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The maintenance of seizure control over time is a clinical priority in patients with epilepsy. The aim of this study was to assess the sustained seizure frequency reduction with adjunctive brivaracetam (BRV) in real-world practice. Patients with focal epilepsy prescribed add-on BRV were identified. Study outcomes included sustained seizure freedom and sustained seizure response, defined as a 100% and a ≥50% reduction in baseline seizure frequency that continued without interruption and without BRV withdrawal through the 12-month follow-up. Nine hundred ninety-four patients with a median age of 45 (interquartile range = 32-56) years were included. During the 1-year study period, sustained seizure freedom was achieved by 142 (14.3%) patients, of whom 72 (50.7%) were seizure-free from Day 1 of BRV treatment. Sustained seizure freedom was maintained for ≥6, ≥9, and 12 months by 14.3%, 11.9%, and 7.2% of patients from the study cohort. Sustained seizure response was reached by 383 (38.5%) patients; 236 of 383 (61.6%) achieved sustained ≥50% reduction in seizure frequency by Day 1, 94 of 383 (24.5%) by Month 4, and 53 of 383 (13.8%) by Month 7 up to Month 12. Adjunctive BRV was associated with sustained seizure frequency reduction from the first day of treatment in a subset of patients with uncontrolled focal epilepsy.
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Anticonvulsivantes , Epilepsias Parciales , Adulto , Anticonvulsivantes/uso terapéutico , Método Doble Ciego , Quimioterapia Combinada , Epilepsias Parciales/tratamiento farmacológico , Libertad , Humanos , Persona de Mediana Edad , Pirrolidinonas/uso terapéutico , Convulsiones/tratamiento farmacológico , Resultado del TratamientoRESUMEN
AIMS: Stroke is the most commonly identified cause of late-onset epilepsy. Risk factors for poststroke epilepsy (PSE) are partially elucidated, and many studies have been performed in recent years. We aimed to update our previous systematic review and meta-analysis on risk factors for PSE. METHODS: PubMed, Google Scholar, and Scopus databases were searched. Articles published in English (1987-2019) were included. Odds ratios (OR) and mean values were calculated for examined variables. RESULTS: Thirty studies with different designs were included, enrolling 26,045 patients who experienced stroke, of whom 1800 had PSE, corresponding to a prevalence of 7%. Cortical lesions (OR: 3.58, 95% confidence interval (CI): 2.35-5.46, pâ¯<â¯0.001), hemorrhagic component (OR: 2.47, 95% CI: 1.68-3.64, pâ¯<â¯0.001), early seizures (ES) (OR: 4.88, 95% CI: 3.08-7.72, pâ¯<â¯0.001), and younger age at stroke onset (difference in means: 2.97â¯years, 95% CI: 0.78 to 5.16, pâ¯=â¯0.008) favor PSE. Sex and acute treatment with recombinant tissue plasminogen activator (rtPA) do not predict the occurrence of PSE. CONCLUSION: Despite limitations due to the uneven quality and design of the studies, the present meta-analysis confirms that cortical involvement, hemorrhagic component, and ES are associated with a higher risk of PSE. In this update, younger age at stroke onset but not thrombolytic treatment seems to increase the risk for PSE. This article is part of the Special Issue "Seizures & Stroke".
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Epilepsia/etiología , Accidente Cerebrovascular/complicaciones , Accidente Cerebrovascular/terapia , Terapia Trombolítica/tendencias , Edad de Inicio , Epilepsia/inducido químicamente , Epilepsia/diagnóstico , Humanos , Valor Predictivo de las Pruebas , Prevalencia , Factores de Riesgo , Convulsiones/inducido químicamente , Convulsiones/diagnóstico , Convulsiones/etiología , Accidente Cerebrovascular/diagnóstico , Terapia Trombolítica/efectos adversos , Activador de Tejido Plasminógeno/efectos adversosRESUMEN
The diagnosis of psychogenic nonepileptic seizures (PNES) by means of electroencephalography (EEG) is not a trivial task during clinical practice for neurologists. No clear PNES electrophysiological biomarker has yet been found, and the only tool available for diagnosis is video EEG monitoring with recording of a typical episode and clinical history of the subject. In this paper, a data-driven machine learning (ML) pipeline for classifying EEG segments (i.e., epochs) of PNES and healthy controls (CNT) is introduced. This software pipeline consists of a semiautomatic signal processing technique and a supervised ML classifier to aid clinical discriminative diagnosis of PNES by means of an EEG time series. In our ML pipeline, statistical features like the mean, standard deviation, kurtosis, and skewness are extracted in a power spectral density (PSD) map split up in five conventional EEG rhythms (delta, theta, alpha, beta, and the whole band, i.e., 1-32 Hz). Then, the feature vector is fed into three different supervised ML algorithms, namely, the support vector machine (SVM), linear discriminant analysis (LDA), and Bayesian network (BN), to perform EEG segment classification tasks for CNT vs. PNES. The performance of the pipeline algorithm was evaluated on a dataset of 20 EEG signals (10 PNES and 10 CNT) that was recorded in eyes-closed resting condition at the Regional Epilepsy Centre, Great Metropolitan Hospital of Reggio Calabria, University of Catanzaro, Italy. The experimental results showed that PNES vs. CNT discrimination tasks performed via the ML algorithm and validated with random split (RS) achieved an average accuracy of 0.97 ± 0.013 (RS-SVM), 0.99 ± 0.02 (RS-LDA), and 0.82 ± 0.109 (RS-BN). Meanwhile, with leave-one-out (LOO) validation, an average accuracy of 0.98 ± 0.0233 (LOO-SVM), 0.98 ± 0.124 (LOO-LDA), and 0.81 ± 0.109 (LOO-BN) was achieved. Our findings showed that BN was outperformed by SVM and LDA. The promising results of the proposed software pipeline suggest that it may be a valuable tool to support existing clinical diagnosis.
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Electroencefalografía/métodos , Aprendizaje Automático , Convulsiones/diagnóstico , Programas Informáticos , Algoritmos , Humanos , Convulsiones/fisiopatología , Máquina de Vectores de SoporteRESUMEN
BACKGROUND: Epilepsy and hypertension are common chronic conditions, both showing high prevalence in older age groups. This review outlines current experimental and clinical evidence on both direct and indirect role of hypertension in epileptogenesis and discusses the principles of drug treatment in patients with hypertension and epilepsy. METHODS: We selected English-written articles on epilepsy, hypertension, stroke, and cerebrovascular disease until December, 2018. RESULTS: Renin-angiotensin system might play a central role in the direct interaction between hypertension and epilepsy, but other mechanisms may be contemplated. Large-artery stroke, small vessel disease and posterior reversible leukoencephalopathy syndrome are hypertension-related brain lesions able to determine epilepsy by indirect mechanisms. The role of hypertension as an independent risk factor for post-stroke epilepsy has not been demonstrated. The role of hypertension-related small vessel disease in adult-onset epilepsy has been demonstrated. Posterior reversible encephalopathy syndrome is an acute condition, often caused by a hypertensive crisis, associated with the occurrence of acute symptomatic seizures. Chronic antiepileptic treatment should consider the risk of drug-drug interactions with antihypertensives. CONCLUSIONS: Current evidence from preclinical and clinical studies supports the vision that hypertension may be a cause of seizures and epilepsy through direct or indirect mechanisms. In both post-stroke epilepsy and small vessel disease-associated epilepsy, chronic antiepileptic treatment is recommended. In posterior reversible encephalopathy syndrome blood pressure must be rapidly lowered and prompt antiepileptic treatment should be initiated.
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Enfermedades de los Pequeños Vasos Cerebrales/complicaciones , Epilepsia/etiología , Hipertensión/complicaciones , Convulsiones/etiología , Accidente Cerebrovascular/complicaciones , HumanosRESUMEN
The aim of this study is to report current clinical practice for sleep induction in Italian epilepsy centers. We administered an online-structured survey between March and November 2017 and collected data from pediatric and adult neurophysiologists belonging to 73 epilepsy centers. The preferred time for EEG recording is variable, depending on daily schedule of each laboratory. To facilitate spontaneous sleep during nap EEGs, almost all centers require sleep deprivation before the examination, with partial loss preferred to total deprivation in most centers (58/73 vs 12/73, p < 0.001). Other non-pharmacological procedures include breast/bottle feeding or listening to music (encouraged in most centers). Pharmacological sleep induction is performed in 40% of laboratories, more commonly in children than in adults (27/60 vs 7/42, p = 0.003). Melatonin is the most frequently prescribed drug to facilitate spontaneous sleep (one third of participating centers). Our study highlights the great heterogeneity among Italian epilepsy centers in current clinical practice for sleep EEG recordings. Expert consensus for sleep induction procedure is warranted.
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Electroencefalografía , Epilepsia/epidemiología , Epilepsia/fisiopatología , Sistemas en Línea , Sueño/fisiología , Adolescente , Adulto , Niño , Femenino , Humanos , Italia/epidemiología , Masculino , Persona de Mediana Edad , Privación de Sueño/fisiopatología , Encuestas y Cuestionarios , Adulto JovenRESUMEN
OBJECTIVE: Actual knowledge on evolution of Angelman syndrome (AS) relies on questionnaire-based cohort studies, phone interviews, or small retrospective cohort studies focused on specific clinical-genetic features. These reports provide conflicting results. The aim of this study was to assess the long-term outcome of epilepsy, sleep disorders, and EEG in a vast series of AS subjects. METHODS: We collected patients with genetically confirmed AS, aged ≥14years, followed in three tertiary epilepsy Centers or attending the meetings of the Italian Organization for AS (OrSA). Retrospective clinical and EEG data were retrieved from hospital archives or family documents. At index evaluation (IE) (last visit at tertiary Centers or single visit during OrSA meetings), caregivers were interviewed about anamnestic data and filled questionnaires on sleep disorders and daily-living skills. Patients underwent general and neurologic evaluation, and video-EEG recordings. All available EEGs were analyzed to compare evolution of spike-wave index (SWI) over the years. RESULTS: Forty-six subjects aged 14-45years were included: 24 from tertiary Centers, 22 from OrSA meetings. During childhood, 42/46 (91.3%) had seizures, which improved over the years in all subjects. Among patients with epilepsy, 27(64%) became seizure-free at a median age of 10years and 4 remained seizure-free even after antiepileptic withdrawal. During childhood, 39/46 (84.8%) had sleep disorders, which improved in 27/39 (69%) over the years. At IE, daily-living skills corresponded to age≤1.6years in 29/46 (63%). Electroencephalogram showed typical AS patterns in 35/46 (76.1%). In EEGs recorded from 10 patients, SWI was not significantly different between infancy/childhood and adolescence/adulthood. CONCLUSION: Improvement of epilepsy or sleep disorders should not disregard the clinical suspicion of AS in adolescent or adult patients with suggestive features. Drug withdrawal might be considered in the management of epilepsy despite the persistence of epileptiform abnormalities.
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Síndrome de Angelman/complicaciones , Epilepsia/complicaciones , Trastornos del Sueño-Vigilia/etiología , Adolescente , Adulto , Análisis de Varianza , Síndrome de Angelman/fisiopatología , Electroencefalografía , Epilepsia/fisiopatología , Femenino , Humanos , Masculino , Persona de Mediana Edad , Estudios Retrospectivos , Trastornos del Sueño-Vigilia/fisiopatología , Adulto JovenRESUMEN
The role of different factors in influencing the risk of seizures during multiple sclerosis (MS) is not known. To perform a systematic review and meta-analysis of risk factors for epilepsy during MS. Pubmed, Google scholar, and Scopus databases were searched. Articles published in English (1986-2016) were included. Nine studies were included (3 retrospective cohort and 6 case-control) enrolling 2845 MS patients (217 with epilepsy; 7.6%). MS patients with epilepsy had a younger age at onset compared to MS patients without seizures (difference in means = -5.42 years, 95% CI -7.19 to -3.66, p < 0.001). Mean EDSS value at inclusion tended to be higher in patients with epilepsy, without reaching statistical significance (difference in means = 0.45, 95% CI -0.01 to 0.91, p = 0.054). No differences were observed in sex distribution (OR = 0.94, 95% CI 0.51-1.72, p = 0.83) and clinical form (OR = 1.03, 95% CI 0.33-3.21, p = 0.96). Two studies evaluated presence and number of cortical lesions as a risk factor for epilepsy in MS using different MRI techniques: in one study, cortical lesions were more frequently observed in patients with epilepsy (OR = 7.06, 95% CI 2.39-20.8; p < 0.001). In the other, cortico-juxtacortical lesions were more frequently observed in patients with epilepsy (OR = 2.6, 95% CI 1.0-6.5; p = 0.047). Studies about risk factors for epilepsy during MS are heterogeneous. Compared to MS patients without seizures, patients with epilepsy have an earlier MS onset and a higher EDSS score after similar disease duration. Clinical form of MS and sex do not predict the appearance of seizures.
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Epilepsia/epidemiología , Epilepsia/etiología , Esclerosis Múltiple/complicaciones , Edad de Inicio , Femenino , Humanos , Masculino , Factores de RiesgoRESUMEN
INTRODUCTION: Post-stroke epilepsy (PSE) is one of the most common causes of acquired epilepsy. Nevertheless, there is limited evidence regarding the clinical profile of antiseizure medications (ASMs) in PSE. This study aims to evaluate the 12-month effectiveness and tolerability of perampanel (PER) used as only add-on treatment in patients with PSE in a real-world setting. METHODS: We performed a subgroup analysis of PSE patients included in a previous retrospective, longitudinal, multicentre observational study on adults. Treatment discontinuation, seizure frequency and adverse events were collected at 3, 6 and 12 months. Sub-analyses by early (≤1 previous ASM) or late PER add-on were also conducted. RESULTS: Our analysis included 56 individuals with PSE, characterized by varying initial treatment modalities and timeframes relative to disease onset. We found notable retention rates (92.8%, 83.7%, and 69% at 3, 6, and 12 months), with treatment withdrawal mainly due to poor tolerability. One year after PER introduction, seizure frequency significantly reduced, with a responder rate (≥50% reduction) of 83.9% and a seizure-free rate of 51.6%. Adverse events occurred in 25 (46.3%) patients, mainly dizziness, irritability, and behavioural disorders. No major statistical differences were found between early (30 patients, 53.6%) and late add-on groups, except for a higher 6-month responder rate in the early add-on group. CONCLUSION: Adjunctive PER was effective and well-tolerated in patients with PSE in a real-world setting. Perampanel demonstrated good efficacy and safety as both early and late add-on treatment, making it a compelling option for this unique patient population.
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Anticonvulsivantes , Epilepsia , Nitrilos , Piridonas , Accidente Cerebrovascular , Humanos , Piridonas/uso terapéutico , Piridonas/efectos adversos , Anticonvulsivantes/uso terapéutico , Femenino , Masculino , Anciano , Persona de Mediana Edad , Epilepsia/tratamiento farmacológico , Estudios Retrospectivos , Accidente Cerebrovascular/complicaciones , Accidente Cerebrovascular/tratamiento farmacológico , Estudios Longitudinales , Resultado del Tratamiento , Quimioterapia Combinada , Anciano de 80 o más Años , AdultoRESUMEN
INTRODUCTION: Drug management of epilepsy in the elderly presents unique but data on this population are scarce. This study aimed to assess the effectiveness and tolerability of perampanel (PER) used as only add-on to a background anti-seizure medication (ASM) in the elderly in a real-world setting. METHODS: We performed a subgroup analysis of patients aged ≥65 years included in a previous 12-month multicenter study on adults. Treatment discontinuation, seizure frequency, and adverse events were recorded at 3, 6 and 12 months after PER introduction. Sub-analyses by early (≤1 previous ASM) or late PER add-on were also conducted. RESULTS: The sample included 65 subjects (mean age: 75.7 ± 7.2 years), with mainly focal (73.8%) epilepsy. The mean PER daily dose was ≈4 mg during all follow-up. Retention rates at 3, 6, and 12 months were 90.5%, 89.6%, and 79.4%ly. The baseline median normalized per 28-day seizure number significantly decreased at 3-, 6- and 12-month visits. One year after PER introduction, the responder rate (≥50% reduction in baseline seizure frequency) was 89.7%, with a seizure freedom rate of 72.4%. Adverse events occurred in 22 (34.9%) patients with dizziness and irritability being the most frequent. No major differences between early (41 patients, 63.1%), and late add-on groups were observed. CONCLUSION: Adjunctive PER was effective and well-tolerated when used as only add-on treatment in elderly people with epilepsy in clinical practice, thus representing a suitable therapeutic option in this age category.
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Anticonvulsivantes , Epilepsia , Anciano , Anciano de 80 o más Años , Humanos , Anticonvulsivantes/efectos adversos , Quimioterapia Combinada , Epilepsia/tratamiento farmacológico , Estudios Retrospectivos , Convulsiones/tratamiento farmacológico , Resultado del Tratamiento , Estudios Multicéntricos como Asunto , Estudios Observacionales como AsuntoRESUMEN
INTRODUCTION: Angelman syndrome (AS) is a neurogenetic disorder due to deficient expression of the maternal copy of the UBE3A gene, which encodes ubiquitin ligase E3A protein. Severe developmental delay, seizures and other neurological disorders characterize AS. AREAS COVERED: In this review, we focus on a comprehensive therapeutic approach to the most disabling neurological manifestations of AS: epilepsy, sleep disturbances, behavioral and movement disorders. Articles were identified through PubMed and Google Scholar up to October 2021. EXPERT OPINION: Evidence for the treatment of neurological manifestations in AS mainly derives from poor quality studies (case reports, small case series, expert opinions). Seizures can be polymorphic and includes atypical absences, myoclonic, generalized tonic-clonic, unilateral clonic, or atonic attacks. Sodium valproate, levetiracetam, and benzodiazepines are the most commonly used anti-seizure medications. Melatonin or mirtazapine seem to improve sleep quality. Antipsychotics, antidepressants, and anxiolytics have been proposed for the treatment of behavioral manifestations, but no evidence-based studies are available. Non-pharmacological approach may also be useful. Mild dystonia is common but usually does not significantly impact patients' motor performances. Well-conducted clinical trials aimed to evaluate treatment of neurological complications of AS are warranted. Gene and molecular precision therapies represent a fascinating area of research in the future.
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Síndrome de Angelman , Epilepsia Generalizada , Epilepsia , Síndrome de Angelman/complicaciones , Síndrome de Angelman/tratamiento farmacológico , Síndrome de Angelman/genética , Anticonvulsivantes/uso terapéutico , Epilepsia/tratamiento farmacológico , Epilepsia Generalizada/tratamiento farmacológico , Humanos , Ácido Valproico/uso terapéuticoRESUMEN
OBJECTIVE: Perampanel (PER) is indicated as adjunctive antiseizure medication (ASM) in adolescents and adults with epilepsy. Data from clinical trials show good efficacy and tolerability, while limited information is available on the routine clinical use of PER, especially when used as only add-on treatment. METHODS: we performed an observational, retrospective, multicenter study on people with focal or generalized epilepsy aged >12 years, consecutively recruited from 52 Italian epilepsy centers. All patients received PER as the only add-on treatment to a background ASM according to standard clinical practice. Retention rate, seizure frequency and adverse events were recorded at 3, 6 and 12 months after PER introduction. Sub-analyses by early or late use of PER and by concomitant ASM were also conducted. RESULTS: 503 patients were included (age 36.5±19.9 years). Eighty-one per cent had focal epilepsy. Overall, the retention rate was very high in the whole group (89% at 12 months) according with efficacy measures. No major differences were observed in the sub-analyses, although patients who used PER as early add-on, as compared with late add-on, more often reached early seizure freedom at 3 months follow-up (66% vs. 53%, p=0.05). Treatment-emergent adverse events occurred in 25%, far less commonly than in PER randomized trials. SIGNIFICANCE: this study confirms the good efficacy and safety of PER for focal or generalized epilepsy in real-life conditions. We provide robust data about its effectiveness as only add-on treatment even in patients with a long-standing history of epilepsy and previously treated with many ASMs.
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Status epilepticus (SE) is a neurological and medical emergency, defined as a condition resulting either from the failure of the mechanisms responsible of seizure self-limitation or from the initiation of mechanisms which lead to atypically prolonged seizures. Further than death, SE can have long-term consequences, including neuronal injury, depending on the type, cause and duration of seizures with severe associated disabilities. In Europe, SE shows an incidence rate ranging about 9 to 40/100,000/y. In adults, mortality of patients with SE is ~30%, and even higher (up to 40%) in refractory status epilepticus. To date, etiology, duration, presence of comorbidity, level of consciousness, semiology and age are the main clinical predictors of SE outcome.
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Introduction: typical absences (TAs), are brief, generalized epileptic seizures of abrupt onset and termination clinically manifesting with impairment of awareness and associated with 3 Hz spike-wave discharges on EEG. TAs may occur in different idiopathic generalized epilepsies (IGE). Despite treatment with adequate anti-seizure medications (ASMs), TAs may persist in ~25% of subjects. This narrative review focuses on the therapeutic approach to difficult-to-treat TAs occurring in the setting of IGE.Areas covered: a literature search was conducted on the topic of treatment of TAs.Expert opinion: ethosuximide (ESX), valproic acid (VPA) and lamotrigine (LTG), alone or in combination, are considered the first-choice drugs. In women of childbearing potential, VPA should be avoided. Alternative therapies (benzodiazepines, levetiracetam, topiramate, or zonisamide) should be considered in subjects unresponsive to monotherapy after the exclusion of pseudo-drug resistance. Newer ASMs such as brivaracetam and perampanel seem to be promising options. Well-conducted clinical trials aimed to evaluate the efficacy of alternative monotherapy (beyond ESX, VPA or LTG) or combination of ASMs on difficult-to-treat TAs, are warranted.
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Anticonvulsivantes/administración & dosificación , Epilepsia Tipo Ausencia/tratamiento farmacológico , Epilepsia Generalizada/tratamiento farmacológico , Resistencia a Medicamentos , Quimioterapia Combinada , Electroencefalografía , Epilepsia Tipo Ausencia/fisiopatología , Epilepsia Generalizada/fisiopatología , HumanosRESUMEN
INTRODUCTION: Angelman syndrome (AS) is a neurodevelopmental disorder characterized by cognitive disability, speech impairment, hyperactivity and seizures. Movement disorders have been reported in almost all AS subjects and they are described as "tremulous movements of limbs, unsteadiness, clumsiness or quick, jerky motions". The presence of dystonia has barely been mentioned in subjects with AS and has never been studied in detail. The purpose of this study is to evaluate the prevalence, clinical features and severity of dystonia in a series of adolescents and adults with AS. METHODS: Whole body video recordings of patients with genetically confirmed AS were evaluated. Dystonia was evaluated by mean of the movement subscale of Burke-Fahn-Marsden Dystonia Rating Scale (BFM). RESULTS: Forty-four subjects with AS were evaluated. Fourteen recordings were excluded due to poor cooperation. We finally analyzed data of 30 subjects (15 F) with a median age of 28 years (range 15-51). Dystonia was present in 28/30 (93.3%) subjects. Among these, dystonia involved the upper limbs in 28/28 (100%), lower limbs in 8/28 (28.5%), mouth in 7/28 (25%), neck in 3/28 (10.7%), trunk in 1/28 (3.6%). Severity of dystonia ranged from slight to moderate. There was a linear correlation between severity of dystonia and increasing age. There was no difference in terms of severity of dystonia among genetic subgroups. CONCLUSIONS: Dystonia is a common and previously underrecognized clinical feature of adults and adolescents with AS.
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Síndrome de Angelman , Estimulación Encefálica Profunda , Distonía , Trastornos Distónicos , Adolescente , Adulto , Síndrome de Angelman/complicaciones , Síndrome de Angelman/diagnóstico , Distonía/diagnóstico , Distonía/epidemiología , Distonía/terapia , Trastornos Distónicos/diagnóstico , Trastornos Distónicos/terapia , Globo Pálido , Humanos , Persona de Mediana Edad , Resultado del Tratamiento , Adulto JovenRESUMEN
INTRODUCTION: Juvenile myoclonic epilepsy (JME), also known as Janz syndrome, is a common form of generalized epilepsy of presumed genetic origin representing up to 10% of all epilepsy cases. Despite adequate anti-seizure medication (ASM) treatment, seizures persist in one-third of JME patients. AREAS COVERED: A literature search was conducted using Pubmed search on the topics of drug-resistant JME. EXPERT OPINION: About 30% of JME patients are drug-resistant. Valproate (VPA) is considered the first-choice drug. In women of childbearing potential, levetiracetam (LEV) should represent the first-choice treatment. Alternative monotherapy or add-on therapy should be considered in subjects with resistant seizures after the exclusion of pseudo-drug resistance. The choice of the add-on ASM depends on the predominant seizure type. In subjects with persistent bilateral tonic-clonic seizures, LEV or lamotrigine should be firstly considered. In patients with difficult-to-treat myoclonic seizures, clonazepam or LEV are recommended. In case of persistent absences, ethosuximide should be considered. With appropriate selection and safeguards in place, VPA should remain available as an option in women of childbearing potential whose seizures are resistant to other treatments.
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Epilepsia Mioclónica Juvenil , Preparaciones Farmacéuticas , Anticonvulsivantes/uso terapéutico , Femenino , Humanos , Levetiracetam/uso terapéutico , Epilepsia Mioclónica Juvenil/diagnóstico , Epilepsia Mioclónica Juvenil/tratamiento farmacológico , Ácido Valproico/uso terapéuticoRESUMEN
Introduction: Status epilepticus (SE) is a neurologic and medical emergency with significant related morbidity and mortality. Hepatic or renal dysfunction can considerably affect the pharmacokinetics of drugs used for SE through a variety of direct or indirect mechanisms.Areas Covered: This review aims to focus on the therapeutic management of SE in patients with hepatic or renal impairment, highlighting drugs' selection and dose changes that may be necessary due to altered drug metabolism and excretion. The references for this review were identified by searches of PubMed and Google Scholar until May 2020.Expert opinion: According to literature evidence and clinical experience, in patients with renal disease, the authors suggest considering lorazepam as the drug of choice in pre-hospital and intra-hospital early-stage SE, phenytoin in definite SE, propofol in refractory or super-refractory SE. In patients with liver disease, the authors suggest the use of lorazepam as drug of choice in pre-hospital and intra-hospital early-stage SE, lacosamide in definite SE, propofol in refractory or super-refractory SE. A list of preferred drugs for all SE stages is provided.
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Enfermedades Renales , Estado Epiléptico , Anticonvulsivantes/uso terapéutico , Humanos , Enfermedades Renales/complicaciones , Enfermedades Renales/tratamiento farmacológico , Hígado , Fenitoína/uso terapéutico , Estado Epiléptico/tratamiento farmacológicoRESUMEN
PURPOSE: Status epilepticus (SE) is associated with high morbidity and mortality. This multicenter retrospective cohort study aims to identify the factors associated with the occurrence of SE and the predictors of its recurrence in patients with adult-onset seizures. METHODS: We retrospectively analyzed data of 1115 patients with seizure onset>18 years, observed from 1983 to 2020 in 7 Italian Centers (median follow-up 2.1 years). Data were collected from the databases of the Centers. Patients with SE were consecutively recruited, and patients without SE history were randomly selected in a 2:1 ratio. To assess determinants of SE, different clinical-demographic variables were evaluated and included in univariate and multivariate logistic regression model. RESULTS: Three hundred forty-seven patients had a SE history, whereas the remaining 768 patients had either isolated seizures or epilepsy without SE history. The occurrence of SE was independently associated with increasing age at onset of disease (OR 1.02, 95% CI 1.01--1.03, p<0.001), female sex (OR 1.39, 95% CI 1.05--1.83, p=0.02) and known etiology (OR 3.58, 95% CI 2.61--4.93, p<0.001). SE recurred in 21% of patients with adult-onset SE and recurrence was associated with increasing number of anti-seizure medications taken at last follow-up (OR 1.88, 95% CI 1.31--2.71, p<0.001). CONCLUSIONS: In patients with adult-onset seizures, SE occurrence is associated with known etiologies, advanced age and female sex. Patients with recurrent SE are likely to have a refractory epilepsy, deserving careful treatment to prevent potentially fatal events.
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Estado Epiléptico , Adulto , Estudios de Cohortes , Femenino , Estudios de Seguimiento , Humanos , Masculino , Estudios Retrospectivos , Convulsiones/epidemiología , Estado Epiléptico/diagnóstico , Estado Epiléptico/epidemiología , Estado Epiléptico/etiologíaRESUMEN
OBJECTIVE: The clinical and neurophysiological characteristics of myoclonus in Angelman syndrome (AS) have been evaluated in single case or small cohorts, with contrasting results. We evaluated the features of myoclonus in a wide cohort of AS patients. METHODS: We performed polygraphic EEG-EMG recording in 24 patients with genetically confirmed AS and myoclonus. Neurophysiological investigations included jerk-locked back-averaging (JLBA), cortico-muscular coherence (CMC) and generalised partial directed coherence (GPDC). CMC and GPDC analyses were compared to those obtained from 10 healthy controls (HC). RESULTS: Twenty-four patients (aged 3-35â¯years, median 20) were evaluated. Sequences of quasi-continuous rhythmic jerks mostly occurred at alpha frequency or just below (mean 8.4⯱â¯1.4â¯Hz), without EEG correlate. JLBA did not show any clear transient preceding the jerks. CMC showed bilateral over-threshold CMC in alpha band that was prominent on the contralateral hemisphere in the patient group as compared to HC group. GPDC showed a significantly higher alpha outflow from both hemispheres toward activated muscles in the patient group, and a significantly higher beta outflow from contralateral hemisphere in the HC group. CONCLUSIONS: These neurophysiological findings suggest a subcortical generator of myoclonus in AS. SIGNIFICANCE: Myoclonus in AS has not a cortical origin as previously hypothesised.
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Ritmo alfa , Síndrome de Angelman/fisiopatología , Encéfalo/fisiopatología , Contracción Muscular , Mioclonía/fisiopatología , Adolescente , Adulto , Síndrome de Angelman/complicaciones , Niño , Preescolar , Femenino , Humanos , Masculino , Mioclonía/etiologíaRESUMEN
Stroke-like migraine attacks after radiation therapy (SMART) is a late-onset complication of cerebral irradiation, clinically characterized by headache, seizures and focal deficits. We describe two patients with SMART presenting with focal status epilepticus and headache. We believe that SMART is a misnomer that misjudge seizures among clinical features and we suggest to rename this entity as " Seizure with Migraine-like Attacks after Radiation Therapy". The new acronym, modified in its meaning but not in its form (SMART), better reflects the main clinical features and may allow neurologists to recognize this condition more easily.