New onset refractory status epilepticus: State of the art.

NORSE (new onset refractory status epilepticus) has recently been defined as a clinical presentation, not a specific diagnosis, in a patient without active epilepsy or other preexisting relevant neurological disorder, with new onset of refractory status epilepticus without a clear acute or active structural, toxic or metabolic cause. It includes the concept of FIRES described in children with a similar condition but preceded by a 2-14-day febrile illness. NORSE constitutes the acute phase of an entity preceded by a prodromal phase which may be accompanied by numerous manifestations (febrile episode, behavioural changes, headache, …), and followed by a chronic phase marked by long-term neurological sequelae, cognitive impairment, epilepsy and functional disability. There are many causes of NORSE: autoimmune, infectious, genetic, toxic, … but in half of the cases, despite an exhaustive assessment, the cause remains undetermined. Paraneoplastic and non-paraneoplastic autoimmune encephalitis remains by far the leading cause of NORSE. For these reasons, immunotherapy should be considered rapidly in parallel with the treatment of the status epilepticus, including in cryptogenic NORSE. Good communication with the family is important because the management of the acute phase is long and difficult. Although mortality remains high (11-22%), and sequelae can be severe, the majority of survivors can have a good or fair outcome.

Experiences of self-conscious emotions in temporal lobe epilepsy.

Self-conscious emotions (SCEs) with a negative valence (such as shame and guilt) or a positive valence (such as pride) are moral emotions that emerge from self-reflection and self-evaluation processes in social contexts. In some neurologic and psychiatric disorders, experiences of SCEs are dysregulated. The objectives of the present study were to (i) evaluate whether patients with temporal lobe epilepsy (TLE) experience SCEs in the same way as nonclinical (control) participants and (ii) probe the relationships between experiences of SCEs on the one hand and the psychological symptoms frequently diagnosed in patients with TLE (anxiety and depression), the patients' clinical characteristics, and their functional outcomes in everyday life on the other. Sixty-one patients with TLE and 61 matched controls completed a self-questionnaire (the Positive and Negative Affect Schedule (PANAS)) that enabled us to evaluate the extent to which they experienced shame, guilt, and pride. Demographic data, cognitive data, the severity of anxiety symptoms, and the severity of depressive symptoms were recorded for all participants. In patients with TLE, data of clinical characteristics and quality of life were also evaluated. Relative to controls, patients with TLE were more likely to experience negative-valence SCEs to a higher extent and positive SCEs to a lesser extent. The patients who experienced negative-valence SCEs to a higher extent (rather than to a lesser extent) had a higher frequency of seizures, more severe anxiety and depressive symptoms, and a greater prevalence of anxiety and depressive disorders. Furthermore, patients who experienced positive-valence SCEs to a lesser extent (rather than to a higher extent) displayed a higher level of anxiety. Lastly, differences in experiences of SCEs by patients with TLE were associated with a lower quality of life. In conclusion, experiences of SCEs can be dysregulated in patients with TLE. This dysregulation is linked to the patients' clinical and psychological symptoms and quality of life. In this context, SCEs might be a target of interest in the management of epilepsy.

Is long-term electroencephalogram more appropriate than standard electroencephalogram in the elderly?

OBJECTIVE: To compare the diagnostic accuracy of standard (st) and long-term video (lt) EEG in elderly patients with suspected non-convulsive seizures. METHODS: Over a 12-month period, we prospectively included all elderly (over-65) hospitalized patients having undergone lt-EEG for suspected non-convulsive seizures (n=43). st-EEG was defined as the first 20min of each lt-EEG. We recorded the patients' clinical and imaging characteristics and final diagnosis and assessed the respective diagnostic values of st-EEG and lt-EEG. RESULTS: Epileptiform discharges were detected on standard EEG in only 7% of patients and in 28% of patients on Lt-EEG (p=0.004). Non-convulsive seizures were recorded in 1 case vs. 4, respectively. Nine of 40 negative standard EEG showed later epileptiform activities. The median time to occurrence of the first epileptiform activities was 46.5min (interquartile range: 36.5-239.75min). Epileptiform activity occurred during sleep only in 33% patients with a negative st-EEG. Dementia was associated with a positive lt-EEG (p:0.047). CONCLUSION: Lt-EEG was clearly superior to standard EEG for detecting epileptiform activity in elderly when suspecting non convulsive seizures. SIGNIFICANCE: St-EEG has a low diagnostic yield in elderly patients with suspected non-convulsive seizures and so lt-EEG is preferable in this situation.

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.

EEG guidelines in the diagnosis of brain death.

In France, for the determination and diagnostic validation of brain death the law requires either two EEG recordings separated by a 4-hour observation period, both showing electrocerebral inactivity; or cerebral angiography examination. Since EEG is available in most hospitals and clinics, it is often used in this indication, at the patient's bedside, especially in the context of organ donation. However, very precise methodology must be followed. The last French guidelines date back to 1989, before the development of digital EEG recording. We present the new guidelines from the Société de Neurophysiologie Clinique de Langue Française. Electrocerebral inactivity may be confirmed when a 30-minute good quality EEG recording shows complete electrocerebral silence, defined as no cerebral activity greater than 2 uV, having first ruled out the possible influence of sedative drugs, metabolic disorders or hypothermia. In the presence of sedative drugs, CT brain angiography will be the gold standard test for this diagnosis. In the newborn, the utmost caution is indicated since electrocerebral inactivity can be observed in the absence of cerebral death. In the infant, the criterion for the observation period to be respected between both EEG recordings needs to be more clearly refined.

[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.

Fractal features for localization of temporal lobe epileptic foci using SPECT imaging.

Single photon emission computed tomography (SPECT) is an accurate imaging method for the diagnosis of refractory partial epilepsy. Two scans are carried out: interictal and ictal. The interest of this method is to provide an image in the ictal period, which allows hyperperfused areas linked to the seizure to be localized. The epileptic foci localization is improved by subtracting the two acquisitions (subtracted ictal SPECT: SIS). In some cases, the SIS method is not effective and does not isolate the seizure foci. In this article, we investigate a new method based on texture analysis using fractal geometry features. Fractal geometry features were extracted from each scan in order to quantify the heterogeneity change resulting from the hyperperfusion. A support vector machine (SVM) classification algorithm was used to classify the voxels into two classes: focal and healthy. Quantitative evaluation was performed on simulated images and clinical images from 22 patients with temporal lobe epilepsy. Results on both experiments showed that the proposed method is more specific and more sensitive than the SIS method.

Oscillatory cortical activity related to voluntary muscle relaxation: influence of normal aging.

OBJECTIVE: In this study we aimed to investigate if there are age-related differences in cortical oscillatory activity induced by self-paced muscular pure relaxation in comparison with muscle contraction as reference movement. METHODS: Event-related (de)synchronization (ERD/ERS) have been recorded related to voluntary muscle contraction and relaxation in 10 young and 10 elderly right-handed healthy subjects. The muscle relaxation task consisted in a voluntary relaxation of maintained wrist extension without any overt, associated muscle contraction. The muscle contraction task corresponded to a self-initiated brief wrist extension. RESULTS: In elderly subjects compared to young ones, mu and beta ERD preceding muscular relaxation was more widespread, beginning significantly earlier over contralateral frontocentral and parietocentral regions (p<0.05) as well as over ipsilateral regions (p<0.05). The beta synchronization was significantly attenuated (p<0.05). CONCLUSIONS: These results suggest an alteration of inhibitory motor systems and an altered post-movement somesthetic inputs processing with normal aging. These alterations were accompanied by compensatory mechanisms. SIGNIFICANCE: These age-related alterations during different phases of muscle relaxation could participate to explain global sensorimotor slowing observed with normal aging.

Predominance of the contralateral movement-related activity in the subthalamo-cortical loop.

OBJECTIVE: Abnormal low- and high-frequency oscillatory activities have been linked to abnormal movement control in Parkinson's disease. We aimed to study how low- and high-frequency oscillatory activities are modulated by movement in the contralateral and ipsilateral subcorticocortical loops. METHODS: We studied mu, beta and gamma rhythm event-related desynchronisation (ERD) and synchronisation (ERS) recorded from electrode contacts in the subthalamic nucleus (STN) areas and over the primary sensorimotor (PSM) cortex. RESULTS: Mu and beta ERD/ERS patterns were very similar when comparing PSM cortex and STN areas and very different when comparing contralateral and ipsilateral structures. Beta rhythm ERS was more predominant over contralateral structures than over ipsilateral ones. Gamma rhythm ERS was only recorded from the contralateral STN area (particularly following administration of L-Dopa). For all patients, the best bipolar derivations - as defined by the earliest mu and beta ERD and the strongest beta and gamma ERS - always included the STN electrode contacts that produced the best clinical results. CONCLUSIONS: Movement-related activity is involved in the movement preparation in the contralateral subthalamo-cortical loop and in the movement execution in the bilateral subthalamo-cortical loops. SIGNIFICANCE: Contralateral beta rhythm ERD seemed to be related to bradykinesia of the limb performing the movement.

[The role of the EEG in epilepsy management]. / Place de l'EEG dans l'épilepsie.

Diagnosis of epilepsy is based upon clinical data. But the electroencephalogram (EEG) remains absolutely necessary for proper management of epileptic patients by the neurologist both in consultation and in hospital practice. Nevertheless, it is very important to keep in mind the actions limit of EEG, especially its sensitivity and specificity. In this paper, we will emphasize the value of EEG in epilepsy, its interest for the management of patients in consultation, the situations when the neurologist can use EEG-monitoring, and what it can bring to the emergency management of epileptic patients in hospital. We will thus see that, in each of these situations, EEG must be interpreted with full knowledge of clinical data.

[Management of patients after a first seizure]. / Conduite á tenir devant une première crise convulsive.

Neuroimaging evaluation in patients after a first seizure could be easily determined on the basis of seizure history, neurological examination, blood sample analysis and electroencephalography. The main objectives of the initial work-up are to differentiate a true seizure event from seizure-like symptoms, to exclude a single seizure as a manifestation of non organic cause and finally to consider the seizure as a result of cerebral lesion or inaugurate epilepsy. When a new onset seizure is diagnosed, urgent neuroimaging is recommended only in patients with focal neurological deficit, persistent or worsening alteration in the level of consciousness and when clinical and biological data lead to a suspected vascular or infectious etiology. Brain CT scan is usually more available in emergency to identify the cause of seizure. It may have an important role for the therapeutic strategy and may defer MRI investigation. Nevertheless, brain MRI must be performed in emergency when CT scan is not conclusive despite a severe clinical condition or in case of cerebral venous thrombosis. Imaging modalities depend on clinical data, patient age and suspected epilepsy type.

Influence of aging on cortical activity associated with a visuo-motor task.

The aim of this study was to determine how cerebral aging influences the pattern of cortical oscillatory activity when a targeting movement with visual control is planned. Changes in cortical oscillatory activity were assessed by recording the event-related (de)synchronization (ERD/S) of micro and beta rhythms. Young and elderly subjects performed a distal movement, a proximal movement and a visuo-guided targeting movement. Our results demonstrated an increase in micro ERD over ipsilateral regions and showed the spatial extent of micro ERD over parietocentral and parietal regions during motor planning in elderly subjects compared to young ones. After the movement, the beta ERS was significantly modified (a decrease in slope and amplitude) in elderly subjects. The most pronounced age-related changes in ERD/S pattern were observed for the targeting movement. Our results suggest that motor planning is less efficient in elderly subjects. This deficit might result from impaired parietal integrative function and/or changes in inputs from subcortical structures. Subsequently, the changes observed in the post-movement phase might reflect a decrease in (reafferent) sensory inputs and hence impaired their input processing.

Changes in oscillatory cortical activity related to a visuomotor task in young and elderly healthy subjects.

OBJECTIVE: In order to better understand the spatio-temporal interaction of the activated cortical areas when the movement is visuo-guided and to assess the age effect on the spatio-temporal pattern of cortical activity, we have compared a proximo-distal movement with visual-motor control and hand-eye coordination (targeting movement) with a distal and a proximal movement. METHODS: Brain's electrical activity was studied using the analysis of event-related (de)synchronizations (ERD/S) of cortical mu and beta rhythms in 17 subjects, 8 young and 9 elderly subjects. RESULTS: In both populations, we found an earlier and broader mu and beta ERD during the preparation of the targeting movement compared to distal and proximal movements, principally involving the contralateral parietal region. During the execution, a spreading over the parietocentral region during proximal movement and over the parietal region during targeting movement was observed. After the execution of proximal and targeting movements, a wider and higher beta ERS was observed only in the young subjects. In the elderly subjects, our results showed a significant decrease of beta ERS during the targeting task. CONCLUSIONS: These results suggest there was a larger recruitment of cortical areas, involving notably the parietal cortex when the movement is visuo-guided. Moreover, cerebral aging-related changes in the spatio-temporal beta ERS pattern suggests an impaired sensory integration.

[Clinical and electrophysiological features of "cortical tremor"]. / Caractéristiques cliniques et électrophysiologiques du << tremblement cortical >>.

The purpose of this paper is to draw attention to one particular variant of tremor named "cortical tremor," which corresponds to rhythmic cortical myoclonus whose principal differential diagnosis is essential tremor. Diagnosis may be established with electrophysiological explorations (electromyography, accelerometry, electroencelography with back averaging, long latency reflexes, giant somesthesic evoked potentials). Clinical and electrophysiological features of 15 patients are reported and compared to cases previously published. Association with generalized or partial seizures is possible. Other cases in the family are often mentioned with a dominant autosomal transmission. The therapeutic approach is based on the use of antiepileptic drugs.

Does post-movement beta synchronization reflect an idling motor cortex?

After the completion of a voluntary movement, a synchronization of cortical beta rhythms is recorded over the contralateral central region, which is assumed to reflect the termination of the motor command. In order to test this hypothesis, we compared in eight healthy subjects the synchronization of EEG beta rhythms following active and passive index extension. The passive movement was also performed after deafferentation by ischaemic nerve block in three subjects. Beta synchronization was present in all subjects after both active and passive movements, and disappeared under ischaemia in all three subjects. Post-movement beta synchronization can not solely be explained by an idling motor cortex. It may also, at least in part, reflect a movement-related somatosensory processing.

Event-related variations in the activity of EEG-rhythms. Application to the physiology and the pathology of movements.

The averaging of the ongoing activity of the electroencephalogram (EEG) allows extracting the potentials that are time-locked and phase-locked to an event. These potentials are described as evoked potentials. There is another type of change in the ongoing EEG, which is time-locked but not phase-locked to an event: the EEG rhythm reactivity, also called "Event-Related Desynchronization and Synchronization" (ERD/ERS) by Pfurtscheller. These changes are often visible to the naked eye but they cannot be extracted by the averaging technique. Their quantification requires another method, which was suggested by Pfurtscheller and Aranibar in 1977. This method consists in measuring the temporal evolution of the power of EEG signal within a given frequency band before, during, and after an event. ERD corresponds to the decrease in power of an EEG rhythm related to an event. Conversely, ERS corresponds to an increase in amplitude of an EEG rhythm related to the event. ERD represents the activation of the subjacent cortical areas. ERS would partly traduce the setting at rest of the cortex; it would also be related to the somesthetics afferents inputs. This method can be applied to the study of cortical activation in many situations: memory tasks, auditory processing, attention, anticipatory behavior, and voluntary movement. Thus, a voluntary self-paced movement of the dominant hand is preceded by an ERD of mu and beta rhythms occurring respectively 2 000 and 1 500 ms before the movement onset. This ERD is recorded over the contralateral central region. It becomes bilateral at the movement onset and reaches its maximum at the movement offset. It is then followed by an ERS of the beta rhythms. We show that ERD/ERS phenomena vary with the type of movement, and that their study allows exploring the modifications of cortical excitability that are observed in Parkinson's disease and in epilepsy with focal motor seizures.

Brief and sustained movements: differences in event-related (de)synchronization (ERD/ERS) patterns.

OBJECTIVE: (1) To determine if there are changes in event-related desynchronization/event-related synchronization (ERD/ERS) patterns when the movement is sustained? (2) To determine, from a technical point of view for ERD calculation, if it is possible to take the reference period during muscular activation? METHODS: Eight healthy subjects performed two series of brief and sustained self-paced extensions with their dominant wrist. The end of the sustained movement was externally triggered by the examinator. ERD/ERS was calculated in mu and beta bands from 13 source derivations covering motor areas, computed from 29 scalp electrodes. Movement onset and offset were determined by electromyographic activity (EMG) of wrist extensors. RESULTS: When the movement was sustained, power in the mu and beta bands returned to baseline values within 4-5 s. Movement duration had little effect, if at all, on both pre and post-movement periods. Compared to brief movement, after the onset of the prolonged movement, mu ERD just returned to baseline, without synchronization. In contrast, beta ERS was still present though earlier and much lower. CONCLUSIONS: The reference period for ERD calculation may be taken during muscular activation if its duration is long enough. Beta synchronization may occur despite a non-deactivated motor cortex, suggesting a contribution from afferent somesthetic inputs.