András Fogarasi

Periictal heart rate variability analysis suggests long-term postictal autonomic disturbance in epilepsy

Background:  One of the possible pathomechanisms of sudden death in epilepsy (SUDEP) is a postictal dysregulation of autonomic nervous system. We performed a heart rate variability (HRV) analysis of the periictal state to analyze whether a cardiac autonomic disturbance exists after an epileptic seizure.

A detailed semiologic analysis of childhood psychogenic nonepileptic seizures

Purpose:  Psychogenic nonepileptic seizure (PNES) is an important differential diagnostic problem in patients with or without epilepsy. There are many studies that have analyzed PNES in adults; currently, however, there is no systematic assessment of purely childhood PNES semiology. Our study based on a large pediatric video‐electroencephalography (EEG) monitoring (VEM) cohort, provides a detailed analysis of childhood PNES and assesses the usability of the current classification system described in adults.

Hippocampal Sclerosis in Severe Myoclonic Epilepsy in Infancy: A Retrospective MRI Study

Summary:  Purpose: Severe myoclonic epilepsy in infancy (SMEI; Dravets syndrome) is a malignant epilepsy syndrome characterized by early prolonged febrile convulsions (PFCs) with secondary psychomotor delay and a variety of therapy‐resistant seizures. Although the initial symptoms are repeated PFCs, the MRI performed at the onset of disease shows no hippocampal structural abnormalities. We aimed to assess clinical and serial MRI data of patients with SMEI with a special attention to the temporomedial structures. To our knowledge, this is the first systematic MRI study in this disease.

History of simple febrile seizures is associated with hippocampal abnormalities in adults

Background: It is unclear whether the hippocampal abnormality in temporal lobe epilepsy (TLE) is a consequence or the cause of afebrile or febrile seizures (FSs). We investigated whether hippocampal abnormalities are present in healthy adults > 15 years after a simple FS.

Are ictal vocalisations related to the lateralisation of frontal lobe epilepsy

The purpose was to analyse whether non-speech vocalisations in seizures originating in the frontal lobe do have lateralising value. Patients were included who had undergone presurgical evaluation with ictal video-EEG monitoring at the Epilepsy Centre, had had resective epilepsy surgery involving the frontal lobe, and who had remained seizure free>1 year postoperatively. Twenty seven patients aged 1–42 years (mean 18) met the inclusion criteria. Age at epilepsy onset ranged from 1 month to 41 years (mean 7.1 years). All selected patients had a unilateral MRI detected lesion within the frontal lobe. Fifteen patients had right sided, 12 patients had left sided epileptogenic zones. Seizures recorded during EEG-video monitoring were re-evaluated to identify the occurrence of ictal vocalisations. Pure ictal vocalisations were distinguished from ictal sound productions due to motor or vegetative seizure activity (for example, cloni or respiratory sounds). Pure ictal vocalisation occurred in 11 patients of whom nine had a left frontal epileptogenic zone (p<0.01). It is concluded that ictal vocalisation could be an additional lateralising sign in frontal lobe epilepsy. The results suggest that not only speech, but vocalisation at a subverbal level also shows a left hemispheric dominance in humans.

Peri-ictal lateralizing signs in children: Blinded multiobserver study of 100 children ≤12 years

Seizures in 100 children with partial epilepsy, 61% of temporal lobe origin, were assessed by multiple observers for lateralizing signs (LSs). Most LSs achieved very good interobserver agreement and high predictive value. Seventy-five percent of children produced LSs. Lack of LSs occurred more frequently among younger children, while some LSs appeared more frequently at older ages. Many frequent and reliable LSs were independent of age. LSs may help in the presurgical evaluation of infants and young children.

Effectiveness of antiepileptic therapy in patients with PCDH19 mutations

PURPOSE PCDH19 mutations cause epilepsy and mental retardation limited to females (EFMR) or Dravet-like syndromes. Especially in the first years of life, epilepsy is known to be highly pharmacoresistant. The aim of our study was to evaluate the effectiveness of antiepileptic therapy in patients with PCDH19 mutations. METHODS We report a retrospective multicenter study of antiepileptic therapy in 58 female patients with PCDH19 mutations and epilepsy aged 2-27 years (mean age 10.6 years). RESULTS The most effective drugs after 3 months were clobazam and bromide, with a responder rate of 68% and 67%, respectively, where response was defined as seizure reduction of at least 50%. Defining long-term response as the proportion of responders after 12 months of treatment with a given drug in relation to the number of patients treated for at least 3 months, the most effective drugs after 12 months were again bromide and clobazam, with a long-term response of 50% and 43%, respectively. Seventy-four percent of the patients became seizure-free for at least 3 months, 47% for at least one year. SIGNIFICANCE The most effective drugs in patients with PCDH19 mutations were bromide and clobazam. Although epilepsy in PCDH19 mutations is often pharmacoresistant, three quarters of the patients became seizure-free for at least for 3 months and half of them for at least one year. However, assessing the effectiveness of the drugs is difficult because a possible age-dependent spontaneous seizure remission must be considered.

Comparative Evaluation of Concomitant Structural and Functional Neuroimages in Rasmussen's Encephalitis

Background and Purpose. Rasmussens encephalitis (RE) is a rare condition of unknown cause characterized by intractable seizures, progressive hemiparesis, mental impairment, and inflammatory histological findings in the cortex. The primary diagnosis is based on biopsy to confirm the typical clinical, electroencephalography, and brain imaging findings. The main objective of this study was to compare simultaneous structural and functional neuroimages in RE. Methods. Concomitant magnetic resonance imaging and 2‐deoxy‐2‐[18F]‐fluoro‐D‐glucose positron emission tomography data from the authorsseries of 5 children and 8 patients described in the literature were analyzed and compared. Results. Typical early findings of RE include metabolic abnormalities (hypermetabolism and hypometabolism) starting in the frontal or temporal regions or, occasionally, involving the whole hemisphere. Focal abnormalities of cerebral glucose metabolism indicate lesions sooner and depict their extent better than concomitant magnetic resonance images. The major structural abnormality remains unilateral; however, contralateral frontal lobe hypometabolism or crossed cerebellar diaschisis can be a finding of this disease. Basal ganglia involvement and whole hemispheric metabolic abnormality appear typically only after several months of disease onset. Conclusion. Concomitant structural and functional neuroimaging provide possibly complementary information in the early noninvasive workup of RE and may facilitate the early diagnosis of this rare disorder.

Beyond the double banana: Improved recognition of temporal lobe seizures in long-term EEG

Purpose: To investigate whether extending the 10-20 array with 6 electrodes in the inferior temporal chain and constructing computed montages increases the diagnostic value of ictal EEG activity originating in the temporal lobe. In addition, the accuracy of computer-assisted spectral source analysis was investigated. Methods: Forty EEG samples were reviewed by 7 EEG experts in various montages (longitudinal and transversal bipolar, common average, source derivation, source montage, current source density, and reference-free montages) using 2 electrode arrays (10-20 and the extended one). Spectral source analysis used source montage to calculate density spectral array, defining the earliest oscillatory onset. From this, phase maps were calculated for localization. The reference standard was the decision of the multidisciplinary epilepsy surgery team on the seizure onset zone. Clinical performance was compared with the double banana (longitudinal bipolar montage, 10-20 array). Results: Adding the inferior temporal electrode chain, computed montages (reference free, common average, and source derivation), and voltage maps significantly increased the sensitivity. Phase maps had the highest sensitivity and identified ictal activity at earlier time-point than visual inspection. There was no significant difference concerning specificity. Conclusions: The findings advocate for the use of these digital EEG technology–derived analysis methods in clinical practice.

Valproate treatment normalizes EEG functional connectivity in successfully treated idiopathic generalized epilepsy patients

AIM To investigate the effect of chronic VPA treatment of EEG functional connectivity in successfully treated idiopathic generalized epilepsy (IGE) patients. PATIENTS AND METHODS 19-channel waking, resting-state EEG records of 26 IGE patients were analyzed before treatment (IGE) and after the 90th day of treatment (VPA), in seizure-free condition. Three minutes of artifact-free EEG background activity (without epileptiform potentials) was analyzed for each patient in both conditions. A group of 26 age-matched healthy normative control persons (NC) was analyzed in the same way. All the EEG samples were processed to LORETA (Low Resolution Electromagnetic Tomography) to localize multiple distributed sources of EEG activity. Current source density time series were generated for 33 regions of interest (ROI) in each hemisphere for four frequency bands. Pearson correlation coefficients (R) were computed between all ROIs in each hemisphere, for four bands across the investigated samples. R values corresponded to intrahemispheric, cortico-cortical functional EEG connectivity (EEGfC). Group and condition differences were analyzed by statistical parametric network method. MAIN RESULTS p<0.05, corrected for multiple comparisons: (1) The untreated IGE group showed increased EEGfC in the delta and theta bands, and decreased EEGfC in the alpha band (as compared to the NC group); (2) VPA treatment normalized EEGfC in the delta, theta and alpha bands; and (3) degree of normalization depended on frequency band and cortical region. CONCLUSIONS VPA treatment normalizes EEGfC in IGE patients.