Migralepsy, what it is and what it is not.

Migralepsy is a nosographical entity depicting a clinical event whose occurrence seems rather exceptional in view of the comorbidity observed between epilepsy and migraine. Defined more precisely as a migraine aura-triggered epileptic seizure (within the time limit of one hour), it is susceptible to numerous diagnoses by excess, undoubtedly stimulated by the elegance of the term diagnosis coined by Lennox and Lennox in 1960. This review points to the main criticisms, which were given to it, but also to the international recognition brought by the International Classification of Headache Disorders (ICHD 3). In fact, there are undoubtedly clinical cases falling under the strict definition of migralepsy, cases which are rare but relevant for understanding the pathophysiology of the two colliding events: migraine aura and epileptic seizure involving the occipital lobe.

Epilepsy and pregnancy: What should the neurologists do?

Epilepsy is one of the most common chronic disorders affecting women of childbearing age. Unfortunately, many women with epilepsy (WWE) still report not receiving key information about pregnancy. They obviously need information about epilepsy and pregnancy prior to conception with a particular emphasis on effective birth control (i.e. contraception), necessity to plan pregnancy, antiepileptic drugs optimization, and folate supplementation. The risks associated with use of antiepileptic drugs during pregnancy have to be balanced against fetal and maternal risks associated with uncontrolled seizures. This report reviews evidence-based counseling and management strategies concerning maternal and fetal risks associated with seizures, teratogenic risks associated with antiepileptic drug exposure with a special emphasis on developmental and behavioural outcomes of children exposed to intra utero antiepileptic drugs.

Postictal syndrome: The forgotten continent. An overview of the clinical, biochemical and imaging features.

Postictal syndrome (PIS) encompasses the clinical, biological, electroencephalographic (EEG) and magnetic resonance imaging (MRI) signs that follow the termination of a seizure. These signs occur as soon as the epileptic discharge ends, but might remain for a substantially long period of time, making them amenable to clinical observation. As a direct consequence, neurologists and intensivists are more frequently attending patients with PIS than during their seizure. Moreover, careful PIS documentation may help physicians to diagnose epileptic seizure from other non-epileptic disorders. Careful analysis of PIS could also be helpful to better characterize the seizure (seizure subtypes, and to some extent, the localization and/or lateralization of the seizure). This article aims to review the main clinical, biological, EEG and MRI components of PIS, discuss differential diagnoses and propose a general clinical attitude, based on the acronym "WAITTT": W for "Watch", to monitor and investigate PIS in order to provide relevant information on seizure, AIT for "Avoid Inappropriate Treatment", to underscore the risk carrying out unnecessary drug injections and intensive care procedures in the setting of a self-limited symptomatology, and TT for "Take Time", to keep in mind that time remains the clinician's best ally for treating patients with PIS.

[Management of tumoral epilepsy in meningioma surgery: Review of the literature and survey of French national practices]. / Gestion de l'épilepsie tumorale dans la chirurgie des méningiomes : revue de la littérature et enquête sur les pratiques nationales.

Prophylaxis or treatment of tumor-associated seizures is adaily concern in neurosurgical practice but is often guided by the surgeon's habits rather than evidence from clinical trials, which is lacking. The present study reviews the literature on the incidence, clinical aspects and treatment of epilepsy and epileptic seizures in patients undergoing surgery for meningioma. Based on the published data, we then performed a French nationwide survey of neurosurgeons' practices regarding perioperative management of meningioma-related epilepsy and epileptic seizures.

To jerk or not to jerk: A clinical pathophysiology of myoclonus.

Myoclonus is a sudden brief (20-250 ms) contraction (positive myoclonus), or a brief and sudden cessation of tonic muscle (negative myoclonus) inducing a simple jerky movement of body part. Myoclonus could have different origins in almost every part of the nervous system, from the cortex to the peripheral nerve, sharing a large panel of etiologies. It is regarded as the paradigmatic movement disorder causing jerks, although not the sole. This paper aims to depict the clinical and neurophysiological characteristics of myoclonus. It shows how neurophysiological investigations including surface polymyography and methods exploring cortical excitability, namely conventional EEG, EEG - jerk-locked back-averaging, somatosensory evoked potentials and C-reflex studies are required to define the generator of myoclonus in the central nervous system and clearly classify myoclonus as cortical, corticothalamic, subcortical - resulting from lesions or dysfunctions of basal ganglia/reticular system - or spinal. This paper also enlightens other movement disorders that may mimic myoclonus appearances, including psychogenic jerks, simple motor tics, spasms and startle syndromes. Finally, it raises few unresolved questions regarding the propriospinal myoclonus or peripheral myoclonus entities, the role of the cerebellum in myoclonic diseases and the relationship between cortical and epileptic myoclonus.

Chronic vagus nerve stimulation in Crohn's disease: a 6-month follow-up pilot study.

The vagus nerve (VN) is a link between the brain and the gut. The VN is a mixed nerve with anti-inflammatory properties through the activation of the hypothalamic-pituitary-adrenal axis by its afferents and by activating the cholinergic anti-inflammatory pathway through its efferents. We have previously shown that VN stimulation (VNS) improves colitis in rats and that the vagal tone is blunted in Crohn's disease (CD) patients. We thus performed a pilot study of chronic VNS in patients with active CD. Seven patients under VNS were followed up for 6 months with a primary endpoint to induce clinical remission and a secondary endpoint to induce biological (CRP and/or fecal calprotectin) and endoscopic remission and to restore vagal tone (heart rate variability). Vagus nerve stimulation was feasible and well-tolerated in all patients. Among the seven patients, two were removed from the study at 3 months for clinical worsening and five evolved toward clinical, biological, and endoscopic remission with a restored vagal tone. These results provide the first evidence that VNS is feasible and appears as an effective tool in the treatment of active CD.

Long-term EEG in adults: sleep-deprived EEG (SDE), ambulatory EEG (Amb-EEG) and long-term video-EEG recording (LTVER).

Long-term EEG in adults includes three modalities: sleep deprived-EEG lasting 1 to 3 hours, 24 hours ambulatory-EEG and continuous prolonged video-EEG lasting from several hours to several days. The main indications of long-term EEG are: syndromic classification of epilepsy; search for interictal discharges when epilepsy is suspected or for the purpose of therapeutic evaluation; positive diagnosis of paroxysmal clinical events; and pre-surgical evaluation of drug-resistant epilepsy. Sleep deprived-EEG and ambulatory-EEG are indicated to detect interictal discharges in order to validate a syndromic classification of epilepsy when standard EEG is negative. These exams can help in evaluating treatment efficacy, especially when clinical evaluation is difficult. Long-term video EEG is indicated for drug-resistant epilepsy, to analyze electro-clinical correlations in a pre-surgical evaluation context, and to refine a positive diagnosis when paroxysmal clinical events are frequent.

[French guidelines on electroencephalogram]. / Recommandations françaises sur l'électroencéphalogramme.

Electroencephalography allows the functional analysis of electrical brain cortical activity and is the gold standard for analyzing electrophysiological processes involved in epilepsy but also in several other dysfunctions of the central nervous system. Morphological imaging yields complementary data, yet it cannot replace the essential functional analysis tool that is EEG. Furthermore, EEG has the great advantage of being non-invasive, easy to perform and allows control tests when follow-up is necessary, even at the patient's bedside. Faced with the advances in knowledge, techniques and indications, the Société de Neurophysiologie Clinique de Langue Française (SNCLF) and the Ligue Française Contre l'Épilepsie (LFCE) found it necessary to provide an update on EEG recommendations. This article will review the methodology applied to this work, refine the various topics detailed in the following chapters. It will go over the summary of recommendations for each of these chapters and underline proposals for writing an EEG report. Some questions could not be answered by the review of the literature; in those cases, an expert advice was given by the working and reading groups in addition to the guidelines.

[Pseudostatus epilepticus: a severe complication of psychogenic nonepileptic seizures (PNES) in children]. / Pseudostatus epilepticus : une complication grave des crises non épileptiques psychogènes (CNEP) chez l'enfant.

Psychogenic nonepileptic seizures are clinical events that mimic epileptic seizures but are not associated with electroencephalographic discharges. These seizures are seldom reported in children in the literature and could be misinterpreted as generalized tonicoclonic seizures. We report the case of a child, already treated for epilepsy, who presented at 8 years of age with several psychogenic seizures leading to pseudostatus epilepticus. After several hospitalizations, the diagnosis of pseudostatus was established on the basis of clinical semiology, lack of EEG abnormalities during the seizures, and a positive provocation maneuver, which elicited and blocked the manifestations. The clinical spectrum of psychogenic seizures is wide and it is particularly difficult to differentiate psychogenic seizures from epileptic seizures, especially when occurring in children, some of whom are already treated for epilepsy. Well-described clinical features can suggest the diagnosis of psychogenic seizure. It is important and necessary to make the diagnosis as soon as possible in order to rapidly begin appropriate treatment including psychotherapy. In fact, the long-term prognosis in children is better than in the adult population. Associated risk factors, such as anxiety as reported in the present case, have to be sought. Recognizing psychogenic seizures will thus avoid their fixation in the child's personality and the risk of inappropriate and escalating treatments leading to iatrogenic complications.

[French guidelines on the use of repetitive transcranial magnetic stimulation (rTMS): safety and therapeutic indications]. / Recommandations françaises sur l'utilisation de la stimulation magnétique transcrânienne répétitive (rTMS) : règles de sécurité et indications thérapeutiques.

During the past decade, a large amount of work on transcranial magnetic stimulation (TMS) has been performed, including the development of new paradigms of stimulation, the integration of imaging data, and the coupling of TMS techniques with electroencephalography or neuroimaging. These accumulating data being difficult to synthesize, several French scientific societies commissioned a group of experts to conduct a comprehensive review of the literature on TMS. This text contains all the consensual findings of the expert group on the mechanisms of action, safety rules and indications of TMS, including repetitive TMS (rTMS). TMS sessions have been conducted in thousands of healthy subjects or patients with various neurological or psychiatric diseases, allowing a better assessment of risks associated with this technique. The number of reported side effects is extremely low, the most serious complication being the occurrence of seizures. In most reported seizures, the stimulation parameters did not follow the previously published recommendations (Wassermann, 1998) [430] and rTMS was associated to medication that could lower the seizure threshold. Recommendations on the safe use of TMS / rTMS were recently updated (Rossi et al., 2009) [348], establishing new limits for stimulation parameters and fixing the contraindications. The recommendations we propose regarding safety are largely based on this previous report with some modifications. By contrast, the issue of therapeutic indications of rTMS has never been addressed before, the present work being the first attempt of a synthesis and expert consensus on this topic. The use of TMS/rTMS is discussed in the context of chronic pain, movement disorders, stroke, epilepsy, tinnitus and psychiatric disorders. There is already a sufficient level of evidence of published data to retain a therapeutic indication of rTMS in clinical practice (grade A) in chronic neuropathic pain, major depressive episodes, and auditory hallucinations. The number of therapeutic indications of rTMS is expected to increase in coming years, in parallel with the optimisation of stimulation parameters.

Brain tumor epilepsy: a reappraisal and six remaining issues to be debated.

Epilepsy associated with brain tumors presents with specific features deserving medical attention. Although commonly reported in patients with brain tumor, either as revealing mode or as a remote complication, limited knowledge is available regarding their epidemiology, clinical evolution, surgical outcome, physiopathology and treatment, providing only clues for clinical management. Seizures appear even more threatening for patients and caregivers, providing seizures could mean tumor progression and recurrence. This factor adds to the negative impact of epilepsy carried on quality of life measures. Pharmacotherapy is complicated by the use of chemotherapy and interaction between antiepileptic drugs and antineoplastic agents are frequent and potentially harmful. The high incidence of epilepsy enlights the question of prophylaxy with antiepileptic drugs, in patients without seizures, or during the perioperative period, and after surgery, when gross total resection has been achieved. This article attempts to provide the reader with an overview of brain tumor epilepsy in its specific aspects and to comment on some remaining issues.

Influence of hypothermia on the prognostic value of early EEG in full-term neonates with hypoxic ischemic encephalopathy.

OBJECTIVE: To determine the prognostic value of early electroencephalograms (EEG) in full-term neonates suffering from hypoxic ischemic encephalopathy (HIE) exposed to whole-body hypothermia (cooled group), compared to neonates treated conventionally (control group). METHODS: The study included all term neonates born at Grenoble Hospital between 2000 and 2006 with symptoms of HIE. The first two EEGs were reviewed retrospectively and classified according to current electrophysiological criteria. In the cooled group, EEGs were recorded with a mean body temperature of 33°C. Neurological outcome was correlated with EEG pattern. RESULTS: An EEG inactive or paroxysmal pattern was associated with death in 60% of the controls, while all survivors had neurological sequels. In the cooled group, this EEG pattern was only predictive of death in 40% while survivors had normal examination at 1 year of age. Mild abnormalities on the first EEG correlated with a good prognosis in both groups. The second EEG had a high predictive value, particularly in the cooled group. Persistence of inactivity at 3 days after birth was always associated with death. CONCLUSIONS: After HIE, the first two EEGs are good prognostic indicators, also in the cooled group. Strong discontinuity in the EEG observed on the first hours after hypothermia induction can be associated with a good outcome. SIGNIFICANCE: Early Stage 4 EEGs recorded during the hypothermia may not always indicate a poor prognosis in HIE.

Directed differential connectivity graph of interictal epileptiform discharges.

In this paper, we study temporal couplings between interictal events of spatially remote regions in order to localize the leading epileptic regions from intracerebral EEG (iEEG). We aim to assess whether quantitative epileptic graph analysis during interictal period may be helpful to predict the seizure onset zone of ictal iEEG. Using wavelet transform, cross-correlation coefficient, and multiple hypothesis test, we propose a differential connectivity graph (DCG) to represent the connections that change significantly between epileptic and nonepileptic states as defined by the interictal events. Postprocessings based on mutual information and multiobjective optimization are proposed to localize the leading epileptic regions through DCG. The suggested approach is applied on iEEG recordings of five patients suffering from focal epilepsy. Quantitative comparisons of the proposed epileptic regions within ictal onset zones detected by visual inspection and using electrically stimulated seizures, reveal good performance of the present method.

Comparison of five directed graph measures for identification of leading interictal epileptic regions.

Directed graphs (digraphs) derived from interictal periods of intracerebral EEG (iEEG) recordings can be used to estimate the leading interictal epileptic regions for presurgery evaluations. For this purpose, quantification of the emittance contribution of each node to the rest of digraph is important. However, the usual digraph measures are not very well suited for this quantification. Here, we compare the efficiency of recently introduced local information (LI) measure and a new measure called total global efficiency with classical measures like global efficiency, local efficiency and node degree. For evaluation, the estimated leading interictal epileptic regions based on five measures are compared with seizure onset zones obtained by visual inspection of epileptologists for five patients. The comparison revealed the superior performance of the LI measure. We showed efficiency of different digraph measures for the purpose of source and sink node identification.