Heritability of alpha and sensorimotor network changes in temporal lobe epilepsy.

Objective: Electroencephalography (EEG) features in the alpha band have been shown to differ between people with epilepsy and healthy controls. Here, in a group of patients with mesial temporal lobe epilepsy (mTLE), we seek to confirm these EEG features, and using simultaneous functional magnetic resonance imaging, we investigate whether brain networks related to the alpha rhythm differ between patients and healthy controls. Additionally, we investigate whether alpha abnormalities are found as an inherited endophenotype in asymptomatic relatives.

Drug-responsive versus drug-refractory mesial temporal lobe epilepsy: a single-center prospective outcome study.

: To evaluate clinical, electrophysiological, and neuroradiological factors which correlate with the prognosis in patients with mesial temporal lobe epilepsy (MTLE). : This was a single-center prospective outcome study in patients with MTLE. The patients' family history, clinical characteristics, neurophysiological data (electroencephalography - EEG), neuroimaging, antiepileptic therapy, and outcome were collected and analyzed.

A hemodynamic network involving the insula, the cingulate, and the basal forebrain correlates with EEG synchronization phases of sleep instability.

The cyclic alternating pattern (CAP) encompasses the pseudoperiodic appearance of synchronized brain waves and rhythms and is considered a regulator of the nonrapid eye movement (NREM) sleep vigilance level, reflecting sleep instability. To determine the brain regions responsible for this phenomenon, we scored and analyzed sleep functional magnetic resonance imaging data acquired with simultaneous electroencephalography (EEG-fMRI). Group analysis revealed a set of brain areas showing statistically significant blood oxygen-level dependent signal correlated positively with the synchronization phase of the CAP, most prominent being the insula, the middle cingulate gyrus, and the basal forebrain.

Abnormal temporal lobe morphology in asymptomatic relatives of patients with hippocampal sclerosis: A replication study.

We investigated gray and white matter morphology in patients with mesial temporal lobe epilepsy with hippocampal sclerosis (mTLE+HS) and first-degree asymptomatic relatives of patients with mTLE+HS. Using T1-weighted magnetic resonance imaging (MRI), we sought to replicate previously reported findings of structural surface abnormalities of the anterior temporal lobe in asymptomatic relatives of patients with mTLE+HS in an independent cohort. We performed whole-brain MRI in 19 patients with mTLE+HS, 14 first-degree asymptomatic relatives of mTLE+HS patients, and 32 healthy control participants.

Peripheral neuropathy in diabetes: it's not always what it looks like.

Background: Hereditary Neuropathy with liability to Pressure Palsies (HNPP) is an autosomal dominant neuropathy, associated with deletion of the Peripheral Myelin Protein-22 (PMP-22) gene, causing recurrent painless palsies with age of onset between 10 and 30 years old. Only a few cases of Type 2 Diabetes and HNPP have been described and the coexistence of HNPP and Type 1 diabetes has never been reported.

Case Report: A 54-year old man with a history of Type 1 diabetes, managed with continuous subcutaneous insulin infusion (CSII), presented with deterioration of long-standing motor and sensory symptoms, previously attributed to golfer's elbow, diabetic neuropathy and spinal degenerative disease.

Spatiotemporal propagation patterns of generalized ictal spikes in childhood absence epilepsy.

Objective: This work investigates the spatial distribution in time of generalized ictal spikes in the typical absences of childhood absence epilepsy (CAE).

Methods: We studied twelve children with CAE, who had more than two typical absences during their routine video-EEG. Seizures were identified, and ictal spikes were marked over the maximum electronegative peak, clustered, waveform-averaged and spatiotemporaly analyzed in 2D electrode space.

Forgetting in temporal lobe epilepsy: When does it become accelerated?

The notion of 'accelerated long-term forgetting' has often been attributed to disrupted 'late' memory consolidation. Nevertheless, methodological issues in the literature have left this theory unproven, leading some to suggest such findings may be reflective of subtle acquisition or early retention deficits. This study attempts to address such issues, and also to explore which pathophysiological variables are associated with forgetting rates.

Interictal estimation of intracranial seizure onset in temporal lobe epilepsy.

Objectives: To evaluate the lateralizing and localizing values of interictal focal slow activity (IFSA), single pulse electrical stimulation (SPES) and (18)FDG PET, in order to estimate their potential to complement ictal intracranial recordings and reduce prolonged monitoring in patients with temporal lobe epilepsy.

Methods: The study includes 30 consecutive patients with bilateral temporal subdural electrodes and focal seizure onset. IFSA, SPES and (18)FDG PET when available, were visually assessed and their combined lateralization was based on the majority of the individual lateralizing tests.

Spatiotemporal profiles of focal and generalised spikes in childhood absence epilepsy.

The EEG in childhood absence epilepsy (CAE) may contain focal and generalised spike-wave discharges (SWDs) with focal, mainly frontal, "lead-in". The term "frontal absence" has been used to imply fast, secondary, 3-Hz generalisation from occult frontal foci with potential impact on clinical EEG interpretation and syndrome classification. The aim of this study was to investigate the relationship between focal and generalised SWDs.

Syncope-like epileptic seizures in Panayiotopoulos syndrome.

Objective: To describe the clinical features of syncope-like epileptic seizures (SLES) and their frequency in Panayiotopoulos syndrome (PS).

Methods: This was a 6-year prospective study of all children aged 1-15 years referred for an EEG. PS was defined by the occurrence of at least one autonomic seizure (AS) in a neurodevelopmentally normal child and at least one EEG with focal spikes.

Focal and generalized EEG paroxysms in childhood absence epilepsy: topographic associations and distinctive behaviors during the first cycle of non-REM sleep.

Purpose:   To better understand the nature of the focal spike-wave discharges (FSWDs) and focally led generalized spike-wave discharges (GSWDs) in typical childhood absence epilepsy (CAE) and by implication their nosologic and taxonomic significance.

Methods:   Twenty-four abnormal video-electroencephalography (EEG) studies from 13 consecutive children with CAE and good response to appropriate antiepileptic drugs (AEDs) were analyzed. We studied the association between the topography of absence onset and the ictal automatisms, and the topographic correlation between FSWDs and GSWDs and their respective behavior during hyperventilation and the different states of phasic and nonphasic non-rapid eye movement (NREM) sleep.

Electroencephalographic assessment of patients with epileptic seizures.

This article reviews the role of EEG in the diagnosis and management of patients with epilepsies. We review the morphologic and behavioral characteristics of the interictal and ictal EEG markers of the different types of epilepsy that should guide recording strategies to augment its diagnostic yield, and we attempt to delineate those particular features that may be relevant to the main epilepsy syndromes. Particular emphasis is placed on the activation methods, including hyperventilation, sleep deprivation and sleep, and specific triggers, as well as how these may differ between idiopathic and cryptogenic/symptomatic generalized and focal epilepsies, commenting on possible diagnostic pitfalls and areas of uncertainty.

Multifocal spatiotemporal distribution of interictal spikes in Panayiotopoulos syndrome.

Objective: To investigate the spatiotemporal course of interictal spikes in Panayiotopoulos syndrome (PS), and in particular whether seemingly independent extra-occipital spikes are truly autonomous or secondary, triggered by occipital spikes.

Methods: Seven children with the most representative interictal spike patterns on visual analysis were studied. Five had a single focus (occipital in two, suggestive of posterior to anterior spike propagation in two, and frontal) and two had two foci over the posterior and the anterior areas independently.

Epileptic discharges and phasic sleep phenomena in patients with juvenile myoclonic epilepsy.

Purpose: Epileptiform discharges (EDs) may be part of the internal arousing stimuli that affect the quality of sleep in patients with epilepsies. We studied the association between EDs and sleep phasic phenomena, and its relevance to seizure control in 19 patients with juvenile myoclonic epilepsy (JME).

Methods: We analyzed the first cycle of non-REM (rapid eye movement) sleep in 22 sleep-deprived electroencephalography (EEG) studies and classified EDs within the cyclical alternating pattern (CAP) frame, grouping separately the EDs that occurred at the transition between phases (B to A and A to B).

Fixation-off sensitivity in epilepsies other than the idiopathic epilepsies of childhood with occipital paroxysms: a 12-year clinical-video EEG study.

Purpose: To define the spectrum of the epileptic syndromes and epilepsies (other than the idiopathic epilepsies of childhood with occipital paroxysms) that can be associated with fixation-off sensitivity (FOS), delineate the electrographic types of FOS abnormalities and identify the patterns that can be associated with clinical seizures, and examine whether there may be a pure form of fixation-off sensitive epilepsy.

Methods: We reviewed the clinical and video EEG data of all our patients with FOS over the last 12 years. Children with idiopathic focal epilepsies and occipital EEG paroxysms were excluded.

Stimulus-induced bilateral central periodic discharges, cortical myoclonus and arousal responses in mild reversible coma.

Objective: To study the association between stimulus-induced periodic epileptiform discharges (PEDs), arousal EEG responses and limb jerking in a comatose patient with reversible viral encephalitis.

Methods: We recorded video EEG in Intensive Care Unit (ICU) for strictly clinical purposes. Back averaging was performed off-line using Neuroscan 4.

Benign childhood focal epilepsies: assessment of established and newly recognized syndromes.

A big advance in epileptology has been the recognition of syndromes with distinct aetiology, clinical and EEG features, treatment and prognosis. A prime and common example of this is rolandic epilepsy that is well known by the general paediatricians for over 50 years, thus allowing a precise diagnosis that predicts an excellent prognosis. However, rolandic is not the only benign childhood epileptic syndrome.

Idiopathic epilepsy with generalized tonic-clonic seizures only versus idiopathic epilepsy with phantom absences and generalized tonic-clonic seizures: one or two syndromes?

Purpose: To define the relationship between two syndromes of idiopathic generalized epilepsy (IGE) with apparently similar phenotypes: The form with generalized tonic-clonic seizures only (IGE-GTCS) and that with phantom absences (IGE-PA).

Methods: We compared the electroclinical features of 33 consecutive patients with GTCS and generalized spike wave (GSW); 18 had only GTCS and were diagnosed as IGE-GTCS, and 15 had hitherto unnoticed mild absences on the electroencephalography (EEG) and were diagnosed as IGE-PA. All patients were subjected to the same diagnostic workout, including video EEG during hyperventilation with breath counting (HBC).