•In patients with progressive myoclonus epilepsy, cortico-muscular coherence significantly decreased during Perampanel treatment.•Out-degree and betweenness centrality increased on contralateral ...motor cortex during Perampanel treatment.•The improvement of the myoclonus induced by Perampanel resulted from restored leadership of the contralateral motor cortex.
To investigate the mechanisms by which Perampanel (PER) reduces the severity of action myoclonus, we studied on MEG signals the changes occurring in cortico-muscular coherence (CMC) and cortico-cortical connectivity in patients with progressive myoclonus epilepsies.
The subjects performed an isometric extension of the hand; CMC and cortico-cortical connectivity were assessed using autoregressive models and generalized partial-directed coherence. The contralateral (Co) sensors showing average CMC values >0.7 of the maximum (set to 1) were grouped as central (C) regions of interest (ROI), while adjacent sensors showing CMC values >0.3 were grouped as Surrounding (Sr) ROIs.
Under PER treatment, CMC decreased on Co C and Sr ROIs, but also on homologous ipsilateral (Ip) ROIs; out-degrees and betweenness centrality increased in Co ROIs and decreased in Ip ROIs. The flow from Ip to Co ROIs and from activated muscles to Ip C ROI decreased.
The improvement of myoclonus corresponded to decreased CMC and recovered leadership of the cortical regions directly involved in the motor task, with a reduced interference of ipsilateral areas.
Our study highlights on mechanisms suitable to treating myoclonus and suggests the role of a reduced local synchronization together a better control of distant synaptic effects.
Rasmussen encephalitis (RE) is a rare but severe immune-mediated brain disorder leading to unilateral hemispheric atrophy, associated progressive neurological dysfunction and intractable seizures. ...Recent data on the pathogenesis of the disease, its clinical and paraclinical presentation, and therapeutic approaches are summarized. Based on these data, we propose formal diagnostic criteria and a therapeutic pathway for the management of RE patients.
Abstract This is the second of three papers that summarize the second symposium on Transition in Epilepsies held in Paris in June 2016. This paper addresses the outcome for some particularly ...challenging childhood-onset epileptic disorders with the goal of recommending the best approach to transition. We have grouped these disorders in five categories with a few examples for each. The first group includes disorders presenting in childhood that may have late- or adult-onset epilepsy (metabolic and mitochondrial disorders). The second group includes disorders with changing problems in adulthood (tuberous sclerosis complex, Rett syndrome, Dravet syndrome, and autism). A third group includes epilepsies that change with age (Childhood Absence Epilepsy, Juvenile Myoclonic Epilepsy, West Syndrome, and Lennox-Gastaut syndrome). A fourth group consists of epilepsies that vary in symptoms and severity depending on the age of onset (autoimmune encephalitis, Rasmussen's syndrome). A fifth group has epilepsy from structural causes that are less likely to evolve in adulthood. Finally we have included a discussion about the risk of later adulthood cerebrovascular disease and dementia following childhood-onset epilepsy. A detailed knowledge of each of these disorders should assist the process of transition to be certain that attention is paid to the most important age-related symptoms and concerns.
To conduct an open-label, add-on trial on safety and efficacy of levetiracetam in severe myoclonic epilepsy of infancy (SMEI).
SMEI patients were recruited from different centers according to the ...following criteria: age > or =3 years; at least four tonic-clonic seizures/month during the last 8 weeks; previous use of at least two drugs. Levetiracetam was orally administrated at starting dose of approximately 10 mg/kg/day up to 50 to 60 mg/kg/day in two doses. Treatment period included a 5- to 6-week up-titration phase and a 12-week evaluation phase. Efficacy variables were responder rate by seizure type and reduction of the mean number per week of each seizure type. Analysis was performed using Fisher exact and Wilcoxon tests.
Twenty-eight patients (mean age: 9.4 +/- 5.6 years) entered the study. Sixteen (57.1%) showed SCN1A mutations. Mean number of concomitant drugs was 2.5. Mean levetiracetam dose achieved was 2,016 mg/day. Twenty-three (82.1%) completed the trial. Responders were 64.2% for tonic-clonic, 60% for myoclonic, 60% for focal, and 44.4% for absence seizures. Number per week of tonic-clonic (median: 3 vs 1; p = 0.0001), myoclonic (median: 21 vs 3; p = 0.002), and focal seizures (median: 7.5 vs 3; p = 0.031) was significantly decreased compared to baseline. Levetiracetam effect was not related to age at onset and duration of epilepsy, genetic status, and concomitant therapy. Levetiracetam was well tolerated by subjects who completed the study. To date, follow-up ranges 6 to 36 months (mean, 16.2 +/- 13.4).
Levetiracetam add-on is effective and well tolerated in severe myoclonic epilepsy of infancy. Placebo-controlled studies should confirm these findings.
Objective
To define the electroclinical phenotype and long‐term outcomes in a cohort of patients with inv dup (15) syndrome.
Material and Methods
The electroclinical data of 45 patients (25 males) ...affected by inv dup (15) and seizures were retrospectively analysed, and long‐term follow‐up of epilepsy was evaluated.
Results
Epilepsy onset was marked by generalized seizures in 53% of patients, epileptic spasms in 51%, focal seizures in 26%, atypical absences in 11% and epileptic falls in 9%. The epileptic syndromes defined were: generalized epilepsy (26.7%), focal epilepsy (22.3%), epileptic encephalopathy with epileptic spasms as the only seizure type (17.7%) and Lennox‐Gastaut syndrome (33.3%). Drug‐resistant epilepsy was detected in 55.5% of patients. There was a significant higher prevalence of seizure‐free patients in those with seizure onset after the age of 5 years and with focal epilepsy, with respect to those with earlier epilepsy onset because most of these later developed an epileptic encephalopathy (69.2% vs 34.4%; P = .03), usually Lennox‐Gastaut Syndrome in type. In fact, among patients with early‐onset epilepsy, those presenting with epileptic spasms as the only seizure type associated with classical hypsarrhythmia achieved seizure freedom (P < .001) compared to patients with spasms and other seizure types associated with modified hypsarrhythmia.
Conclusions
Epilepsy in inv dup (15) leads to a more severe burden of disease. Frequently, these patients show drug resistance, in particular when epilepsy onset is before the age of five and features epileptic encephalopathy.
Abstract Background Patients presenting to the ED with altered mental status (AMS) and alcohol intoxication can clinically resemble patients with an intracranial hemorrhage (ICH). While ICH is ...quickly excluded with a head CT scan, it is common practice to defer imaging and allow the patient to metabolize in order to spare ED resources and minimize radiation exposure to the patient. While this reduces unnecessary scans, it may delay treatment in patients with occult intracranial hemorrhage, which some fear may increase morbidity and mortality. We sought to evaluate the safety of deferred CT imaging in these patients by evaluating whether time-to-scan significantly affects the rate of neurosurgical intervention. Methods In this retrospective chart review, all clinically alcohol intoxicated patients presenting to two university emergency departments were included. Time to order CT imaging, findings on imaging, and outcomes of these patients were determined. Patients were assessed in three groups: CT ordered within 1 hour of triage, CT ordered 1–3 hours from triage, and CT ordered 3 or more hours from triage. Results During the study period, 5943 patients were included in the study. Out of these, 0 patients who were scanned in less than 3 hours had intracranial findings on imaging requiring neurosurgery, while 1 patient with a deferred CT scan required a neurosurgical intervention, however it was not emergently performed. Conclusion Routine CT scanning of alcohol intoxicated patients with altered mental status is of low clinical value. Deferring CT imaging while monitoring improving clinical status appears to be a safe practice.
Seagrass beds have traditionally been considered to act as sinks for particles due to the reduction of flow velocities by the plant canopy. Yet, there is a paucity of measurements to confirm this ...role. In this work we illustrate changes in flow in the presence and absence of
Posidonia oceanica using an ADV, and provide direct measures of particle trapping by the use of sediment traps. We also describe a model to estimate sediment resuspension after measuring particle flux at different distances from the bottom. Measurements of particle flux are conducted parallel to the study of structural parameters of the
Posidonia meadow potentially involved in both particle trapping and retention. Data obtained on velocity profiles confirm previous findings that seagrass canopies slow down current velocities with intensities proportional to the canopy height of the plants. The projected surface area of the plants (LAI) significantly correlated with the total amount of particles trapped within the
Posidonia meadow, thus indicating seagrass canopy slightly increased particle trapping in the absence of resuspension. The trapping capacity of the canopy was not linearly correlated to LAI but significantly decreased at LAI above four, thus suggesting that other factors such as bending of the leaves and particle attachment to the surface may interfere with particle free sinking within the canopy at high projected surface area. The model proposed to estimate resuspension allowed to measure the retention capacity of the
P. oceanica meadow, this being up to 15 times higher compared to a barren bottom during situations of high energy (large eddies reaching the bottom). The results obtained provide direct quantitative support to seagrass beds promoting sediment accretion and demonstrate a promising avenue to provide the needed empirical support for the effect of seagrasses on depositional processes.
The authors investigated immunomodulatory treatments in 15 patients with Rasmussen encephalitis (RE) (14 with childhood and one with adolescent onset RE). Positive time-limited responses were ...obtained in 11 patients using variable combinations of corticosteroids, apheresis, and high-dose IV immunoglobulins. Although surgical exclusion of the affected hemisphere is the only treatment that halts disease progression, immunomodulation can be considered when early surgery is not feasible, in late-onset patients with slower disease progression, and in the few cases of bilateral disease.
Magnetoencephalography (MEG) recordings of interictal and/or ictal discharges can provide valuable information about the location of the epileptogenic area in the brain. Rasmussen Encephalitis (RE) ...is characterized by intractable epilepsy, progressive hemiparesis, and unilateral hemispheric atrophy. The progression of the symptoms to significant neurological impairment usually occurs within months to a few years. Five patients (mean age at first observation 7.6 ± 3 years) fitting Bien’s criteria for the diagnosis of RE were enrolled for the study and follow-up for at least 1.5 years (4–7 MEG measures per patient), in order to evaluate correlations between the evolution of clinical picture, neuroradiological and MEG findings. MEG data were visually inspected and interictal spikes were selected for the source localization by means of equivalent current dipoles (ECDs). Cortical thickness was evaluated from Magnetic Resonance Imaging (MRI). All patients showed ictal/interictal multifocality, reduction of background cortical magnetic activity, and progressive focal cortical atrophy on MRI of the affected hemisphere. Moreover, our preliminary data showed that dipole clusters were found mainly in the most atrophic cortical regions, suggesting a close relationship between MRI and MEG alterations.
Fine-scale spatial effects of a seagrass meadow on suspended particle transport were assessed from current speeds, orbital wave velocities, turbulent Reynolds stress,in situparticle concentrations, ...and sedimentation rates for a horizontal grid in a coastal seagrass (Posidonia oceanica) meadow at 2 depths and during low- and high-energy periods. For the low-energy period, the vertical reduction of the total kinetic energy, from 100 cm to ≈10 cm above the bottom, was larger in the meadow (up to 95%) than over the sand (35 to 75%). Velocity maps suggest that a recirculating flow formed in the meadow with a higher Reynolds stress at the edge of the meadow. Near the bed, concentrations of small particles (<10 μm diameter) were lower inside the meadow than over barren sand, while concentrations of large particles (>10 μm) were lower over the barren sand. For the period of stronger current and wave activity following a storm, nearbed turbulence and orbital wave velocity were elevated, though still lower inside the meadow than over the sand. For this high energy period, particle concentrations increased over the whole study area, but were still lowest deep inside the meadow. Overall, the horizontal spatial distribution of plants in the study area had a profound effect on the flow field and on vertical transport, even during the high-energy period. The reduced nearbed turbulence and lower sedimentation rate below the canopy confirms it as a calm zone with lower mixing compared to unvegetated areas.
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