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.

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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.

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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.

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.

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 β 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 β analysis showed a significant increase of β over the frontal regions in patients as compared to controls during resting-state and an increase of β 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.

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.