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.

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.

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.

[Astasia-abasia: psychogenic and non-psychogenic causes]. / Astasie-abasie : causes psychogènes et non psychogènes.

Astasia-abasia is defined as the inability to stand and to walk, despite sparing of motor function underlying the required balance and gestures. Initially, astasia-abasia was considered a psychogenic gait disorder, but later on, the description of "high-order" gait disorders mimicking this pure functional deficit led authors to refer to "astasia-abasia" as a pure descriptive term, without a presupposed etiological or anatomical substrate. In this paper, the main clinical characteristics of both psychogenic and non-psychogenic astasia-abasia are presented and discussed.

Anxiety and depression in psychogenic movement disorder and non-epileptic seizures: a prospective comparative study.

OBJECTIVE: This study compared anxiety and depression in patients presenting with psychogenic non-epileptic seizures (PNES) with those suffering from psychogenic movement disorders (PMD) to assess the link between these psychiatric pathologies and neurological symptoms. METHODS: This clinically descriptive, prospective study involved consecutive patients who fulfilled the clinical and video-EEG criteria for PNES and PMD, and who were recruited over an 18-month period. Semi-structured (according to DSM-IV criteria) psychiatric interviews and self-evaluation using the Beck Depression Inventory and Spielberger State-Trait Anxiety Inventory were carried out. Clinical follow-up was conducted 8-12 months after the first evaluation. RESULTS: A total of 17 patients were recruited: nine presented with PNES; and eight had PMD. Both patient groups had similar demographic and clinical data as well as depression and personality disorders. Although not statistically significant, there was a trend towards an increased prevalence of a familial medical history of epilepsy and a higher incidence of anxiety disorders among patients with PNES. CONCLUSION: The data from this prospective study underscore the clinical and psychiatric similarity between PNES and PMD patients. Further studies involving a larger number of subjects should confirm, from a statistical point of view, the differences suggested in the present investigation and, in particular, the greater incidence of anxiety disorders in PNES patients and the presence of an epileptic parent as a risk factor for PNES.

[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.

[Epileptic seizure and migraine visual aura: revisiting migralepsy]. / Aura visuelle migraineuse et crise épileptique: la migralepsie revisitée.

INTRODUCTION: The term of "migralepsy" has been proposed to define migraine-triggered epileptic seizures. Although already reported in the literature for more than fifty years, a number of observations remain debatable because of possible confusion between migraine and epileptic seizure clinical manifestations, including hemifield visual hallucinations, digestive signs and severe headache. OBSERVATION: We report on the case of a young patient suffering from both diseases, in whom a visual aura preceded either migraine attacks or epileptic generalized tonic-clonic seizure. Subtle modification in the primitive visual hallucination, which suddenly contained colored figures and was accompanied by fear before a prolonged loss of contact, suggested a continuum between migraine aura and epileptic seizure in this patient. Brain MRI was normal and EEG showed some sharp waves in the right posterior area. CONCLUSION: The presence of a neurophysiological continuum between migrainous aura and epileptic seizure is supported by this observation of "migralepsy". Recent findings from genetic and epidemiological studies further support this link.

[Basal ganglia deep-brain stimulation for treatment of drug-resistant epilepsy: review and current data]. / La stimulation cérébrale profonde des ganglions de la base comme traitement des épilepsies pharmacorésistantes: revue et données actuelles.

The surgical treatment of intractable epilepsies involving eloquent areas of the cortex is still challenging. Deep-brain stimulation could be an alternative to resective surgery because it can modulate the remote control systems of epilepsy, such as the thalamus and basal ganglia. The surgical experience acquired in the field of movement disorder surgery and the low morbidity of this technic could allow one to apply DBS to intractable epilepsies, such as generalized, motor and bitemporal epilepsies. Here we discuss the main experimental and clinical data reported so far in the literature and taken from our own experience.

Epsilon sarcoglycan mutations and phenotype in French patients with myoclonic syndromes.

BACKGROUND: Myoclonus dystonia syndrome (MDS) is an autosomal dominant movement disorder caused by mutations in the epsilon-sarcoglycan gene (SGCE) on chromosome 7q21. METHODS: We have screened for SGCE mutations in index cases from 76 French patients with myoclonic syndromes, including myoclonus dystonia (M-D), essential myoclonus (E-M), primary myoclonic dystonia, generalised dystonia, dystonia with tremor, and benign hereditary chorea. All coding exons of the SGCE gene were analysed. The DYT1 mutation was also tested. RESULTS: Sixteen index cases had SGCE mutations while one case with primary myoclonic dystonia carried the DYT1 mutation. Thirteen different mutations were found: three nonsense mutations, three missense mutations, three splice site mutations, three deletions, and one insertion. Eleven of the SGCE index cases had M-D and five E-M. No SGCE mutations were detected in patients with other phenotypes. The total number of mutation carriers in the families was 38, six of whom were asymptomatic. Penetrance was complete in paternal transmissions and null in maternal transmissions. MDS patients with SGCE mutation had a significantly earlier onset than the non-carriers. None of the patients had severe psychiatric disorders. CONCLUSION: This large cohort of index patients shows that SGCE mutations are primarily found in patients with M-D and to a lesser extent E-M, but are present in only 30% of these patients combined (M-D and E-M).

Myoclonus and movement disorders.

Myoclonus is a movement disorder characterized by the occurrence of an involuntary abrupt muscle contraction causing a sudden unexpected jerk. Many other movement disorders can present with the same jerky, shock-like appearance. This paper reviews the clinical and neurophysiologic arguments supporting the distinction between true myoclonus and various imitators, including chorea, ballism, tics, dystonia, stereotypy, tremor and restless limbs. To be differentiated from myoclonus, these movement disorders, despite their heterogeneity, are distinctive through the patterned profile of muscle activation, the longer duration of the muscle contraction, the conditions in which they occur, and their suppressibility at will.

[EEG contribution to the diagnosis of baclofen overdose]. / Apport de l' EEG dans le diagnostic d'une intoxication au baclofène.

In a young woman presenting with severe coma, the EEG helped diagnosing baclofen overdose. In this patient, the first EEG showed continuous multifocal pseudoperiodic sharp waves. The diagnosis was confirmed by the plasma dosage providing an 8-fold increase above normal baclofen therapeutic range. Following symptomatic therapy, the patient improved within a few days and the EEG normalised. Few other drugs may be responsible for such EEG changes, namely lithium, cephalosporin, and bismuth. In such cases, EEG contribution to the diagnosis should not be ignored.

[Oxytocin and maternal stress during the post-partum period]. / Ocytocine et stress de la mère au cours de la lactation en post-partum. L'ocytocine est-elle impliquée dans la confiance en soi de la mère allaitante? Revue de la littérature.

Oxytocin is required for lactation by promoting milk expulsion. Oxytocin has also been reported to exert a positive role in social attachment. The postpartum period has been shown to be crucial for maternal behavior initiation, and required self-trust reinforcement. However, this period is also remarkable for the high risk exposure of either psychic or physical stress. A negative impact on young mother is suspected, both in the short, medium or long term, which can even be deleterious for child-mother relationships. During lactation in female rats and sheep, oxytocin production has been proved to decrease stress-induced hormonal changes and later consequences. In human beings, only the first hour after breast-feeding seems to protect against physical or psychic stress. Oxytocin improves the stress-induced response by reducing the ACTH and cortisol secretion thus representing a potential therapeutic pathway in post-partum pathologies such as depression. Thus, this review of recent literature about oxytocin and stress during post-partum period, leads to the assumption that oxytocin, at the moment of installation of breastfeeding, acts not only on the physiological condition, but also on the psychic condition of the mother.

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.