[Management of convulsive status epilepticus in infants and children]. / Prise en charge d'un état de mal épileptique de l'enfant (nouveau-né exclu).

Convulsive status epilepticus in childhood is a life threatening condition with serious risk of neurological sequelae which constitutes a medical emergency. Clinical and experimental data suggest that prolonged seizures can have immediate and long-term adverse consequences on the immature and developing brain. So the child who presents with a continuous generalized convulsive seizure lasting greater than five minutes should be promptly treated. The outcome is mainly determined by the underlying etiology, age and duration of status epilepticus. In children the mortality from status epilepticus ranges from 3 to 5% and the morbidity is two-fold higher. Mortality and morbidity are highest with status epilepticus associated with central nervous system infections, which is the most important cause of status epilepticus. There are few evidence-based data to guide management decisions for the child with status epilepticus. Immediate goals are stabilization of airways, breathing and circulation and termination of seizures. Benzodiazepines remain the first-line drugs recommended for prompt termination of seizures. As intravenous lorazepam is not available in France, we suggest clonazepam as the best choice for initial therapy. Rectal diazepam or buccal midazolam remain important options. Intravenous phenytoin/fosphenytoin and phenobarbital are the second-line drugs. Phenytoin is being increasingly substituted by fosphenytoin, but pediatric data are scarce and fosphenytoin is not authorized for use in France below five years old. In children, phenytoin is often preferred to phenobarbital, even though no comparative studies have demonstrated a better efficacy. To manage status epilepticus refractory to a benzodiazepine and administration of phenytoin and/or phenobarbital, many pediatricians today prefer high-dose midazolam infusion rather than thiopental to minimize serious side effects from barbiturate anesthesia. There is no benefit/risk ratio to support the use of propofol for children with refractory status epilepticus.

Severe attacks of familial hemiplegic migraine, childhood epilepsy and ATP1A2 mutation.

We studied four members of a family suffering from typical attacks of familial hemiplegic migraine (FHM) caused by a new mutation, R548C, of ATP1A2 gene in exon 12. One individual had also childhood absence epilepsy and generalized tonic-clonic seizures (GTCS). GTCS were followed by a severe attack of hemiplegic migraine at four times. Sodium valproate enabled control of both the epileptic seizures and the most severe FHM attacks. This association of FHM and epileptic seizures and their control with the same treatment suggest similar pathophysiological mechanisms.

Clinical features of primary headache in children: a multicentre hospital-based study in France.

The aim of this study was to evaluate the concordance between clinical diagnosis and the International Classification of Headache Disorders, 2nd edn (ICHD-II) in children and adolescents with primary headaches. This 6-month prospective multicentre study of 486 patients (mean 9.8 +/- 3.1 years; 52.6% girls) assessed the headache features through a structured questionnaire. In 398 patients with a single type of headache, headaches were bilateral (78.1%), frontal (62.4%), pulsatile (56.1%), with associated symptoms in 84.4%. The most frequently assigned diagnoses were migraine without aura (50.8%), probable migraine (14.1%), migraine with aura (11.1%) and frequent episodic tension-type headache (7.5%). For most of the diagnostic categories, the consistency of the investigator's diagnosis with the ICHD-II criteria was good (kappa > 0.6 and < or = 0.8) or excellent (kappa > 0.8). We conclude that migraine was predominant with regard to headache diagnoses repartition and that the ICHD-II seems usable in practice for evaluation of primary headache in French children and adolescents.

Usefulness of multidetector-row computerized tomographic angiography for the surgical planning in stereoelectroencephalography.

PURPOSE: Surgical planning of depth electrode implantation in stereo-electro-encephalography (SEEG) routinely uses magnetic resonance imaging (MRI) alone. Accurate visualization of arteries and veins in the vicinity of the electrode is essential to plan a safe trajectory to presumably reduce the risk of intracranial bleeding. The goal of this study was to compare multidetector row computerized tomographic angiography (MDCTA) with MRI for the visualization of vessels along each planned trajectory in patients who undergo SEEG. MATERIALS AND METHODS: Ten consecutive patients who were scheduled to undergo SEEG procedure were included. T1-weighted gadolinium-chelate enhanced MR sequence, stereotactic MDCT and MDCTA were performed after fixation of Leksell's frame. For each of the 106 planned stereotactic trajectories, the number of vessels in a 4.0mm diameter circle around the trajectory from the dura mater to the target that were visible on MDCTA were compared to that of visible vessels in the same areas on MRI. RESULTS: Ten vessels (10/106; 9.4%) were seen on MRI and 66 (66/106; 62.3%) on MDCTA (P<0.0001). All vessels visible on MRI were visible on MDCTA. The difference in number of visible vessels between the two techniques remained significant for the different lobes (i.e., frontal lobe, temporal lobe and parieto-occipital lobe). CONCLUSION: MDCTA enabled visualization of more vessels than MRI based SEEG. MDCTA may help neurosurgeons better define the trajectory of the electrode and reduce the risk of intracranial bleeding.

Continuous EEG monitoring in adults in the intensive care unit (ICU).

Continuous EEG monitoring in the ICU is different from planned EEG due to the rather urgent nature of the indications, explaining the fact that recording is started in certain cases by the clinical team in charge of the patient's care. Close collaboration between neurophysiology teams and intensive care teams is essential. Continuous EEG monitoring can be facilitated by quantified analysis systems. This kind of analysis is based on certain signal characteristics, such as amplitude or frequency content, but raw EEG data should always be interpreted if possible, since artefacts can sometimes impair quantified EEG analysis. It is preferable to work within a tele-EEG network, so that the neurophysiologist has the possibility to give an interpretation on call. Continuous EEG monitoring is thus useful in the diagnosis of non-convulsive epileptic seizures or purely electrical discharges and in the monitoring of status epilepticus when consciousness disorders persist after initial treatment. A number of other indications are currently under evaluation.

Vagal nerve stimulation in tuberous sclerosis complex patients.

This is an open-label, retrospective, multicenter study to determine the outcome of intermittent stimulation of the left vagal nerve in children with tuberous sclerosis complex and medically refractory epilepsy. The records of all children treated with vagal nerve stimulation were reviewed in five pediatric epilepsy centers to locate those with tuberous sclerosis complex who had been treated with vagal nerve stimulation for at least 6 months. These patients were compared with (1) a series of patients obtained from the literature, (2) 10 similar control patients with epilepsy obtained from a registry of patients receiving vagal nerve stimulation, and (3) four published series of tuberous sclerosis complex patients whose epilepsy was surgically managed. Ten tuberous sclerosis complex patients with medically refractory epilepsy treated with vagal nerve stimulation were found. Nine experienced at least a 50% reduction in seizure frequency, and half had a 90% or greater reduction in seizure frequency. No adverse events were encountered. Comparison with published and registry patients revealed improved seizure control in the tuberous sclerosis complex patients. Comparison with the group undergoing seizure surgery demonstrated improved outcomes after surgery. Vagal nerve stimulation appears to be an effective and well-tolerated adjunctive therapy in patients with tuberous sclerosis complex and seizures refractory to medical therapy. Resective surgery has a better prospect for improved seizure control.

[Multifocal epileptic crises following mumps]. / Crises épileptiques multifocales post-ourliennes.

A 15 years-old girl with no previous history of epilepsy or neurological disease presented three types of epileptic symptoms the same day: 1) clusters of rhythmic myoclonus of the left hemiface; 2) episodes of painful paresthesias of the left arm followed by secondary generalised seizures; 3) episodes of elementary visual hallucinations of the right hemifield. She had several seizures each hour and some were recorded. There were no EEG abnormalities during the facial myoclonus but rapid rhythms were seen during the sensory and visual partial seizures on the right parietal and left occipital lobes. There was no fever and no drowsiness. The CSF tap showed pleocytosis. Serological studies indicated recent mumps. The drugs were initially inefficient. The seizures disappeared after a month. The drugs were stopped after three months and the seizures had not relapsed after a one year's follow-up. Though there were no other sign of encephalitis, we believe that episode of multifocal seizures was due to mumps encephalitis.

[Contribution of the EEG and evoked potentials in the prognosis of post-anoxic comas in children]. / Apport de l'EEG et des potentiels évoqués dans le pronostic des comas postanoxiques de l'enfant.

Six children with post-anoxic coma were studied and there was evidence of preserved brainstem function together with the presence of electrocerebral activity on EEG. There is no definite prognosis with slow EEG; patients can either die, survive, but with major neurological damage, or recover completely. In that case, evoked potentials have a good prognostic value. Normal BAEP indicate that patients will survive. However, absence of the cortical component of SEP indicates major cerebral damage. Evolution towards a vegetative state can therefore be predicted.

[Generalized or focal photosensitive epilepsies]. / Les épilepsies photosensibles généralisées ou focales.

Photosensitivity is defined by a pattern of occipital or more diffuse spikes and waves. Several techniques are needed for exploration: intermittent light stimulation (ILS), patterns, TV-screen, video games. Photosensitivity is a genetic characteristic. Only diffuse spikes and waves induced by ILS are correlated with epilepsy. Pure photogenic epilepsy is characterized by seizures induced by visual stimuli alone, usually by TV-screen. Video games may also have a triggering effect due to the slow-moving patterns or intense brightness. Several epileptic syndromes are associated with photosensitivity with or without visually-induced seizures. These syndromes are most often generalized and idiopathic.

[Non-edematous spontaneously resolving cerebellar softening. Diagnostic possibilities and value (author's transl)]. / Ramollissement cérébelleux non oedémateux, spontanément curable. Possibilités et intérêt de son dépistage.

Non edematous cerebellar softenings are usually unrecognized even though their relatively high frequency of occurrence is proved by systematic anatomical studies. They should be suspected when an acute late-onset vestibular syndrome is noted, in which there is a particular type of homolateral disequilibrium and nystagmus, good vestibular reactivity, and possible association with a cerebellar syndrome. Softening can usually be confirmed after several days by the complementary examinations presently available: computer tomography of the posterior fossa and scintigraphy with labelled bleomycin. Non-edematous forms are spontaneously resolving disorders but surveillance during the first few days is necessary together with anti-edematous therapy as early signs are common to those of edematous softening, responsible for rapid involvement of the brain stem and blocking of fluid pathways.

[Congenital oculomotor apraxia. 2 cases with an electro-oculography study]. / Apraxie oculomotrice congénitale. Deux cas étude électro-oculographique.

Congenital oculomotor apraxia is characterized by the inability of the young child to produce horizontal saccades when his head is fixed; two cases are reported. In a free condition this trouble is compensated by thrust head movements allowing the child to lead his gaze to the stimulus or to reach it by successive hypometric jerks released by movement. A blink usually precedes the eye movement probably allowing the child to escape from an excessive fixation. This apraxia is combined with a motor and psychological development delay. The evolution of all the troubles is usually good with minimal sequelaes (difficulty for reading). Without any anatomical argument, it may be assumed that a dysfunction of area 8 and of the fronto-mesencephalic pathway is responsible for this abnormality. The resemblance of this disturbance to an earlier phylogenic period of the oculomotor development (inferior mammalian) and its spontaneous good evolution evoke more a maturation delay of the supranuclear control system of saccades than a true disease.

[EEG recordings during episodes of palinacousis and palinopsia]. / Enregistrements EEG contemporains d'épisodes de palinacousie et de palinopsie.

Palinopsia (visual perseveration) and palinacousis (auditory perseveration) are reported in a 51-year-old woman with a left temporo parietal astrocytoma. EEG showed a left temporal delta-focus with sharp waves. EEG was not modified during neither palinacousis nor palinopsia. The relationship between the two hallucinatory phenomena and epilepsy is discussed.

[Photosensitive epilepsy and television epilepsy]. / Epilepsies photosensibles et épilepsie de la télévision.

Photosensitivity is defined by the appearance of occipital or more diffuse electroencephalographic spikes and waves induced by intermittent light stimulation (ILS), particular patterns, TV-watching, and video games. Photosensitivity is a genetic characteristic. Only the diffuse spikes and waves induced by ILS are correlated with epilepsy. Pure photogenic epilepsy is characterized by seizures which are only visually induced, usually by watching TV. Video games sometimes add a trigger effect due to slowly moving patterns or intense brightness. Several epileptic syndromes are associated with a photosensitivity with or without visually-induced seizures, mainly generalized idiopathic epilepsy.

[Localized neonatal convulsions and cerebral arterial infarction]. / Convulsions néonatales focalisées et infarctus artériel cérébral.

BACKGROUND: Stroke is a rare cause of neonatal seizures. CASE REPORTS: During a 5-year period, eight full-term infants were admitted to hospital for seizures due to a stroke. Seizures began shortly after birth and were always one-sided. Early CT scans showed cerebral infarctions. Motor disabilities such as hemiparesis were found in three out of seven cases; language difficulties were observed in the same proportion; however all the children had not reached school age. CONCLUSION: Neonatal localized seizures may be symptomatic of a stroke and therefore justify a computerized tomography (CT) scan. Motor and cognitive sequelae require early management.

Large-field repetitive transcranial magnetic stimulation with circular coil in the treatment of functional neurological symptoms.

OBJECTIVE: Patients with functional neurological symptoms (FNS) are frequently encountered by neurologists and are difficult to treat. Symptoms are multiple and may appear concurrently or successively in the same patient. To date, few studies have been published on focal repetitive transcranial magnetic stimulation (rTMS) in FNS. This type of stimulation induces a focal current, vertically in the cortex. Results are contradictory, probably because it is difficult to identify a limited cortical area that triggers these symptoms. We assessed the efficacy of another type of rTMS: large-field stimulation by means of a circular coil covering a surface area approximately 20 times greater and inducing a circular current tangentially to the cortex. PUBLISHED STUDIES: We analysed two studies on the efficacy of large-field rTMS in functional paralysis and in functional movement disorders. The efficacy of large-field rTMS was very marked in these two studies. PERSONAL NON-PUBLISHED STUDIES: We reported several open series, including patients with functional sensory loss, functional visual loss, and non-epileptic seizures. METHOD: For all patients, one or several sessions of 60 stimuli with circular coil were carried out with a protocol depending on the symptoms. RESULTS: The efficacy of large-field rTMS was dramatic in all patient series. Additionally, we discuss the possible involved mechanism: placebo effect, cognitive behavioural effect or neuromodulatory effect. CONCLUSION: According to the data from these different studies, large-field rTMS could be a new therapy for patients with FNS. However, controlled studies are mandatory.

[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.

Migraine aura lasting 1-24 h in children: a sequence of EEG slow-wave abnormalities vs. vascular events.

The aim of this study was to describe the abnormalities associated with migraine aura lasting 1-24 h in children as shown by EEG, trancranial Doppler (TCD) and single photon emission computed tomography (SPECT). In this retrospective study, 11 patients each underwent EEG, TCD and brain SPECT on the day of admission and the day thereafter. On the day of admission, the migrainous hemisphere of all patients showed that the mean velocities were decreased in the middle cerebral artery by TCD, slow-wave abnormalities were recorded after several hours of aura by EEG and the SPECT showed hypoperfusion. On the day after, in the same hemisphere, slow waves were recorded only in the occipital area by EEG, and SPECT showed slight hyperperfusion. In these patients, there was a clear sequence of EEG, TCD and SPECT abnormalities.