwent resective surgeries, the most common being lesionectomy (46.7%), followed by anterior temporal lobectomy with amygdalectomy (21.9%). Among resective surgeries, 73.6% used intraoperative ECOG. Most surgeries were in the temporal lobe (68.8%), and mesial sclerosis was the most frequent etiology (33.8%), followed by long-term epilepsy-associated tumors (LEAT) (25.6%). Seizure freedom at 15 years was achieved by 57.5% of patients, and most of the remaining patients (63.2%) had rare disabling seizures. The majority (65%) discontinued at least one ASM. Temporal surgeries (χ ²(1) = 8.444, p < 0.05), left-sided surgeries (χ ²(1) = 6.436, p = 0.04), mesial sclerosis (χ ²(1) = 50.870, p = 0.024), and the use of intraoperative ECOG (χ ²(1) = 23.235, p < 0.001) were associated with a better prognosis. No differences in outcome were found between the different temporal lobe surgeries (Fisher's exact test value = 0.859, p = 0.659).

Significance: Appropriate referral to a refractory epilepsy center permits a multidisciplinary approach that can result in long-term seizure freedom for most patients undergoing surgery, especially for left-temporal lobe surgeries performed with the aid of intraoperative monitoring techniques.

Objective: The ketogenic diet (KD) is the standard treatment for glucose transporter type 1 deficiency syndrome (GLUT1-DS), typically yielding seizure reduction and cognitive/motor gains. However, a small subset of patients shows limited or no clinical benefit. This phenomenon, referred to as "KD resistance," remains poorly understood and inconsistently defined. We propose a working definition, outline evaluation domains, summarize candidate mechanisms, and indicate management steps.

Methods: Narrative review of published evidence and expert opinion across clinical, biochemical, EEG, and adherence domains.

Results: KD resistance may be considered when all are present: (1) confirmed therapeutic ketosis (serial blood β-hydroxybutyrate ≥2.0-2.5 mmol/L on repeated measurements); (2) adequate dietary adherence verified by dietetic assessment and, when available, validated tools; (3) sufficient trial duration (≥3 months; longer for primarily cognitive/motor goals); and (4) lack of meaningful improvement on symptom-relevant standardized measures. EEG interictal epileptiform discharge burden can be used as an adjunct marker but is not required. KD resistance may involve several dimensions: failure to achieve therapeutic ketosis, lack of symptom improvement despite confirmed ketosis, or challenges with adherence that limit efficacy. Possible contributing factors include genotypic variability in SLC2A1, mitochondrial dysfunction, impaired blood-brain barrier transport, hormonal influences, and epigenetic regulation. We outline a multidomain evaluation framework with suggested metrics, summarize candidate mechanisms (ketone transport/utilization, mitochondrial function, neurotransmission, ion channels/neuromodulators, inflammation/oxidative stress, epigenetic regulation), and indicate when to introduce a KD-compatible anti-seizure medication.

Significance: The proposed definition and framework standardize terminology and reporting, guide decisions for suboptimal responders, and set priorities for multicenter validation.

Objective: Language serves as an indispensable source of information for diagnosing epilepsy, and its computational analysis is increasingly explored. This study assessed - and compared - the diagnostic value of different language model applications in extracting information. The aim is to identify language patterns that may contain useful clinical information that is not overtly considered by the clinician from first-visit documentation to improve the early diagnosis of childhood epilepsy.

Methods: We analyzed 1561 patient letters from the first two seizure clinics. The dataset was divided into training and test sets to evaluate performance and generalizability. We employed an established Naïve Bayes model as a natural language processing technique and a sentence-embedding (large language) model based on the Bidirectional Encoder Representations from Transformers (BERT) architecture. Both models analyzed anamnesis texts as noted by the treating physician only. Within the training sets, we identified predictive features consisting of keywords indicative of 'epilepsy' or 'no epilepsy.' Model outputs were compared to the clinician's final diagnosis (gold standard) after a two-year follow-up period. We computed accuracy, sensitivity, and specificity for both models.

Results: The Naïve Bayes model achieved an accuracy of 0.73 (95% CI: 0.68-0.78), with a sensitivity of 0.79 (95% CI: 0.74-0.85) and a specificity of 0.62 (95% CI: 0.52-0.72). The sentence-embedding model demonstrated comparable performance with an accuracy of 0.74 (95% CI: 0.68-0.79), a sensitivity of 0.74 (95% CI: 0.68-0.80), and a specificity of 0.73 (95% CI: 0.61-0.84).

Significance: Both models demonstrated relatively good performance in diagnosing childhood epilepsy solely based on the first-visit patient anamnesis text. Notably, the more advanced sentence-embedding model showed no improvement over the computationally simpler Naïve Bayes model. This suggests that modeling of anamnesis data does depend on word order for this particular classification task. Further refinement and exploration of language models and computational linguistic approaches are necessary to enhance diagnostic accuracy in clinical practice.

Objective: Myoclonic status epilepticus (MSE) is a critical neurological condition in elderly patients, characterized by near-continuous myoclonus associated with epileptiform EEG abnormalities. Despite its clinical significance, its features, etiologies, and prognoses are still not well understood in this population. To describe the ictal semiology, polygraphic findings, etiology, and prognosis of elderly patients with MSE and to evaluate the correlation between etiology and prognosis, as well as the relationship between MSE duration and clinical outcomes.

Methods: A retrospective study was conducted on patients over 60 years old diagnosed with MSE between 2013 and August 2024. Clinical and electroencephalographic variables were analyzed, and statistical analysis was performed to assess correlations between etiology, MSE duration, and prognosis.

Results: Twenty-seven patients with MSE were included, with a mean age of 77.6 ± 10.5 years, and 51.9% were female. Proximal myoclonus was observed in multiple regions, predominantly in the upper limbs and face. All patients exhibited bilateral frontocentral epileptiform or rhythmic EEG correlates of myoclonus. Etiologically, 9 cases involved hypoxic-ischemic encephalopathy (HIE), 15 were due to toxic-metabolic or infectious causes, and 3 were linked to neurodegenerative processes. Most patients with prolonged MSE (more than 72 h) required ICU admission, and complete recovery was achieved in 66.7% of cases. Mortality was observed in 9 patients, all of whom had refractory, prolonged MSE and were treated in the ICU. Significant correlations were found between etiology and prognosis, and between MSE duration and clinical outcomes (p < .001).

Significance: This study highlights two patterns of MSE in a case series of elderly patients: toxic-metabolic MSE, associated with a favorable prognosis, and HIE-associated MSE, which was refractory and linked to high mortality. These findings emphasize the importance of early etiological identification in guiding treatment and defining prognosis in elderly patients with MSE.

We conducted a systematic review and meta-analysis of the ictal semiology associated with supplementary motor area (SMA) and pre-supplementary motor area (pre-SMA) epilepsy, to summarize current knowledge of related anatomo-clinical correlations in the context of presurgical evaluation. We conducted the review and reported its results according to the Preferred Reporting Items for Systematic Review and Meta-Analysis statement (PRISMA). We searched PubMed and Embase using relevant keywords related to the SMA and pre-SMA localization, seizure semiology, and scalp electroencephalography (EEG) or stereo-EEG. The risk of bias was evaluated using the QUADAS2 score. Twenty articles were included, with extractable data from 37 patients. We analyzed the included studies and extracted data on the presence of 12 different symptoms. We then performed a meta-analysis of the proportion of patients with each symptom. The most frequently reported ictal feature in SMA epilepsy was asymmetric tonic posturing, observed in 47% of cases. Automatisms (25%) and versive seizures (23%) were also common, while loss of consciousness occurred in 19% of patients. Sensory phenomena (11%) and speech arrest/inhibition (10%) were less frequent. Other features, including symmetric tonic posturing, elementary motor signs, hyperkinetic patterns, affective phenomena, grimacing, and negative motor phenomena, were rarely observed. Little evidence is available on the distinct involvement of the pre-SMA. Although asymmetric tonic posturing appears to be the most common feature of SMA epilepsy, it occurs in fewer than 50% of patients and the level of evidence of this association remains low. The same semiological feature may result from the rapid propagation to adjacent or connected regions; hence, semiology should always be interpreted in the context of a multimodal evaluation. Stereo-EEG investigation remains crucial when EEG and imaging are inconclusive or conflicting.

Objective: To identify long-term predictors of seizure outcome after anterior temporal lobectomy (ATL) in a large, homogeneous cohort of patients with drug-resistant temporal lobe epilepsy (TLE) and MRI-defined unilateral hippocampal sclerosis (HS), all operated on by a single neurosurgeon with extended follow-up.

Methods: We retrospectively analyzed 281 consecutive patients with unilateral HS who underwent standardized ATL performed by the same senior neurosurgeon. All patients had at least two years of follow-up (mean 10.8 ± 5.79 years). Clinical history, neuropsychological evaluation, long-term video-EEG monitoring, and 1.5T MRI constituted the preoperative dataset. Twenty-one variables were assessed as potential predictors of seizure outcome. Kaplan-Meier survival curves and univariate log-rank tests identified candidate predictors; variables with p < .10 were entered into a multivariate Cox regression model. Cognitive and quality-of-life outcomes were evaluated using standardized neuropsychological batteries and the ESI-55 questionnaire.

Results: At 10 years postoperatively, 62.6% of patients remained seizure-free (Engel I). Univariate analysis identified seven factors associated with seizure freedom, including history of focal-to-bilateral tonic-clonic seizures, history of status epilepticus, presence of psychogenic non-epileptic seizures, IED predominance or exclusivity in the operated lobe, ictal onset exclusively in the operated lobe, and a preoperative neuropsychological deficit confined to the operated temporal lobe. Multivariate analysis revealed three independent predictors of seizure outcome: history of status epilepticus (HR = 2.11; p = .002), ictal onset confined to the operated temporal lobe (HR = .57; p = .018), and preoperative neuropsychological deficit restricted to the operated temporal lobe (HR = .59; p = .040). Cognitive outcomes were generally stable; left ATL was associated with greater verbal memory decline. Quality-of-life improved significantly at 2-year follow-up (p < .001), with better outcomes among seizure-free patients.

Significance: In this large single-surgeon cohort with one of the longest follow-up durations reported, most patients with unilateral HS achieved durable seizure freedom after ATL. Status epilepticus, consistent ictal localization to the operated temporal lobe, and concordant preoperative neuropsychological deficit emerged as robust long-term predictors. These findings reinforce the value of detailed presurgical evaluation-particularly ictal EEG concordance and neuropsychological lateralization-in optimizing surgical counseling, risk stratification, and patient selection.

Epilepsy is more common in the elderly population, with a higher prevalence of epilepsy-related neurological diseases, such as stroke and traumatic brain injury. Although dementia is also known to be a potential cause of epilepsy in the elderly, estimating its accurate contribution is challenging because of its high prevalence in the general elderly population. To characterize the clinical features of patients with comorbid dementia, we compared the characteristics of these patients with those of elderly patients with post-stroke epilepsy and an unidentified etiology. Of the 494 patients who were first diagnosed with epilepsy after the age of 65 years, 194 were classified as having post-stroke epilepsy, 105 as having comorbid dementia, and 71 as having epilepsy of unidentified etiology. Patients with comorbid dementia were older at seizure onset (p < .001) and at the last treatment (p < .001) than those in the other groups. They were more likely to have depression (p = .04) and were more frequently treated with acetylcholine esterase inhibitors and memantine before the diagnosis of epilepsy (p < .001). In contrast, stroke-related risk factors, such as hypertension (p < .001) and dyslipidemia (p = .01), were more prevalent in patients with post-stroke epilepsy. Considering that stroke itself is a well-recognized risk factor for dementia and epilepsy in the elderly, our study suggests that dementia may be a major comorbidity and a risk factor for late-onset epilepsy.