Identifying risk for status epilepticus with the ADAN scale: a prospective multicenter validation study.

Objectives: Status epilepticus (SE) is a serious event associated with high mortality. This study aims to validate the recently developed ADAN (Abnormal speech, ocular Deviation, Automatisms, and Number of motor epileptic seizures) scale for detecting high risk for SE.

Material And Methods: Prospective, multicenter, observational study in adults with suspected epileptic seizures.

Exploratory study of blood biomarkers in patients with post-stroke epilepsy.

Introduction: In addition to clinical factors, blood-based biomarkers can provide useful information on the risk of developing post-stroke epilepsy (PSE). Our aim was to identify serum biomarkers at stroke onset that could contribute to predicting patients at higher risk of PSE.

Patients And Methods: From a previous study in which 895 acute stroke patients were followed-up, 51 patients developed PSE.

Epileptiform electroencephalogram discharges increase seizure recurrence risk in patients with acute symptomatic seizure due to a structural brain lesion.

Objective: The risk factors for seizure recurrence after acute symptomatic seizure due to a structural brain lesion are not well established. The aim of this study was to analyze possible associations between demographic, clinical, and electroencephalographic variables and epilepsy development in patients with acute symptomatic seizure due to an acute structural brain lesion.

Methods: We designed an observational prospective study of patients with acute symptomatic seizure due to an acute structural brain lesion (hemorrhagic stroke, ischemic stroke, traumatic brain injury, or meningoencephalitis) who underwent EEG during their initial admission between January 2015 and January 2020.

Anakinra and tocilizumab in the chronic phase of febrile infection-related epilepsy syndrome (FIRES): Effectiveness and safety from a case-series.

Purpose: There is scarce evidence of effective treatments for the chronic phase of Febrile infection-related epilepsy syndrome (FIRES). This study aimed to analyze the outcomes of treatment with anakinra and tocilizumab.

Methods: Retrospective study including patients receiving either anti-interleukin-1 (anti-IL-1, anakinra) or anti-IL-6 (tocilizumab) during the chronic phase of FIRES.

ERN GENTURIS clinical practice guidelines for the diagnosis, treatment, management and surveillance of people with schwannomatosis.

A Guideline Group (GG) was convened from multiple specialties and patients to develop the first comprehensive schwannomatosis guideline. The GG undertook thorough literature review and wrote recommendations for treatment and surveillance. A modified Delphi process was used to gain approval for recommendations which were further altered for maximal consensus.

Neurofibromatosis type 1 families with first-degree relatives harbouring distinct pathogenic variants. Genetic counselling and familial diagnosis: what should be offered?

Unlabelled: Neurofibromatosis type 1 (NF1) is an autosomal dominant disorder caused by pathogenic variants in Recently, testing has been included as a clinical criterion for NF1 diagnosis. Additionally, preconception genetic counselling in patients with NF1 focuses on a 50% risk of transmitting the familial variant as the risk of having a sporadic NF1 is considered the same as the general population.

Methods: 829 individuals, 583 NF1 sporadic cases and 246 patients with NF1 with documented family history, underwent genetic testing for NF1.

Revisiting the UK Genetic Severity Score for NF2: a proposal for the addition of a functional genetic component.

Background: Neurofibromatosis type 2 (NF2) is an autosomal dominant disorder characterised by the development of multiple schwannomas, especially on vestibular nerves, and meningiomas. The UK NF2 Genetic Severity Score (GSS) is useful to predict the progression of the disease from germline pathogenic variants, which allows the clinical follow-up and the genetic counselling offered to affected families to be optimised.

Methods: 52 Spanish patients were classified using the GSS, and patients' clinical severity was measured and compared between GSS groups.

Psychogenic nonepileptic seizures: The effect of accurate diagnosis on cognition.

Objective: Much remains to be elucidated about the cognitive profile of patients with psychogenic nonepileptic seizures (PNES) and about how this changes over time and compares to that of patients with epilepsy. The aim of this study was to study the neuropsychological profile of patients with PNES and an age-matched group of patients with temporal lobe epilepsy (TLE) during admission to a video electroencephalography monitoring unit (VEMU) and 1 year after discharge.

Methods: Patients diagnosed with PNES or TLE at a VEMU were prospectively recruited.

Clinical predictors of adverse events during continuous video-EEG monitoring in an epilepsy unit.

Patients admitted to epilepsy monitoring units (EMUs) for diagnostic and presurgical evaluation have an increased risk of seizure-related injury, particularly in the many cases in which medication is withdrawn. The purpose of this study was to assess the prevalence of adverse events (AEs) in this setting and to analyse associated clinical factors and costs. We evaluated consecutive patients admitted to an EMU at a tertiary care hospital over a 10-year period based on a descriptive, longitudinal study.

Correlation of blood biomarkers with early-onset seizures after an acute stroke event.

Introduction: Blood biomarkers have not been widely studied in stroke-related seizures. In this study, we aimed to describe clinical factors and biomarkers present during acute stroke and to analyze their association with early-onset seizures.

Methods: We retrospectively evaluated a panel of 14 blood biomarkers in 1115 patients with ischemic and hemorrhagic stroke.

Intravenous lacosamide (LCM) in status epilepticus (SE): Weight-adjusted dose and efficacy.

Background: Some studies suggest higher efficacy of lacosamide (LCM) in status epilepticus (SE) with higher loading doses; however, this weight-adjusted dose has not been evaluated.

Objective: The objective was to evaluate the relationship between loading weight-adjusted dose and efficacy of LCM in SE.

Methods: A group of patients with SE treated with LCM from Spanish hospitals was examined retrospectively.

Refractory status epilepticus: Impact of baseline comorbidity and usefulness of STESS and EMSE scoring systems in predicting mortality and functional outcome.

Purpose: Little has been published on the prognostic value of the Status Epilepticus Severity Score (STESS) or the Epidemiology-based Mortality score in Status Epilepticus (EMSE) in refractory status epilepticus (RSE). We sought to analyze the prognostic value of STESS and EMSE and the impact of baseline comorbidities in mortality and functional outcome in RSE.

Methods: We designed an observational retrospective study of patients diagnosed with RSE between August 2013 and September 2017.

Early Genetic Diagnosis of Neurofibromatosis Type 2 From Skin Plaque Plexiform Schwannomas in Childhood.

Importance: Neurofibromatosis type 2 (NF2) is a devastating genetic condition characterized by the development of multiple tumors of the nervous system. An early diagnosis of individuals with NF2 would facilitate treatment and reduction of disease impact because most severe effects of the disease do not usually develop before adolescence. Little attention has traditionally been paid to dermatological signs in NF2.

Diagnostic Yield of Routine Electroencephalography With Concurrent Video Recording in Detecting Interictal Epileptiform Discharges in Relation to Reasons for Request: A Prospective Study of 1,080 Video-Electroencephalograms.

Purpose: The aim of this study was to prospectively analyze the sensitivity and specificity of routine electroencephalography with concurrent video recording (vEEG) in relation to the reasons for requesting the test and to investigate when routine vEEG should be requested.

Methods: We prospectively analyzed 1,080 consecutive vEEGs performed between April 2015 and April 2016. The requests for vEEG were classified as requests with a low suspicion of epilepsy (syncope, confusion or delirium, suspicion of psychogenic nonepileptic seizures, and paroxysmal focal neurological deficit) or requests with a high suspicion of epilepsy (first clinical seizure, suspected status epilepticus, follow-up study of a patient with epilepsy, and acute symptomatic seizures).

Importance of neuropsychological and clinical features to predict seizure control in medically treated patients with mesial temporal epilepsy and hippocampal sclerosis.

Objective: It is not yet understood why seizures in certain patients with mesial temporal lobe epilepsy and hippocampal sclerosis (MTLE-HS) develop resistance to antiepileptic drugs (AEDs) while others achieve good seizure control with this treatment. We analyzed clinical and neuropsychological features associated with seizure control in patients with MTLE-HS who had not undergone resective surgery.

Methods: We enrolled 40 patients with medically treated MTLE-HS and retrospectively collected the following data from prospective databases: sex, febrile seizures, central nervous system infection, history of head trauma, cognitive impairment, psychiatric disturbances, history of status epilepticus, age at onset of epilepsy, aura, seizure type and frequency, electroencephalography abnormalities, HS side, AEDs, global cognitive status, and neuropsychological functions such as cognitive processing speed, attention and executive functions, verbal and visual memory, language, and visuospatial ability.

Multiple Intracranial Arteriovenous Fistulas in Cowden Syndrome.

Cowden syndrome is a rare autosomal dominant disease. It is characterized by multiple noncancerous tumorlike growths called hamartomas, which typically are found in the skin, oral mucosa, thyroid, breast, and gastrointestinal tract. It carries with it a potential risk of malignant transformation, especially of the breast and thyroid.

Hypoglycemic seizures and epilepsy in type I diabetes mellitus.

Purpose: (1) To determine the characteristics of seizures and/or epilepsy among patients with adult onset type 1 diabetes mellitus (T1DM), and (2) to determine glutamic acid decarboxylase antibody (antiGADab) titres and other autoimmune characteristics of T1DM patients with seizure and/or epilepsy.

Methods: Patients were recruited after reviewing the databases of one Diabetes Unit and two Epilepsy Units. Prevalence of suffering epilepsy and/or seizures was calculated in the group of adult onset T1DM patients seen consecutively in the Diabetes Unit.

Pilomotor seizures: an autonomic semiology of limbic encephalitis?

Purpose: Ictal piloerection is an infrequent seizure semiology that is commonly overlooked as an ictal epileptic manifestation. Piloerection is considered to be principally caused by temporal lobe activity although frontal and hypothalamic seizure origins have been reported. The described etiology has shown a wide variety of structural causes such as mesial temporal sclerosis, tumors, posttraumatic, cavernomas and cryptogenic epilepsies.

Review of therapeutic options for adjuvant treatment of focal seizures in epilepsy: focus on lacosamide.

Epilepsy is one of the most common serious neurological conditions worldwide, with an age-adjusted incidence of approximately 50 per 100,000 persons per year in developed countries. Antiepileptic therapy can result in long-term remission in 60-70% of patients, but many patients will require combination treatment to achieve optimal seizure control, as monotherapy is ineffective at controlling seizures in 30-53% of patients. Despite the increase in available treatment options, patient outcomes have not improved significantly and there is still a need for more effective therapies.

Seizure frequency and social outcome in drug resistant epilepsy patients who do not undergo epilepsy surgery.

Little is known about the long term prognosis of refractory epilepsy patients who do not undergo epilepsy surgery. We performed a telephone survey and chart review of patients who underwent presurgical evaluation in our Unit but did not have surgery, from 1998 until 2004. We contacted 84 patients; mean follow-up was 6.

Pregabalin as add-on therapy for refractory partial seizures in every day clinical practice.

We retrospectively reviewed our clinical experience with PGB when used as add-on therapy in 101 patients (56 women and 45 men) with refractory partial epilepsy, who have been followed up for at least 1 year. Mean age was 40 years (16-64); mean number of concomitant AEDs was 2.8.