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We report on a 64 year-old woman presenting with Epilepsia Partialis Continua (EPC) affecting the left hand since the age of 24 without neurological deficit. Structural MRI showed a region of focal cortical dysplasia (FCD) over the right central gyrus and lesions in the mesial frontal and occipital cortex secondary to perinatal hypoxic injury. Ictal spike haemodynamic mapping using simultaneous EEG-fMRI revealed significant BOLD signal changes prominent in the region of FCD (larger cluster), occipital cortex (global statistical maximum), prefrontal cortex and cerebellum. The cluster over FCD was in good agreement with the result of EEG source analysis. Our findings provide an interesting illustration of the ability of EEG-fMRI to reveal epileptogenic networks confirming the intrinsic epileptogenic properties of dysplastic neurons. [ABSTRACT FROM AUTHOR]

In recent years, imaging has emerged as a promising source of several intriguing biomarkers in epilepsy, due to the impressive growth of imaging technology, supported by methodological advances and integrations of post-processing techniques. Bearing in mind the mutually influencing connection between sleep and epilepsy, we focused on sleep-related hypermotor epilepsy (SHE) and sudden unexpected death in epilepsy (SUDEP), aiming to make order and clarify possible clinical utility of emerging multimodal imaging biomarkers of these two epilepsy-related entities commonly occurring during sleep. Regarding SHE, advanced structural techniques might soon emerge as a promising source of diagnostic and predictive biomarkers, tailoring a targeted therapeutic (surgical) approach for MRI-negative subjects. Functional and metabolic imaging may instead unveil SHE's extensive and night-related altered brain networks, providing insights into distinctions and similarities with non-epileptic sleep phenomena, such as parasomnias. SUDEP is considered a storm that strikes without warning signals, but objective subtle structural and functional alterations in autonomic, cardiorespiratory, and arousal centers are present in patients eventually experiencing SUDEP. These alterations could be seen both as susceptibility and diagnostic biomarkers of the underlying pathological ongoing loop ultimately ending in death. Finally, given that SHE and SUDEP are rare phenomena, most evidence on the topic is derived from small single-center experiences with scarcely comparable results, hampering the possibility of performing any meta-analytic approach. Multicenter, longitudinal, well-designed studies are strongly encouraged., Competing Interests: Declaration of competing interest A.E. Vaudano received personal compensation as scientific advisory board member for Angelini Pharma. None of other authors reported disclosures relevant financial or other conflicts of interest., (Copyright © 2023. Published by Elsevier Ltd.)

Objective: Nearly half of people with epilepsy (PWE) are expected to develop seizure clusters (SC), with the subsequent risk of hospitalization. The aim of the present study was to evaluate the use, effectiveness and safety of intravenous (IV) brivaracetam (BRV) in the treatment of SC., Methods: Retrospective multicentric study of patients with SC (≥ 2 seizures/24 h) who received IV BRV. Data collection occurred from January 2019 to April 2022 in 25 Italian neurology units. Primary efficacy outcome was seizure freedom up to 24 h from BRV administration. We also evaluated the risk of evolution into Status Epilepticus (SE) at 6, 12 and 24 h after treatment initiation. A Cox regression model was used to identify outcome predictors., Results: 97 patients were included (mean age 62 years), 74 (76%) of whom had a history of epilepsy (with drug resistant seizures in 49% of cases). BRV was administered as first line treatment in 16% of the episodes, while it was used as first or second drug after benzodiazepines failure in 49% and 35% of episodes, respectively. On the one hand, 58% patients were seizure free at 24 h after BRV administration and no other rescue medications were used in 75 out of 97 cases (77%) On the other hand, SC evolved into SE in 17% of cases. A higher probability of seizure relapse and/or evolution into SE was observed in patients without a prior history of epilepsy (HR 2.0; 95% CI 1.03 - 4.1) and in case of BRV administration as second/third line drug (HR 3.2; 95% CI 1.1 - 9.7). No severe treatment emergent adverse events were observed., Significance: In our cohort, IV BRV resulted to be well tolerated for the treatment of SC and it could be considered as a treatment option, particularly in case of in-hospital onset. However, the underlying etiology seems to be the main outcome predictor., Competing Interests: Declaration of Competing Interest S. Meletti received research grant support from the Ministry of Health (MOH, Italy); has received personal compensation as scientific advisory board member for UCB and EISAI. . Orlandi has received consultancy fees from EISAI. D. Audenino received personal compensation as scientific advisory board member for Eisai and UCB. S. Lattanzi has received speaker's or consultancy fees from Eisai, GW Pharmaceuticals, and UCB Pharma and has served on advisory boards for Angelini Pharma, Arvelle Therapeutics, BIAL, and GW Pharmaceuticals S. Cavalli, M. Coletti Moja, A. Estraneo, F. Giglia, G. Giovannini, A. Labate, A. Laganà, D. Marino, S. Miniello, P. Nazerian, A. Nilo, G. d'Orsi, G. Pauletto, M. Piccioli, F. Pescini, F. Ranzato, R. Renna, A. Sabetta, S. Servo, L. Sicurella, C. Vollono, S. Zappulla, A. Zini, L. Zummo report no disclosures., (Copyright © 2023 British Epilepsy Association. Published by Elsevier Ltd. All rights reserved.)

Background: Knowledge regarding consequences among status epilepticus (SE) survivors is still scarce. We assessed the risk of recurrence in a cohort of first-ever adult SE survivors, comparing the clinical features of patients with recurrent and incident events., Methods: We reviewed our prospective register of consecutive SE patients, from September 1st 2013 to September 1st 2020. We excluded post-anoxic events and those patients with a SE prior the study period. We examined the effect of clinical predictors on the risk of subsequent SE through Cox proportional hazard regression, while the risk of recurrence was estimated through a survival analysis., Results: 430 patients were considered (mean follow-up: 23.3 months). 44 patients experienced SE recurrence, whereas 386 patients presented an isolated event. The highest risk of recurrence was observed within 6 months from the index event (7.9%), whereas the cumulative recurrence rate was 9.5%, 13%, and 20.5% at 6 months, 1 year, and 4-years respectively. SE recurrence was independently associated to remote (HR 2.8 - 95% CI 1.4 to 6.0) or progressive symptomatic etiologies (HR 3.9 - 95% CI 1.8 to 8.7) and it was higher for Super-Refractory SE (SRSE) cases (HR 3.3 - 95% CI 1.4 to 7.8). High STESS values (p = 0.01) and SE refractoriness (p = 0.01) were associated with early relapses (within 6 months from the index event)., Conclusions: SE recurrence involved a significantly proportion of our cohort. Etiology other than acute symptomatic and SRSE were independently associated with a higher risk of recurrence, in particular within 6 months from the index event., (Copyright © 2022 British Epilepsy Association. Published by Elsevier Ltd. All rights reserved.)

Introduction: Status epilepticus (SE) is a neurological emergency and in particular nonconvulsive SE (NCSE) represents a diagnostic challenge. To improve clinical decision-making, cerebral perfusion-computed tomography (PCT) has been shown as a helpful tool to support the diagnosis of focal NCSE., Materials and Methods: This is a monocentric retrospective study. Among the 602 cases of SE observed between September 2013 and April 2020 we included 21 patients that were studied with PCT. The perfusion maps were first visually analysed then a quantitative analysis (by regions of interest, ROI) was obtained. For each patient, the diagnostic EEG was reviewed and classified in accordance to the Salzburg Criteria for NCSE (SCC) as definite (D-NCSE) and possible (P-NCSE). Finally, we analysed the relationship between PCT and EEG patterns., Results: Hyper-perfusion was observed in 18 patients (86%), while in the remaining 3 (14%) a normo-perfused pattern was present. Hyper-perfusion was observed in 14 of the D-NCSE group (88%) and in the two patients with a P-NCSE (100%). No one among the patients with a P-NCSE had a thalamic hyper-perfusion, while among the 6 patients with continuous sustained epileptiform discharges > 2.5 Hz (pattern 1 of SCC), 4 (67%) showed cortical plus thalamic hyper-perfusion., Conclusions: PCT could facilitate the differential diagnosis and speed-up the diagnostic process of NCSE in emergency situations. Finding cortical multi-lobar hyper-perfusion, especially if present together with homolateral thalamic hyper-perfusion in a patient with an acute-onset of motor/sensory/language deficits is highly suggestive for the presence of NCSE and is particularly related to continuous/sustained ictal patterns., (Copyright © 2021 British Epilepsy Association. Published by Elsevier Ltd. All rights reserved.)

Purpose: to evaluate the use, effectiveness, and adverse events of intravenous brivaracetam (BRV) in status epilepticus (SE)., Methods: a retrospective multicentric study involving 24 Italian neurology units was performed from March 2018 to June 2020. A shared case report form was used across participating centres to limit biases of retrospective data collection. Diagnosis and classification of SE followed the 2015 ILAE proposal. We considered a trial with BRV a success when it was the last administered drug prior the clinical and/or EEG resolution of seizures, and the SE did not recur during hospital observation. In addition, we considered cases with early response, defined as SE resolved within 6 h after BRV administration., Results: 56 patients were included (mean age 62 years; 57 % male). A previous diagnosis of epilepsy was present in 21 (38 %). Regarding SE etiology classification 46 % were acute symptomatic, 18 % remote and 16 % progressive symptomatic. SE episodes with prominent motor features were the majority (80 %). BRV was administered as first drug after benzodiazepine failure in 21 % episodes, while it was used as the second or the third (or more) drug in the 38 % and 38 % of episodes respectively. The median loading dose was 100 mg (range 50-300 mg). BRV was effective in 32 cases (57 %). An early response was documented in 22 patients (39 % of the whole sample). The use of the BRV within 6 h from SE onset was independently associated to an early SE resolution (OR 32; 95 % CI 3.39-202; p = 0.002). No severe treatment emergent adverse events were observed., Conclusion: BRV proved to be useful and safe for the treatment of SE. Time to seizures resolution appears shorter when it is administered in the early phases of SE., (Copyright © 2021 British Epilepsy Association. Published by Elsevier Ltd. All rights reserved.)