Epileptic Disorders最新文献

Objectives: New-onset refractory status epilepticus (NORSE), a subtype of status epilepticus, poses a critical neurological emergency marked by considerable morbidity and mortality. This study aimed to characterize NORSE phenotypically and assess its outcomes, interleukin levels, and miRNA levels.

Methods: Over a 3-year period, patients presenting with NORSE were enrolled. Clinical data were documented, and serum and cerebrospinal fluid (CSF) were collected for inflammatory marker analysis. Results were compared with matched controls, and statistical analyses were conducted.

Results: The study comprised 37 patients (M: F-22: 15), with a median age of 23 (IQR: 14-31) years at presentation. The median hospital stay was 22 days, with an in-hospital mortality rate of 32%, and 51% of patients experienced poor outcomes at discharge. Approximately 62% required anesthetic agents and immunomodulators for seizure control, and 42% of survivors developed epilepsy during a mean 9-month follow-up. Serum and CSF levels of IL-6 and IL-8 were elevated in cases compared to controls, though serum IL-8 levels did not reach statistical significance. Levels of miRNA 132 and miRNA 134 were lower in cases than controls, but statistical analysis was limited by sample size.

Significance: NORSE represents a grave neurological emergency with substantial mortality and morbidity as well as the development of epilepsy during follow-up. The study indicates upregulation of certain interleukins and downregulation of specific miRNA in the serum and CSF of NORSE patients, yet further investigation is warranted to establish correlations with prognosis, treatment response, and outcomes.

We performed a systematic review of the localization value of ictal mimic automatisms-including gelastic, dacrystic, fearful, ritualistic, and kissing semiology-in focal epilepsy. We performed a comprehensive literature search (Medline, EMBASE, Cochrane, Scopus) for patient-level studies, following a PRISMA and QUADAS2 approach. Patients with focal epilepsy displaying mimic automatism, defined as "stereotyped mimicry or behavior that resembles the usual way one expresses oneself to reflect an affect and that is not accompanied by the corresponding emotion" were included. Patients with subjective emotional experiences during a seizure, which the patient is aware of, were excluded. Analysis included 936 patients from 104 studies. Gelastic semiology was primarily associated with hypothalamic hamartomas (HH), and secondarily with frontal (cingulate; superior frontal gyrus), temporal (hippocampus; amygdala; pole; parahippocampal and fusiform gyri), insular, and orbitofrontal involvement, without consistent hemispheric lateralization. Dacrystic semiology was associated with HH, as well as temporal lobe seizure onset (mesial and anterior), followed by the less frequent involvement of orbitofrontal (anterior; baso-lateral), insular, and frontal regions; also without consistent hemispheric lateralization. Fearful ictal semiology was equally associated with frontal (cingulate; superior frontal gyrus), orbitofrontal (mesial; posterior), and temporal (hippocampus; amygdala) involvement; most often, but not exclusively, of right hemispheric lateralization. Ritualistic behavior was associated with the temporal lobe origin (hippocampus; amygdala; pole), with strong right hemispheric lateralization. Kissing ictal behavior was associated with temporal lobe origin (pole; hippocampus; amygdala), and secondarily with the frontal lobe (cingulate); most often, but not exclusively, of right hemispheric lateralization. Our systematic review-derived localization of the various manifestations of mimic automatisms supports anatomo-clinical correlations and helps guide interpretation of ictal semiology within the framework of pre-surgical evaluation in focal epilepsies.

Ideally, pediatric electroencephalograms (EEGs) should be performed by accredited neurophysiology technologists and interpreted by specialists trained in epileptology However, low- and middle-income countries (LMICs) lack such specialists.

Aim: To collate expert consensus on essential curriculum content for non-epilepsy specialists in EEG interpretation and safe post-training practice.

Method: A qualitative study on pediatric EEG training curricula needs was designed in collaboration with an adult education specialist. Data were collected via interviews from 15 epilepsy experts with training experience across high- to low-income settings. Thematic analysis was used to identify sub-themes. The experts voted on the key statements in a two-round Delphi to ascertain consensus.

Results: Twelve aspects of pediatric EEG training were identified and categorized thematically: relevance; exposure to pediatrics; focus on pediatrics; barriers; resource-limited setting; entry skills; best pedagogy; assessment; critical skills; reinforcement of skills; training model; and recommendations.

Conclusion: This study was driven by the inadequate access to training in pediatric EEG for non-epilepsy specialists, which is further exacerbated by the lack of epileptologists and neurophysiologists. The outcomes from the expert consensus opinions promoted consolidation, adaptation, and evolution of existing models that are viable for practice and to be used worldwide. The Delphi consensus demonstrated alignment among regionally located specialists towards the promotion of effective and maintained training for non-epilepsy specialists, as well as highlighting barriers that should be considered and addressed.

Objective: Neonatal seizures have an incidence of 1-5/1000 newborns. Their clinical, etiology, and therapeutic characterization is a clinical challenge. The objective of this study was to characterize neonatal seizures recorded over 6 years, considering the clinical presentation, management, and follow-up.

Methods: A retrospective analysis of clinical, neurophysiological (video-electroencephalogram), and imaging data of all newborns who had suspected neonatal seizures with electroencephalographic findings (paroxysmal activity, electrographic-only seizures, and/or electroclinical seizures) was performed. The study considered all cases that occurred between January 2010 and December 2015, with the respective follow-up equal to or longer than 7 years.

Results: Seizures were diagnosed in 85 newborns, 87.1% within the first 48 h of life. In 72.9% of suspected cases, only paroxysmal activity in the v-EEG was recorded, in another 16.5% electrical-only seizures were found, and in 10.6% there were electroclinical seizures. Epilepsy was diagnosed in 23.5% of cases, with 14% currently maintaining therapy. Motor sequelae occurred in 44.3% of children and 41.4% had normal neurological examination. There were also 11.4% of deaths in the neonatal period.

Significance: Neonatal seizures occur mostly in the first 48 h of life and mainly due to hypoxic-ischemic injuries. During follow-up, we recorded epilepsy in ¼ of patients.

Objective: Ketogenic diet (KD) is an effective treatment for epilepsy that has been widely used. Linear growth restriction has been a concern as a potential adverse effect of the KD, especially in young children and infants. Studies on the classic ketogenic diet (CKD) and its potential negative impact on linear growth have had varying results. Additional studies are needed to assess this concern in other varieties of the KD, such as the modified Atkins diet (MAD). The purpose of our study was to assess the impact that MAD had on growth metrics at various time points, paying particular attention to linear growth.

Methods: This study was a retrospective analysis of the MAD in children and infants with epilepsy. All participants were patients in the Ketogenic Diet clinic at The University of Chicago Comer Children's Hospital. Data were gathered at initial evaluation and again at visits at the 1-, 6-, and 12-month marks.

Results: Twenty-three patients were included in the analysis. Height, weight, and BMI Z-scores were compared from baseline to the 6- and 12-month marks. The analysis of the study showed no statistically significant difference in height Z-score from baseline to 6 months (.12, p = .05) and 12 months (.269, p = .05) after the MAD initiation. When stratified by age group, there was no significant difference in height Z-score from baseline to 12 months, including in the infant age group, age under 3 years, (.07, p = .05). Higher degrees of metabolic acidosis (serum bicarbonate <20 mEq/L), however, showed a statistically significant negative impact on linear growth while this association was not seen with the levels of average ketosis.

Significance: In our study, the MAD does not have a significant negative impact on linear growth in infants and children with epilepsy for at least 12 months of treatment.

Objective: Typical semiology with associated 5As (Antecedent, Aura, Arrest, Automatisms, and Amnesia) is the prototypical seizure manifestation of temporal lobe epilepsy (TLE) in adults. However, patients with TLE can present with seizure phenomenology referred to as "hypermotor TLE" (HMS-TLE) which can mimic extratemporal focal epilepsy. We studied the clinico-electrographic, imaging profile, and surgical outcomes of drug-resistant HMS-TLE patients.

Methods: We analyzed the ictal signs and electroencephalography data of HMS-TLE patients who became seizure free following epilepsy surgery with a minimum follow-up of 1 year and compared them with patients with typical TLE (1:2 ratio). Hypermotor seizures were identified based on the presence of stereotyped ictal complex movements at seizure onset including body rocking, pelvic thrusting, and bimanual and bipedal movements with/without affective components.

Results: Out of 684 patients with drug-resistant TLE who underwent surgery during 2010-2020, 16 patients (2.34%) met criteria for HMS-TLE and were compared with 32 patients with typical TLE. Predominant "hypermotor" components included motor agitation, bicycling/kicking movements, body gyration followed by violent automatisms, and pelvic thrusting. Age at epilepsy onset, age at video telemetry recording, age at surgery, duration of epilepsy at surgery, gender distribution, and presence of aura were comparable between the two groups. History of febrile seizures (31.3% vs. 65.6%; p-value = .024), presence of hippocampal sclerosis on MRI, and histopathology (56.3% vs. 90.6%; p-value = .006 and 50% vs. 96.9%; p-value = .002, respectively) were lower, and temporal neocortical abnormalities on MRI (87.5% vs. 59.4%, p-value = .048), dysplasia on histopathological evaluation (25% vs. 0%, p-value = .003) were higher in HMS-TLE. HMS-TLE group had lower number of patients with focal-onset ictal patterns (37.5% vs. 90.6%, p-value = .0001).

Significance: Hypermotor seizures are rare in TLE, however it does not preclude non-invasive selection for surgery. Hence, physicians should be aware of this subtype of drug-resistant TLE as early intervention can have good surgical outcomes, akin to typical TLE.