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Vertigo and dizziness are extremely common complaints, related to either peripheral or central nervous system disorders. Among the latter, epilepsy has to be taken into consideration: indeed, vertigo may be part of the initial aura of a focal epileptic seizure in association with other signs/symptoms, or represent the only ictal manifestation, a rare phenomenon known as "vertiginous" or "vestibular" seizure. These ictal symptoms are usually related to a discharge arising from/involving temporal or parietal areas, which are supposed to be a crucial component of the so-called "vestibular cortex". In this paper, we describe three patients suffering from drug-resistant focal epilepsy, symptomatic of malformations of cortical development or perinatal hypoxic/ischemic lesions located in the posterior regions, who presented clusters of vertiginous seizures. The high recurrence rate of such events, recorded during video-EEG monitoring sessions, offered the opportunity to perform an ictal EEG/fMRI study to identify seizure-related hemodynamic changes. The ictal EEG/fMRI revealed the main activation clusters in the temporo-parieto-occipital regions, which are widely recognized to be involved in the processing of vestibular information. Interestingly, ictal deactivation was also detected in the ipsilateral cerebellar hemisphere, suggesting the ictal involvement of cortical-subcortical structures known to be part of the vestibular integration network., (Copyright © 2016 Elsevier Inc. All rights reserved.)

Purpose: To provide imaging biomarkers of generalized spike-and-wave discharges (GSWD) in patients with GLUT1 deficiency syndrome (GLUT1DS)., Methods: Eighteen GLUT1DS patients with pathogenetic mutation in SLC2A1 gene were studied by means of Video-EEG simultaneously recorded with functional MRI (VideoEEG-fMRI). A control group of sex and age-matched patients affected by Genetic Generalized Epilepsy (GGE) with GSWD were investigated with the same protocol. Within and between groups comparison was performed as appropriated. For GLUT1DS, correlations analyses between the contrast of interest and the main clinical measurements were provided., Results: EEG during fMRI revealed interictal GSWD in 10 GLUT1DS patients. Group-level analysis showed BOLD signal increases at the premotor cortex and putamen. With respect to GGE, GLUT1DS patients demonstrated increased neuronal activity in the putamen, precuneus, cingulate cortex, SMA and paracentral lobule. Whole-brain correlation analyses disclosed a linear relationship between the GSWD-related BOLD changes and the levels of glycorrhachia at diagnosis over the sensory-motor cortex and superior parietal lobuli., Conclusion: The BOLD dynamics related to GSWD in GLUT1DS are substantially different from typical GGE showing the former an increased activity in the premotor-striatal network and a decrease in the thalamus. The revealed hemodynamic maps might represent imaging biomarkers of GLUT1DS, being potentially useful for a precocious diagnosis of this genetic disorder.

Objectives: To evaluate the spectral and spatial features of the cortical rhythms in patients affected by ring chromosome 20 - [r(20)]-syndrome., Methods: Twelve patients with [r(20)] syndrome were studied. As controls we enrolled 12 patients with idiopathic generalized epilepsy (IGE) and 12 healthy volunteers (HV). Blind source separation, spectral analyses and source reconstruction were applied in all cases in order to identify reliable spatio-temporal patterns of cortical activity., Results: A theta-delta EEG rhythm was identified in [r(20)] patients, with spectral peak ranging between 3 and 7Hz and whose generators mapped over the sensory-motor cortices. A second peak laying at a frequency about double with respect to the first one was present in 6 cases. Analogue methodological approach in HV and IGE groups failed to show similar findings., Conclusions: EEG of [r(20)] patients reveals the existence of a highly reproducible EEG pattern arising from the sensory-motor system., Significance: The recognition of this peculiar EEG pattern could help the diagnostic work-up. Additionally, our findings supports the existence of a parallelism between this EEG trait and the physiological "mu" rhythm which is generate by the sensory-motor system. Such link suggests a sensory-motor system dysfunction in [r(20)] patients., (Copyright © 2013 International Federation of Clinical Neurophysiology. Published by Elsevier Ireland Ltd. All rights reserved.)

Background: Generalised spike wave (GSW) discharges are the electroencephalographic (EEG) hallmark of absence seizures, clinically characterised by a transitory interruption of ongoing activities and impaired consciousness, occurring during states of reduced awareness. Several theories have been proposed to explain the pathophysiology of GSW discharges and the role of thalamus and cortex as generators. In this work we extend the existing theories by hypothesizing a role for the precuneus, a brain region neglected in previous works on GSW generation but already known to be linked to consciousness and awareness. We analysed fMRI data using dynamic causal modelling (DCM) to investigate the effective connectivity between precuneus, thalamus and prefrontal cortex in patients with GSW discharges., Methodology and Principal Findings: We analysed fMRI data from seven patients affected by Idiopathic Generalized Epilepsy (IGE) with frequent GSW discharges and significant GSW-correlated haemodynamic signal changes in the thalamus, the prefrontal cortex and the precuneus. Using DCM we assessed their effective connectivity, i.e. which region drives another region. Three dynamic causal models were constructed: GSW was modelled as autonomous input to the thalamus (model A), ventromedial prefrontal cortex (model B), and precuneus (model C). Bayesian model comparison revealed Model C (GSW as autonomous input to precuneus), to be the best in 5 patients while model A prevailed in two cases. At the group level model C dominated and at the population-level the p value of model C was approximately 1., Conclusion: Our results provide strong evidence that activity in the precuneus gates GSW discharges in the thalamo-(fronto) cortical network. This study is the first demonstration of a causal link between haemodynamic changes in the precuneus -- an index of awareness -- and the occurrence of pathological discharges in epilepsy.

Purpose: Electroencephalography/functional magnetic resonance imaging (EEG/fMRI) has been proposed recently as a tool to study electrophysiological activity and, consequently, detect brain regions activated during epileptiform EEG abnormalities. The purpose of the study was to review our two-year experience with studying ictal and interictal activities in patients with epilepsy., Methods: Using EEG/fMRI, we studied hemodynamic changes associated with ictal and interictal EEG abnormalities in 43 patients with partial (31 cases) or generalized (12 cases) epilepsy. Using two different paradigms (block design and event-related design), we studied several forms of EEG activity consisting of (i) interictal abnormalities constantly elicitable by specific stimulation (8 cases); (ii) focal and generalized interictal activity, such as focal spikes or typical and atypical generalized spike-and-wave discharges (18 cases); and (iii) focal and generalized ictal electro-clinical activity, such as tonic seizures or pseudo-absences in frontal lobe epilepsy, typical absences in idiopathic generalized epilepsy, complex partial seizures in temporal lobe epilepsy, and perisylvian seizures in special syndromes (17 patients)., Results: EEG/fMRI revealed clear hemodynamic changes related to EEG abnormalities in 21 patients. In 18 of these patients, the changes were highly concordant with electro-clinical findings. In the remaining 22 patients, fMRI analysis data failed to show activation or deactivation clusters, probably owing either to lack or inadequate amount of temporal distribution of abnormal EEG activity, or to intrinsic methodological problems., Conclusions: By defining the electro-clinical and hemodynamic correlates of EEG activity, fMRI may shed light on the neurophysiological mechanisms underlying epileptic phenomena. However, as several methodological issues have yet to be addressed, further studies are warranted to assess the reliability and usefulness of EEG/fMRI in clinical practice.