Welcome to the WCN 2021 Interactive Program
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- Umberto Aguglia (Italy)
USEFULNESS OF 24-HOUR AMBULATORY EEG MONITORING IN THE DIAGNOSIS OF TYPICAL ABSENCES
- Alessia Giugno (Italy)
Abstract
Background and Aims:
Voluntary hyperventilation (VH) is believed to elicit successfully typical absences (TAs), especially in patients with genetic generalized epilepsies (GGE) and absences, including childhood and juvenile absence epilepsy (CAE and JAE) respectively. Failure in recording TAs may lead to diagnostic and therapeutic difficulties. This study evaluated the diagnostic yield of 24-hour ambulatory EEG monitoring (EEG/DIN), compared with VH, in patients with suspected or definite TAs/GGE and possible influence of clinical factors on HV efficacy.
Methods:
108 consecutive individuals (53 women, mean age 12.6±5.4 years), clinically suspected of TAs, underwent EEG recording with VH for 4-5 min of maximal effort from the subject, with monitoring of respiratory excursions by EEG technologist. If standard EEG was uninformative, they were investigated with EEG/DIN.
Results:
Three distinct groups were defined on the basis of HV and EEG/DIN findings: -ì. In 61/108 (56%) subjects (41/61 with CAE, 20/61 with JAE, 43/61 on antiepileptic drugs [AEDs]), HV triggered TAs. -ii. In 36/108 (34%) individuals (17/36 with CAE, 19/36 with JAE; and 26/36 on AEDs), HV was unsuccessful, but EEG/DIN showed TAs or electrophysiological hallmarks of them. -iii. The remaining 11/108 patients had normal EEG with HV and EEG/DIN; the diagnosis of TAs/GGE was ruled out in 9/11 of them. Thereafter, AED was discontinued in 2/4 individuals on therapy.
Conclusions:
This study illustrated a higher diagnostic yield of EEG/DIN than HV in individuals with TAs/GGE. HV was less effective in JAE, regardless of AED therapy. Thus, EEG/DIN greatly helps establish the diagnosis and monitor TAs, especially in JAE.
SIALIDOSIS TYPE 1: LONG-TERM CARE OF TWO UNRELATED PATIENTS AND EFFECTIVENESS OF LOW DOSES OF PERAMPANEL
- Claudia Cuccurullo (Italy)
Abstract
Background and Aims:
Sialidosis type 1 is a rare autosomal recessive disease, considered among the progressive myoclonous epilepsies (PME) and characterized by cortical myoclonous, seizures, ataxia, macular cherry-red spot and visual defects1. Recent clinical studies show a significative effectiveness of Perampanel (PER) on myoclonous and seizures in some cases of PME2,3. We describe a comprehensive clinical, neuroradiological, electrophysiological and ophtalmological long-term follow-up of two unrelated patients affected by Sialidosis type 1. Furthermore, we assess the effectiveness on myoclonous and tolerability of PER in add-on.
Methods:
two patients affected by Sialidosis type 1 underwent to brain MRI, fundus oculi, optical-coherence-tomography (OCT), video-EEG-polygraphy, somatosensory-evoked-potentials (SEPs). PER was added onto the tratment. Myoclonous severity has been evaluated by Unified-Myoclonous-Rating-Scale (UMRS) at baseline, after 3 and 6 months from PER introduction, and by neurophysiological investigations.
Results:
MRI showed cerebellar atrophy, ophtalmological exams found out macular cherry-red spot bilaterally and Bergmeister’s papilla, neurophysiological investigations revealed cortical myoclonous, confirmed by JL-BackAVG analysis, a giant SEP and the presence of C-reflex. The UMRS-score was 144(Pt1) and 102(Pt2) at baseline, 100(Pt1) and 50(Pt2) after 3 months. None referred adverse effets nor seizures.
Conclusions:
we believe that a comprehensive evaluation is essential to help shorten the time lag to genetic diagnosis and subsequently assist these patients the most. Myoclonus, together with ataxia, led to a severe disability, resulting in loss of self-sufficiency within a few years from onset. Our patients benefited from PER add-on, which proved to be effective and safe even at doses as low as 4 mg/day.
COGNITIVE DECLINE RISK STRATIFICATION IN PEOPLE WITH LATE-ONSET EPILEPSY OF UNKNOWN ETIOLOGY: AN ELECTROENCEPHALOGRAPHIC CONNECTIVITY AND GRAPH THEORY PILOT STUDY
- Cinzia Costa (Italy)
Abstract
Background and Aims:
Although people with late onset epilepsy of unknown etiology (LOEU) are at higher risk of cognitive decline compared to the general population, we still lack affordable tools to predict and stratify their risk of dementia. Electroencephalography (EEG) network small-world (SW) analysis has been proven cost-effective, feasible and reliable in predicting cognitive decline in mild cognitive impairment (MCI). This pilot-study investigates the potential application of EEG network SW properties in predicting cognitive decline among patients with LOEU.
Methods:
People diagnosed with LOEU and normal cognitive examination at the time of epilepsy diagnosis were included. Cerebrospinal fluid biomarkers, brain imaging and neuropsychological assessment were performed at the time of epilepsy diagnosis. Baseline EEG was analyzed for SW properties. Patients were followed-up over time with neuropsychological tests.
Results:
Over 5.1 years of follow-up, among 24 patients diagnosed with LOEU, thirteen developed MCI, and four dementia. Patients with LOEU developing MCI had lower values of SW coefficients in the in the delta (p=.03) band and higher SW values in the alpha frequency bands (p=.02) compared to patients having normal cognition at last follow-up. A similar gradient was confirmed for patients developing dementia compared to those with normal cognitive function as well as to those developing MCI.
Conclusions:
EEG network SW properties might be able to identify people with LOEU at risk of cognitive decline. Given EEG is frequently performed in LOEU. Baseline EEG analysis through SW is worth investigating as an affordable, widely available tool to stratify people for their risk of cognitive decline.
QUANTITATIVE ELECTROENCEPHALOGRAPHY (QEEG) MODIFICATIONS DURING OVARIAN CYCLE IN PATIENTS WITH CATAMENIAL C1-PATTERN TEMPORAL LOBE EPILEPSY (TLE)
- Fedele Dono (Italy)
Abstract
Background and Aims:
The term ‘‘catamenial epilepsy’’ refers to cyclic seizure exacerbation in relation to the ovarian cycle. There are three recognized patterns: perimenstrual (C1), peri-ovulatory (C2) and entire luteal phase in anovulatory cycles (C3). Temporal lobe can define a direct influence on the function of the hypothalamic-pituitary axis which is involved in the production of sex steroids. Vice-versa, sex steroids can modulate brain activity. The aim of this study is to evaluate quantitative electroencephalography (qEEG) modifications during ovarian cycle in patients with catamenial C1-pattern temporal lobe epilepsy (TLE).
Methods:
Thirty-five women with diagnosis of TLE were enrolled. According to the presence of catamenial C1-pattern, patients were divided into 2 subgroups: catamenial TLE (17 patients), non-catamenial TLE (18 patients). Each patient underwent a 30-minute EEG recording during menstrual, follicular and luteal phase of the ovarian cycle. A total of 90 epochs of 2 s-long per patient were processed. The mean power spectrum (MPS) was divided automatically into four frequency bands: delta (1-3.9 Hz), theta (4-7.9 Hz), alpha (8-12 Hz) and beta (13-18 Hz).
Results:
During menstrual phase, patients with catamenial TLE showed a significantly lower alpha MPS and a significantly higher theta MPS in temporal derivations, as compared to follicular and luteal phases
Conclusions:
Reduction of alpha rhythm during M phase may account for seizure exacerbations in catamenial TLE patients. This specific qEEG pattern could represent a biomarker of C1 pattern, supporting diagnosis and aiming to differentiate it from other catamenial patterns.
EMERGENCY DEPARTMENT PRIORITIZATION OF TRANSIENT LOSS OF CONSCIOUSNESS DUE TO EPILEPTIC SEIZURES
- Bruna Nucera (Italy)
Abstract
Background and Aims:
To evaluate the Manchester Triage System (MTS) in prioritizing patients presenting to the Emergency Department(ED) with non-traumatic transient loss of consciousness(TLOC) secondary to epileptic seizures(ES) and the concordance in the diagnosis of ES between medical and nursing staff in the ED.
Methods:
Single-center observational retrospective study. We dichotomized MTS codes into low(codes blue, green, yellow) and high priority(codes orange, red). We compared the association of MTS priority classification codes with a final diagnosis of a TLOC due to ES, calculating the sensitivity(proportion of TLOC due to ES categorized with high priority), specificity(proportion of TLOC unrelated to ES categorized with low priority) and accuracy. We also evaluated the inter-rater agreement of ED nurses and physicians in the evaluation of clinical features suspicious of ES and in the diagnosis of ES.
Results:
Over 30 months, 2291 triage evaluations on TLOC were included, of which 201(8.8%) due to ES. In the overall population: sensitivity 43.2%, specificity 85.5%, accuracy 81.8%; excluding cardiogenic syncope: sensitivity 43.2%, specificity 87.9%, accuracy, 83.7%;excluding pre-syncope: sensitivity 43.1%, specificity 84.2%, accuracy 79.1%; including only alert and responsive patients: sensitivity 74.1%, specificity 86.6%, accuracy 82.8%. Agreement in the evaluation of clinical features suspicious of ES between nurses and physicians: 99.4%(Kappa Cohen 0.96; p <0.001); agreement in the final diagnosis of ES: 99.5%(Kappa Cohen 0.97; p <0.001).
Conclusions:
MTS adequately categorizes TLOC unrelated to ES, but is not equally effective in prioritizing TLOC due to ES. There is high agreement between ED nurses and physicians in evaluation and final diagnosis of TLOC due to ES.
A MULTIMODAL NEUROIMAGING APPROACH TO NON LESIONAL FRONTAL LOBE EPILEPSY
- Pio Zoleo (Italy)
Abstract
Background and Aims:
Frontal Lobe Epilepsy (FLE) is a common form of epilepsy usually caused by structural lesions on frontal areas. Some FLE patients are defined “non-lesional” (nlFLE) because of the absence of clearly identifiable abnormalities on qualitative brain MRI. Our aim was to investigate nlFLE patients to identify possible microstructural abnormalities, through advanced neuroimaging techniques.
Methods:
We enrolled 127 nlFLE patients and 127 age and sex-matched HC. Diagnosis of nfFLE was based on typical ictal semeiology and interictal frontal EEG discharges; all the study participants underwent 3T brain MRI. Voxel-based morphometry (VBM), cortical-thickness (CT), Diffusion-tensor imaging (DTI) and Tract-based spatial statistics (TBSS) were the whole-brain multimodal MRI analyzes performed. We also focused on the corpus callosum (CC), evaluating thickness, fractional anisotropy (FA) and mean diffusivity (MD) from 50 regions of interest along the callosal midsagittal profile.
Results:
VBM analysis revealed regional atrophy in rolandic operculum in nlFLE patients compared to HC (p-value<0.05, TFCE-corrected). TBSS analysis showed significantly increased callosal FA and MD (p-value<0.05, TFCE-corrected) in nlFLE patients compared with controls, especially in sections I (rostrum, genu and rostral body) and III (posterior midbody) of the CC.
Conclusions:
Our study demonstrates that subtle MRI functional and morphological anomalies exist in FLE patients who did not show qualitative brain alterations, and that corpus callosum is one of the most involved cerebral structures. Our results possibly suggest a contribution from white matter alterations to the network abnormalities sustaining frontal lobe seizures, further questioning the traditional concept of epilepsy as a “cortical–only disease”.
IN JME PATIENTS, PROLONGED AND SHORT EPILEPTIFORM DISCHARGES HAVE AN OPPOSITE RELATIONSHIP WITH THE SLEEP-WAKE CYCLE.
- Francesco Turco (Italy)
Abstract
Background and Aims:
We recently demonstrated that the recording of epileptiform discharges (EDs) longer than 2,68 seconds with prolonged EEG, in Juvenile Myoclonic patients predict motor seizure recurrence. The aim of this study is to verify whether the longer and shorter EDs have a different distribution in relation to the sleep-wake cycle.
Methods:
We further analyzed the 32 EEG recordings of our work, studying the temporal distribution of EDs longer and shorter than 2.68 seconds. By applying a mixed-effects Poisson regression model (subject = random factor), we tested whether the frequency of longer or shorter EDs: 1) depends on the "time of day" parameter 2), is greater in wakefulness or sleep, 3) is maximum upon awakening or at the sleep onset.
Results:
EDs longer than 2.68 seconds, but not shorter ones, peak at 7 AM (p = 0.022). Regarding the sleep-wake cycle, longer EDs are more frequent in wakefulness (p <0.001), shorter ones in sleep (p <0.001); the frequency of EDs longer than 2.68 seconds is maximum in the hour following morning or afternoon awakening (p <0.001), when the occurrence of short epileptiform discharges is significantly less likely (p <0.001), when compared to the others hours of the day.
Conclusions:
Prolonged EDs, which predict seizure recurrence, have a circadian distribution and a dependence on the sleep-wake cycle opposite to the shorter ones. Their peak after awakenings reflects the characteristic temporal distribution of seizures in Juvenile Myoclonic Epilepsy. This simple observation may guide future studies aimed at resolving the intricate relationship between epileptiform discharge and seizures.
CORRELATES OF PSYCHOLOGICAL DISTRESS IN EPILEPTIC PATIENTS DURING THE COVID-19 OUTBREAK
- ALFONSO GIORDANO (Italy)
Abstract
Background and Aims:
Following the severe consequences of the coronavirus disease 2019 (COVID-19) outbreak, on March 9th, 2020 the Italian Government implemented extraordinary measures to limit viral transmission, including restrictive quarantine measures. Psychological distress represents the seizureprecipitating factor most often reported by patients with epilepsy. To date, no studies have analyzed the role played by the different dimensions of psychological distress quarantine-induced in patients with epilepsy.
Methods:
We included a total of 40 patients, 18 suffered from generalized, and 22 from focal epilepsy. The patients previously seen in the outpatient clinic during the pre-lockdown period between January and February 2020 were reevaluated after the lockdown period. Psychological distress was evaluated by using the three subscales of Impact of Event Scale-Revised (IES-R). Finally, we employed logistic regression analyses to explore the demographic and clinical features associated to high scores on IES-R.
Results:
Patients with higher scores on IES-R Intrusion and IES-R Avoidance subscales demonstrated an increased number of epileptic attacks compared to prelockdown period. Multivariate logistic regression analyses showed that a specific subgroup of patients (i.e., older, female with more anxious symptoms) are at higher risk of increased seizure frequency
Conclusions:
Our study confirmed that the frequency of epileptic seizures increased during lockdown when compared to pre-lockdown period. The early identification of patients more vulnerable to worsening is crucial to limit the risk of requiring hospital or clinical treatment during the COVID-19 outbreak