COVID-19-associated immune-mediated encephalitis mimicking acute-onset Creutzfeldt-Jakob disease.

We report a subtype of immune-mediated encephalitis associated with COVID-19, which closely mimics acute-onset sporadic Creutzfeldt-Jakob disease. A 64-year-old man presented with confusion, aphasia, myoclonus, and a silent interstitial pneumonia. He tested positive for SARS-CoV-2.

Comorbidities in patients with epilepsy: Frequency, mechanisms and effects on long-term outcome.

Objective: To assess frequency, types, and mechanisms of comorbidities in people with epilepsy and verify their association with disease features and outcome.

Methods: This cohort study was performed in 13 Italian epilepsy centers with nationwide distribution and accurate records. Eligible patients were children and adults diagnosed before December 31, 2005, and followed for a minimum of 10 years.

Discontinuation of antiseizure medications in seizure-free patients with long-term follow-up: Patients' profile, seizure recurrence, and risk factors.

Objective: To compare withdrawal of antiseizure medications (ASM) to continued treatment in newly diagnosed individuals achieving seizure freedom, and assess the risk of relapse and factors associated with relapse.

Methods: This is a multicenter retrospective cohort study with long-term follow-up. Patients with newly diagnosed epilepsy were identified from the medical records of 13 Italian epilepsy centers and followed up until the most recent visit or death.

Prognostic patterns and predictors in epilepsy: a multicentre study (PRO-LONG).

Objectives: To describe the long-term prognosis of epilepsy and prognostic patterns in a large cohort of newly diagnosed patients and identify prognostic factors.

Methods: Study participants were 13 Italian epilepsy centres with accessible records dating back to 2005 or earlier, complete data on seizure outcome and treatments, precise epilepsy diagnosis, and follow-up of at least 10 years. Records were examined by trained neurology residents for demographics, seizure characteristics, neurological signs, psychiatric comorbidity, first electroencephalogram (EEG) and MRI/CT, epilepsy type and aetiology, antiepileptic drugs (AEDs), and 1-year, 2-year, 5-year and 10-year seizure remissions.

Neurologic outcome of postanoxic refractory status epilepticus after aggressive treatment.

Objective: To investigate neurologic outcome of patients with cardiac arrest with refractory status epilepticus (RSE) treated with a standardized aggressive protocol with antiepileptic drugs and anesthetics compared to patients with other EEG patterns.

Methods: In the prospective cohort study, 166 consecutive patients with cardiac arrest in coma were stratified according to 4 independent EEG patterns (benign, RSE, generalized periodic discharges [GPDs], malignant nonepileptiform) and multimodal prognostic indicators. Primary outcomes were survival and cerebral performance category (CPC) at 6 months.

Efficacy and safety of perampanel oral loading in postanoxic super-refractory status epilepticus: A pilot study.

Refractory nonconvulsive status epilepticus (NCSE) occurs in 10%-30% of patients following resuscitation after cardiac arrest. Both the optimal treatment and prognosis of postanoxic status epilepticus remain uncertain. We analyzed acute electroencephalographic changes, neurological outcome at 3 months, and adverse effects in consecutive postanoxic patients with super-refractory NCSE treated with add-on oral loading of perampanel.

Long-term applicability of the new ILAE definition of epilepsy. Results from the PRO-LONG study.

Objective: The new epilepsy definition adopted by the International League Against Epilepsy (ILAE) includes patients with one unprovoked seizure with a probability of further seizures, similar to the general recurrence risk after two unprovoked seizures, occurring in a 10-year period. Long-term follow-up of patients diagnosed after a single seizure is needed to assess the applicability of the new epilepsy definition in clinical practice.

Methods: Patients with newly diagnosed epilepsy were recruited retrospectively with a minimum follow-up of 10 years.

Risk factors for a first epileptic seizure symptomatic of brain tumour or brain vascular malformation. A case control study.

Principles: The risk of seizures increases in patients with brain tumours (BT) and brain vascular malformations (BVM), but not all risk factors are known. We aimed to identify factors that increase the risk of a first seizure in patients with BT or BVM.

Methods: Multicentre case-control study; 102 cases with a first seizure as a presenting symptom of BT or BVM; 121 hospital controls with BT or BVM, but without seizures, matched by centre, gender and age.

Early versus late remission in a cohort of patients with newly diagnosed epilepsy.

Purpose: To count patients with newly diagnosed epilepsy entering early and late remission and to identify prognostic predictors of late remission.

Methods: Children and adults with previously untreated epilepsy from two Italian tertiary centers (Monza, Bari) were the study population. All patients received monotherapy at treatment start; drug choice and schedule were left to the physician's judgment.

Risk factors for a first epileptic seizure after stroke: a case control study.

Introduction: The risk of seizures increases after stroke, but not all risk factors are known. We aimed to identify factors that increase the risk of a first seizure after a stroke.

Methods: Multicenter case-control study of 161 patients with a first seizure after stroke (105 provoked/acute and 56 unprovoked/remote symptomatic) matched to 279 hospital stroke controls by center, gender, age and timing of stroke.

Epileptogenic drugs: a systematic review.

A wide range of substances, including drugs and illicit compounds, increase the risk of epileptic seizures. In this systematic review, the authors address the issue of the epileptogenic potential of marketed drugs, with the aims of providing criteria for the assessment of the cause-effect relationship between drug exposure and the risk of seizures; and to identify the compounds better fulfilling the requirements of an epileptogenic drug. Finding a correlation between drug exposure and occurrence of seizures does not necessarily establish a causal association.

Fas-mediated T-cell apoptosis is impaired in patients with chronic inflammatory demyelinating polyneuropathy.

The Fas death receptor is expressed by activated lymphocytes and is involved in switching-off the immune response. Its inherited defects cause auto-immune lymphoproliferative syndrome. Impaired Fas function may also play a role in other auto-immune diseases, such as multiple sclerosis and type 1 diabetes mellitus.

Incidence and clinical features of acute inflammatory polyradiculoneuropathy in Lombardy, Italy, 1996.

Objective: To assess the annual incidence of typical Guillain-Barré syndrome (GBS) and its main variants (atypical GBS) in a well-defined population from a large area.

Material And Methods: A population-based prospective survey of GBS was undertaken during the calendar year 1996 in Lombardy, Italy (population 8,891,652). Typical and atypical GBS was diagnosed using the National Institutes of Neurological and Communicative Disorders and Stroke (NINCDS) and Ropper criteria.

Emotional and affective disturbances in patients with epilepsy.

We sought to assess whether epilepsy is associated with a higher risk of emotional reactions to frustrating stimuli, aggressive behavior, apathy, and depression, and whether these psychiatric patterns are specific to the epileptic condition. The study population consisted of referral patients 17 years and older with idiopathic or cryptogenic epilepsy (i.e.

Reliability of the El Escorial diagnostic criteria for amyotrophic lateral sclerosis.

Background: The El Escorial diagnostic criteria are the most commonly used in clinical studies and therapeutic trials in patients with amyotrophic lateral sclerosis (ALS). The accuracy of the El Escorial criteria was tested in clinical practice, but the reliability is unknown when the diagnosis of ALS must be assessed on the basis of medical records.

Objective: To assess the reliability of the El Escorial criteria for the diagnosis of ALS in different settings.