Eyelid myoclonia with absences: Electroclinical features and prognostic factors.

OBJECTIVE: Eyelid myoclonia with absences (EMA) is a syndrome characterized by eyelid myoclonia with or without absences, eye closure-induced generalized electroencephalographic (EEG) paroxysms and photosensitivity. Few data are available about the prognostic factors of this syndrome. The main objectives of our study were to describe the clinical and EEG features of a group of patients with EMA and to evaluate the presence of prognostic factors. METHODS: We retrospectively selected a cohort of patients with diagnosis of EMA evaluated in the epilepsy service of the Neurological Clinic of Catania, in the Neurology and Clinical Neurophysiopathology Unit of Oasi Research Institute, Troina and in the Regional Epilepsy Centre of Bianchi-Melacrino-Morelli Hospital of Reggio Calabria. We considered the features of the patients during the first year of disease, and at the last follow-up visit. We stratified the patients into two groups: "seizure-free", defined as the absence of seizures for at least 2 years, and "not seizure-free" and we evaluated the evolution of their characteristics and the presence of factors associated with outcome. RESULTS: We enrolled 51 patients (40 women (78%); mean age: 30.8 years ± 15.5 [range 10-79]). The mean follow-up time was 8.7 ± 5.8 years. Eleven patients (21.6%) achieved the condition of seizure-free. Family history of epilepsy was associated with the condition of seizure-free (P = 0.05). At the last follow-up visit, EEG photosensitivity and eye closure sensitivity were significantly associated with the condition of "not seizure-free". SIGNIFICANCE: The results of our study revealed that a positive family history of epilepsy might be associated with a better outcome in EMA. Furthermore, the persistence of photosensitivity and eye closure sensitivity might indicate persistence of seizures, offering an aid in therapeutic management.

Abnormal visual sensitivity in eyelid myoclonia with absences: Evidence from electrocortical connectivity and non-linear quantitative analysis of EEG signal.

PURPOSE: Eyelid myoclonia with absences (EMA) is an epileptic syndrome characterized by eyelid myoclonia with or without absences, eyes closure-induced EEG paroxysms and photosensitivity. Pathophysiological mechanisms of visual sensitivity in EMA are not-fully understood. The objective of the present study was to analyze the electrophysiological dynamics implicated in the visual sensitivity in patients with EMA. METHODS: We analyzed data of 10 subjects with diagnosis of EMA and of 10 healthy control subjects. For both patients and controls, 4-seconds artifacts-free electroencephalographic signal epochs recorded were analyzed, during resting state, eyes-opened and eyes-closed tasks. Resting state networks in EEG have been computed using independent components analysis (ICA) LORETA. Moreover, the power law exponent ß was obtained for each coordinate as minus the slope of the power spectrum versus frequency in a Log-Log scale. RESULTS: Using LORETA ICA, patients during resting state showed significant differences as compared to controls with a reduction of the physiological alpha activity over the occipital lobe and of the physiological beta activity over the frontal lobe. Immediately after eye closure, a significant increase of beta activity over the frontal lobe was found in the group of patients compared to controls. Power law exponent ß analysis showed a significant increase of ß over the frontal regions in patients as compared to controls during resting-state and an increase of ß over the parieto-occipital regions after eye closure. CONCLUSION: Abnormal occipital and frontal cortex activities seem to be related with the visual sensitivity and eyelid myoclonia observed in patients with EMA.

Does continuous EEG influence prognosis in patients after cardiac arrest?

AIM: Electroencephalography (EEG) is a key modality for assessment of prognosis following cardiac arrest (CA); however, whether continuous EEG (cEEG) is superior to routine intermittent EEG (rEEG) remains debated. We examined the impact of cEEG (>18 h) vs. rEEG (<30 min) on outcome in comatose CA patients as part of multimodal prognostication. METHODS: We analysed a large prospective registry of comatose post-CA adults (n = 497; 2009-2018), stratified based on whether they received cEEG (n = 62) or rEEG (n = 435), including standardized reactivity testing at two time-points. The primary endpoint was the impact of cEEG vs. rEEG on Glasgow-Pittsburgh Cerebral Performance Categories (CPC) at three months; we also assessed impact on time to death. RESULTS: Main patients' baseline clinical characteristics and CPC scores were comparable between the EEG groups. By multivariable analysis age, non-shockable rhythm, presence of early myoclonus, absent EEG background reactivity, absent somato-sensory evoked potentials, and serum NSE were independently associated with poor neurological outcome (CPC 3-5), while the EEG approach had no impact on patient prognosis and time to death. CONCLUSIONS: Our data suggest that cEEG does not confer any advantage over intermittent rEEG regarding outcome in patients with CA, and does not influence the time to death.

Use of newer antiepileptic drugs and prognosis in adults with status epilepticus: Comparison between 2009 and 2017.

It is unclear whether the outcome of status epilepticus (SE), a neurological emergency associated with high mortality and morbidity, has changed over the past decade. We investigated the difference in prognosis (mortality, functional status at discharge) after adult SE episodes in a large registry between 2 time points (the years 2009 and 2017), exploring factors potentially associated with outcome, with particular attention to use of newer antiepileptic drugs (AEDs). Newer AEDs were more often prescribed in SE episodes in 2017 (80.2% vs 38.5%, P < .001); this independently correlated with year of observation, SE refractoriness, nonconvulsive SE forms, and number of AEDs. We observed a higher proportion of deterioration from baseline conditions in 2017 (67.7% vs 42.3%, P < .001), which was independently associated with, among other variables, use of newer AEDs (odds ratio = 2.91, 95% confidence interval = 1.13-7.48), whereas mortality seemed more stable over time (16.1% vs 6.3%, P = .08) without any relationship with newer AEDs. These observations suggest that newer AEDs might affect functional status but not mortality. Further investigations are necessary to improve therapeutic strategies, which currently rely on weak evidence.

Adult-Onset Sleepwalking Secondary to Hyperthyroidism: Polygraphic Evidence.

ABSTRACT: Sleepwalking is a disorder characterized by complex motor behaviors arising from slow wave sleep usually occurring in children. The adult onset of sleepwalking suggests the presence of an external precipitating factor leading to the occurrence of the disorder. Hyperthyroidism has been reported to be the possible cause of sleepwalking in a few cases. We present the case of a 36-year-old man who reported a sudden appearance of nocturnal episodes of sleepwalking. He underwent a complete video polysomnography (VPSG), which showed a polygraphic pattern arising from stage N3 sleep related to the presence of simple motor behaviors. Routine blood tests showed a mild hyperthyroidism. After 4 months of thyrostatic treatment, the patient reported no more sleepwalking events. A VPSG performed at the last follow-up showed the absence of pathological electroclinical events arising from stage N3 sleep. Therefore, we hypothesize that there is a link between sleepwalking and thyroid dysfunction in our patient.

Electroclinical findings of minor motor events during sleep in temporal lobe epilepsy.

OBJECTIVE: It is well known that sleep-related motor seizures can originate from the temporal lobe. However, little is known about the clinical features of minor motor manifestations during sleep in patients with temporal lobe epilepsy. The main objective of our study was to verify the existence of minor motor events during sleep in patients with mesial temporal lobe epilepsy (MTLE) and to define their clinical features and electroencephalography (EEG) correlations. METHODS: We enrolled in the study patients with diagnosis of symptomatic MTLE and a group of healthy controls. All patients and controls underwent long-term video -EEG monitoring, including at least one night of nocturnal sleep. We analyzed all the movements recorded during nocturnal sleep of patients and controls and their electroencephalographic correlations. RESULTS: We analyzed the nocturnal sleep of 15 patients with symptomatic MTLE (8 males and 7 females; mean age ± standard deviation [SD]31.8 ± 14.9 years) and of 15 healthy controls (6 males and 9 females; mean age ± SD 32.8 ± 11.2 years). The analysis of movements during sleep revealed significant differences between groups, with the patients presenting significantly more movements in sleep than healthy controls (56.7 ± 39.2 vs. 15 ± 6.1; p < 0.001) with significant differences regarding oroalimentary automatisms, limb dystonia, straightening movements and gestural automatisms. EEG analysis showed that the proportion of movements preceded by EEG abnormalities was significantly higher in patients than in controls (57.8 ± 35.9 movements vs. 16.6 ± 13.4 movements; p < 0.001). SIGNIFICANCE: The results of our study demonstrated the presence of minor motor events during sleep in patients with MTLE, suggesting an epileptic origin of these episodes. The study of nocturnal sleep in MTLE patients is useful in helping the clinicians in the diagnostic and therapeutic workup of these patients.

Deep Learning Representation from Electroencephalography of Early-Stage Creutzfeldt-Jakob Disease and Features for Differentiation from Rapidly Progressive Dementia.

A novel technique of quantitative EEG for differentiating patients with early-stage Creutzfeldt-Jakob disease (CJD) from other forms of rapidly progressive dementia (RPD) is proposed. The discrimination is based on the extraction of suitable features from the time-frequency representation of the EEG signals through continuous wavelet transform (CWT). An average measure of complexity of the EEG signal obtained by permutation entropy (PE) is also included. The dimensionality of the feature space is reduced through a multilayer processing system based on the recently emerged deep learning (DL) concept. The DL processor includes a stacked auto-encoder, trained by unsupervised learning techniques, and a classifier whose parameters are determined in a supervised way by associating the known category labels to the reduced vector of high-level features generated by the previous processing blocks. The supervised learning step is carried out by using either support vector machines (SVM) or multilayer neural networks (MLP-NN). A subset of EEG from patients suffering from Alzheimer's Disease (AD) and healthy controls (HC) is considered for differentiating CJD patients. When fine-tuning the parameters of the global processing system by a supervised learning procedure, the proposed system is able to achieve an average accuracy of 89%, an average sensitivity of 92%, and an average specificity of 89% in differentiating CJD from RPD. Similar results are obtained for CJD versus AD and CJD versus HC.

Epilepsy associated with Leukoaraiosis mainly affects temporal lobe: a casual or causal relationship?

OBJECTIVE: To compare anatomo-electro-clinical findings between patients with epilepsy associated with leukoaraiosis only (EAL) and patients with a well-defined vascular lesion, i.e. post-stroke epilepsy (PSE). METHODS: Two hundred eighty-three subjects with epilepsy and cerebrovascular disease, consecutively seen in our epilepsy centres from January 2000 to March 2014, were retrospectively considered. Inclusion criteria were: history of one or more unprovoked seizures and MRI evidence of one or more vascular lesions. Exclusion criteria were: inadequate neuroimaging data, coexistence of nonvascular lesions, and psychogenic seizures. Subjects were divided in two groups: PSE and EAL, based onclinical and MRI findings. Epileptogenic focus was identified according to ictal semiology and EEG findings. In PSE group, coherence between the vascular lesion(s) and epileptogenic focus was scored as likely or unlikely. RESULTS: One hundred seventeen subjects were included: 58 had PSE, 59 EAL. Coherence was identified as likely in 38 (95%) and unlikely in 2 (5%). At univariate analysis, abnormal EEG and frontal localization were associated with a lower EAL probability [odds ratio (OR) 0.36, 95% confidence interval (CI) 0.15-0.87, p=0.02 and OR 0.12, 95% CI 0.04-0.37, p<0.001, respectively], while temporal localization was associated with a higher EAL probability (OR 4.0, 95% CI 1.8-9.0, p<0.001). Multivariate confirmed these associations. CONCLUSIONS: While in PSE epileptogenic focus is coherent with the vascular lesions, in EAL temporal lobe epilepsy predominates. In EAL, causal relationship between vascular lesions and epilepsy is not straightforward, and the role of adjunctive factors needs to be elucidated.