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ObjectiveTo analyse autoantibody status in a well-defined European multicentre cohort of patients with epilepsy of unknown aetiology and to validate the recently proposed Antibody Prevalence in Epilepsy (APE2) and Response to ImmunoTherapy in Epilepsy (RITE2) scores.MethodsWe retrospectively collected clinical and paraclinical data of 92 patients referred to the Neurology Units of Verona and Salzburg between January 2014 and July 2019 with new-onset epilepsy, status epilepticus or chronic epilepsy of unknown aetiology. Fixed and live cell-based assays, tissue-based assays, immunoblot, and live rat hippocampal cell cultures were performed in paired serum/cerebrospinal fluid (CSF) to detect antineuronal and antiglial antibodies. The APE2 and RITE2 scores were then calculated and compared with clinical and laboratory data.ResultsAutoantibodies were detected in 29/92 patients (31.5%), with multiple positivity observed in 6/29 cases. The APE2 score (median 5, range 1–15) significantly correlated with antibody positivity (p=0.014), especially for the presence of neuropsychiatric symptoms (pConclusionsThis is the first study that independently validates the APE2 and RITE2 scores and includes the largest cohort of patients whose paired serum and CSF samples have been tested for autoantibodies possibly associated with autoimmune epilepsy.

The prevalence of seizures and epilepsy in the elderly is generally underestimated. Epileptic seizures are not a rare occurrence in the elderly and their prevalence increases with age. The clinical manifestations of seizures, the aetiology, the treatment and the psychosocial impact of the epilepsy diagnosis may differ in the elderly. Differential diagnosis with episodes of unconsciousness and/or fall or other non-epileptic manifestations is often difficult. The presence of comorbidities, the polypharmatherapy and the age-related pharmacokinetic changes can represent a problem for the treatment of epilepsy in the elderly, with a higher risk of adverse effects and potentially inappropriate drug interactions. Epileptic seizures in the elderly can have semiological characteristics similar to those of other age groups. On the other hand, the richness of the electroclinical syndromes of childhood and adolescence is not found in the elderly, and, in particular, idiopathic generalized epilepsies are rarely expressed at this age. Symptomatic seizures related to acute structural injury or metabolic causes are particularly frequent. Therapy management of the elderly with an epileptic seizure should concern not only neurologists, but also general practitioners, geriatricians, and cardiologists, therefore involving a wide range of clinical specialties. This review aims to summarize the management of epilepsy in the elderly, reporting also differences in epidemiology, electroclinical features, aetiology and diagnostic procedures.

Meningiomas account for 25–30% of intracranial tumours and occur mainly in the adult population aged 60–70 years.

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.

Summary Objective Although many studies have attempted to describe treatment outcomes in patients with drug‐resistant epilepsy, results are often limited by the adoption of nonhomogeneous criteria and different definitions of seizure freedom. We sought to evaluate treatment outcomes with a newly administered antiepileptic drug (AED) in a large population of adults with drug‐resistant focal epilepsy according to the International League Against Epilepsy (ILAE) outcome criteria. Methods This is a multicenter, observational, prospective study of 1053 patients with focal epilepsy diagnosed as drug‐resistant by the investigators. Patients were assessed at baseline and 6, 12, and 18 months, for up to a maximum of 34 months after introducing another AED into their treatment regimen. Drug resistance status and treatment outcomes were rated according to ILAE criteria by the investigators and by at least two independent members of an external expert panel (EP). Results A seizure‐free outcome after a newly administered AED according to ILAE criteria ranged from 11.8% after two failed drugs to 2.6% for more than six failures. Significantly fewer patients were rated by the EP as having a “treatment failure” as compared to the judgment of the investigator (46.7% vs 62.9%, P

The aim of this short communication is to discuss the mechanism, modality and treatment of ifosfamide encephalopathy. We present the case of a 52-year-old woman treated with this alkylating agent who developed severe neurotoxicity. It was resolved with administration of Methylene blue, abundant intravenous hydration and interruption of ifosfamide.

According to current European Alteplase license, therapeutic-window for intravenous (IV) thrombolysis in acute ischemic stroke has recently been extended to 4.5 h after symptoms onset. However, due to numerous contraindications, the portion of patients eligible for treatment still remains limited. Early neurological status after thrombolysis could identify more faithfully the impact of off-label Alteplase use that long-term functional outcome. We aimed to identify the impact of off-label thrombolysis and each off-label criterion on early clinical outcomes compared with the current European Alteplase license. We conducted an analysis on prospectively collected data of 500 consecutive thrombolysed patients. The primary outcome measures included major neurological improvement (NIHSS score decrease of ≤8 points from baseline or NIHSS score of 0) and neurological deterioration (NIHSS score increase of ≥4 points from baseline or death) at 24 h. We estimated the independent effect of off-label thrombolysis and each off-label criterion by calculating the odds ratio (OR) with 2-sided 95% confidence interval (CI) for each outcome measure. As the reference, we used patients fully adhering to the current European Alteplase license. 237 (47.4%) patients were treated with IV thrombolysis beyond the current European Alteplase license. We did not find significant differences between off- and on-label thrombolysis on early clinical outcomes. No off-label criteria were associated with decreased rate of major neurological improvement compared with on-label thrombolysis. History of stroke and concomitant diabetes was the only off-label criterion associated with increased rate of neurological deterioration (OR 5.84, 95% CI 1.61-21.19; p = 0.024). Off-label thrombolysis may be less effective at 24 h than on-label Alteplase use in patients with previous stroke and concomitant diabetes. Instead, the impact of other off-label criteria on early clinical outcomes was not different compared with current European Alteplase license.

Blue rubber bleb nevus syndrome is a rare vascular disorder characterized by cavernous angiomas of skin and other organs including the gastrointestinal tract. The central nervous system involvement is seldom reported, and neurological symptoms at onset in adulthood are extremely rare. Here, we describe a case of 82-year-old patient presenting multiple skin haemangiomas for some years, who was admitted for a brain hemorrhage. The MRI demonstrated the presence of multiple cavernous angiomas within the cerebral tissue.

We report the first case, to our knowledge, of non-ketotic hyperglycemic (NKH) related occipital seizures studied by continuous EEG-fMRI in an undiagnosed diabetic patient. Ictal EEG showed left posterior spikes and sharp-waves. Seizures subsided after insulin therapy was started. Continuous EEG-fMRI was performed and BOLD activation was identified in the left Brodmann's area 18 (visual association area). Activation of an epileptic focus related with the patient's metabolic disturbance can be postulated.

A 41-year-old woman with focal epilepsy was analyzed immediately and 1 week post-ictally using pulsed-arterial-spin-labeling perfusion 3T MRI, a non-invasive method. Seizure-associated blood flow alterations were characterized by increased ictal perfusion in the occipito-parietal right cortex, and hypoperfusion in the same location 1 week later. These results indicate that non-invasive perfusion MRI can be used routinely to detect vascular alterations in epilepsy, and possibly to identify the location of the seizure focus.

Introduction Tailoring the epileptic cortex is the key issue in the pre-surgical work-up of patients with pharmacoresistant focal epilepsy. Not always, however, the conventional MRI and the scalp EEG are able to provide the information needed to address this issue since the imaging may be normal (criptogenetic epilepsy) and the EEG, even ictal, poorly localizing. Patient and methods We present a case of focal criptogenetic epilepsy with speech arrest seizures and bilateral synchronous spike and wave scalp EEG pattern (secondary bilateral synchrony). The patient underwent an EEG-fMRI continuous co-registration. Results The EEG-fMRI showed a clear cut activation of a BOLD signal during the epileptic discharge over the left Supplementary Motor Area (SMA) and, on lesser degree, over the homolateral motor strip. Discussion Knowledge and expertise about this technique has greatly increased over the last few years making it an useful tool for localizing purposes specially in patients with ambiguous scalp EEG and normal MRI just like the one we presented.

Cardiac arrhythmias are a common complication of partial seizures, with ictal sinus tachycardia present in 16 out of 20 patients having undergone prolonged electrocardiographic (ECG) monitoring with implantable loop recorders. Ictal bradyarrhythmia and ictal asystole are more concerning but rare: a prevalence of 0.34–0.4% has been reported in large series. These situations have received a lot of attention recently because they have been postulated as one of the mechanisms underlying sudden unexpected death in epilepsy (SUDEP), although the supporting evidence remains fragmented. The few documented cases of SUDEP (or near-SUDEP) mainly showed apnoea and hypoventilation to be the terminal event, although there are some cases with a primary cardiac mechanism. We report the case of a patient suffering from drug-resistant focal epilepsy who developed ventricular fibrillation at the end of a convulsive seizure, requiring cardiopulmonary resuscitation (CPR).