Publications by authors named "Didato G"
Neuronal ceroid lipofuscinoses (NCLs) are genetically heterogeneous neurodegenerative disorders, characterized by progressive cognitive and motor decline, epilepsy, visual impairment, and shortened life-expectancy. CLN6-related NCLs include both late-infantile and adult myoclonic form. We report a 21-year-old patient, with mild developmental delay, who developed occipital seizures at 14 years, and subsequently cognitive decline, cortical myoclonus, and photosensitivity at low and higher frequencies.
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- This case report emphasizes the need for comprehensive testing methods, including video-EEG, MEG, and MRI, to accurately diagnose negative motor seizures (NMSs) and differentiate them from other conditions.
- The study presents a 62-year-old patient with drug-resistant focal epilepsy and NMSs, highlighting the importance of monitoring that indicated specific brain activity and muscular responses during seizures.
- Findings from the multimodal evaluations revealed an epileptic zone in the right opercular region, associated with brain abnormalities, thereby improving our understanding of NMSs and their underlying brain mechanisms.
Article Synopsis
- The IRDiRC Telehealth Task Force investigated how telehealth can enhance diagnosis, care, research, and education related to rare diseases by reviewing literature from 2017 to 2023 and identifying effective models and strategies.
- The COVID-19 pandemic significantly boosted the adoption of telehealth, highlighting its ability to overcome geographical barriers and improve access to specialized care for rare disease patients.
- While telehealth presents several benefits, such as decentralized clinical research and enhanced education for local healthcare providers, it also has limitations, including challenges in performing physical exams and building personal relationships with healthcare professionals, thus suggesting it should complement traditional care rather than replace it.
Objective: Benchmarking has been proposed to reflect surgical quality and represents the highest standard reference values for desirable results. We sought to determine benchmark outcomes in patients after surgery for drug-resistant mesial temporal lobe epilepsy (MTLE).
Methods: This retrospective multicenter study included patients who underwent MTLE surgery at 19 expert centers on five continents.
Objective: This study aimed to explore the effectiveness of brivaracetam (BRV) according to baseline seizure frequency and past treatment history in subjects with focal epilepsy who were included in the Brivaracetam Add-On First Italian Network Study (BRIVAFIRST).
Methods: BRIVAFIRST was a 12-month retrospective, multicenter study including adults prescribed adjunctive BRV. Study outcomes included sustained seizure response (SSR), sustained seizure freedom (SSF), and the rates of treatment discontinuation and adverse events (AEs).
Background And Objectives: To report the progressive introduction of the exoscope (EX) from surface lesionectomy to antero-mesial temporal lobectomy (AMTL) in an epilepsy surgery practice.
Methods: We describe a population of ten consecutive patients undergoing EX surgery, with a minimum follow-up of 6 months, that was compared to a similar population of patients referred to operative microscopic surgery (OM).
Results: All surgeries were performed with the use of EX or OM alone.
Background: Sleep abnormalities have been reported in Charcot-Marie-Tooth disease (CMT), but data are scanty. We investigated their presence and correlation in a large CMT patients' series.
Methods: Epworth Sleepiness Scale (ESS) and Pittsburgh Sleep Quality Index (PSQI) were administered to CMT patients of the Italian registry and controls.
Objective: Encephaloceles (ENCs) may cause clinical complications, including drug-resistant epilepsy that can be cured with epilepsy surgery.
Methods: We describe clinical, diagnostic, and neuropathological findings of 12 patients with temporal ENC and epilepsy evaluated for surgery and compare them with a control group of 26 temporal lobe epilepsy (TLE) patients.
Results: Six patients had unilateral and 6 bilateral temporal ENCs.
Introduction: In randomized controlled trials, add-on brivaracetam (BRV) reduced seizure frequency in patients with drug-resistant focal epilepsy. Most real-world research on BRV has focused on refractory epilepsy. The aim of this analysis was to assess the 12-month effectiveness and tolerability of adjunctive BRV when used as early or late adjunctive treatment in patients included in the BRIVAracetam add-on First Italian netwoRk Study (BRIVAFIRST).
View Article and Find Full Text PDFBackground: The management of epilepsy in older adults has become part of daily practice because of an aging population. Older patients with epilepsy represent a distinct and more vulnerable clinical group as compared with younger patients, and they are generally under-represented in randomized placebo-controlled trials. Real-world studies can therefore be a useful complement to characterize the drug's profile.
View Article and Find Full Text PDFObjective: Post-stroke epilepsy (PSE) is one of the most common causes of acquired epilepsy and accounts for about 10-15% of all newly diagnosed epilepsy cases. However, evidence about the clinical profile of antiseizure medications in the PSE setting is currently limited. Brivaracetam (BRV) is a rationally developed compound characterized by high-affinity binding to synaptic vesicle protein 2A.
View Article and Find Full Text PDFThe 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.
View Article and Find Full Text PDFBackground And Objectives: The identification of possible hippocampal alterations is a crucial point for the diagnosis and therapy of patients with unilateral temporal lobe epilepsy (TLE). This study aims to investigate the role of neurite orientation dispersion and density imaging (NODDI) compared to diffusion tensor imaging (DTI) in the comprehension of hippocampal microstructure in TLE.
Methods: DTI and NODDI metrics were calculated in the hippocampi of adult patients with TLE, with and without histology-confirmed hippocampal sclerosis (HS), and in age/sex-matched healthy controls (HC).
Article Synopsis
- Late-onset Rasmussen encephalitis (LoRE) is a rare neurological disorder characterized by progressive inflammation in one hemisphere, leading to neurological deficits and seizures.
- A study of 13 patients analyzed multiple MR images to observe changes like volume loss and abnormalities over time, revealing that the frontal and temporal lobes were most impacted.
- The study found milder atrophy in other regions and highlighted that changes in LoRE's MRI are less severe and progress more slowly compared to those seen in the childhood version of the disease.
Introduction: From the beginning of the COVID-19 pandemic, healthcare workers had to face unprecedented emergency needs associated with an extraordinary amount of psychological distress. In this cross-sectional multicenter study, we investigated sleep disturbances, and the level of anxiety and depression among the healthcare and non-healthcare staff of three hospitals in Milan (Italy) during the COVID-19 outbreak. Moreover, we explored potential predisposing factors for affective symptoms and poor sleep.
View Article and Find Full Text PDFOptimizing patient safety and quality improvement is increasingly important in surgery. Benchmarks and clinical quality registries are being developed to assess the best achievable results for several surgical procedures and reduce unwarranted variation between different centers. However, there is no clinical database from international centers for establishing standardized reference values of patients undergoing surgery for mesial temporal lobe epilepsy.
View Article and Find Full Text PDFObjective: We use co-registration of foramen-ovale and scalp-EEG to investigate network alterations in temporal-lobe epilepsy during focal seizures without (aura) or with impairment of awareness (SIA).
Methods: One aura and one SIA were selected from six patients. Temporal dynamic among 4 epochs, as well as the differences between aura and SIA, were analyzed through partial directed coherence and graph theory-based indices of centrality.