Epilepsia
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The International League Against Epilepsy (ILAE) standardized classification and terminology for "epileptic seizures" of 1981 and "epilepsies and epileptic syndromes" of 1989 provide a fundamental framework for organizing and differentiating the epilepsies. However, a revision of these classifications is mandated by recent major technologic and scientific advances. Since 1997, the relevant ILAE Commissions have made significant efforts to achieve better and internationally uniform classifications as reflected in their reports of 2001, 2006, and 2010. ⋯ The ILAE Commission could benefit by asking experts in basic and clinical science to provide a concise statement in their field of expertise as, for example, what are focal, myoclonic, or absence seizures, and their subtypes, their manifestations, and their possible pathophysiology. Areas of certainties and uncertainties, agreements and disagreements should be identified and stated clearly, with documentation of the reasons for it. Probably this is the only way forward for a truly scientific, sound, and clinically meaningful organizational system for the epileptic seizures and the epilepsies.
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By definition idiopathic generalized epilepsy (IGE) is not associated with structural abnormalities on conventional magnetic resonance imaging (MRI). However, recent quantitative studies suggest white and gray matter alterations in IGE. The purpose of this study was to investigate whether there are white and/or gray matter structural differences between controls and two subsets of IGE, namely juvenile myoclonic epilepsy (JME) and IGE with generalized tonic-clonic seizures only (IGE-GTC). ⋯ The findings support the idea that the clinical syndromes of JME and IGE-GTC have unique anatomic substrates. The fact that the primary clinical difference between JME and IGE-GTC is the occurrence of myoclonus in the former raises the possibility that disruption of white matter integrity may be the underlying mechanism responsible for myoclonus in JME. The cross-sectional study design and relatively small number of subjects limits the conclusions that can be drawn here; however, the absence of a correlation between fractional anisotropy and lifetime seizures is suggestive that the white matter abnormalities observed in JME may not be secondary to seizures.
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Seizure-like activities generated in anterior cingulate cortex (ACC) are usually classified as simple partial and are associated with changes in autonomic function, motivation, and thought. Previous studies have shown that thalamic inputs can modulate ACC seizure, but the exact mechanisms have not been studied thoroughly. Therefore, we investigated the role of thalamic inputs in modulating ACC seizure-like activities. In addition, seizure onset and propagation are difficult to determine in vivo in ACC. We studied the spatiotemporal changes in epileptiform activity in this cortex in a thalamic-ACC slice to clearly determine seizure onset. ⋯ Cingulate seizure-like activities were modulated significantly by thalamic inputs. Repeated stimulation of the thalamus efficiently inhibited epileptiform activity, demonstrating that the desynchronization was pathway-specific. The clinical implications of deep thalamic stimulation in the modulation of cingulate epileptic activity require further investigation.
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Cardiac arrhythmias and respiratory disturbances have been proposed as likely causes for sudden unexpected death in epilepsy. Oxygen desaturation occurs in one-third of patients with localization-related epilepsy (LRE) undergoing inpatient video-electroencephalography (EEG) telemetry (VET) as part of their presurgical workup. Ictal-related oxygen desaturation is accompanied by hypercapnia. Both abnormal lengthening and shortening of the corrected QT interval (QTc) on electrocardiography (ECG) have been reported with seizures. QTc abnormalities are associated with increased risk of sudden cardiac death. We hypothesized that there may be an association between ictal hypoxemia and cardiac repolarization abnormalities. ⋯ We have shown that the likelihood of abnormal QTcH prolongation is increased 4.3-fold with seizures that are associated with oxygen desaturation when compared with seizures that are not accompanied with oxygen desaturation. The likelihood of abnormally shortened QTcH increases with seizures that are accompanied by oxygen desaturation with an odds ratio of 2.13 compared with that in seizures without desaturations. There is a significant association between the depth and duration of oxygen desaturation and QTr increase. These findings may be related to the pathophysiology of SUDEP.
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Psychogenic nonepileptic seizures (PNES) continue to represent a serious diagnostic challenge for neurologists. Video-electroencephalography (EEG) studies have provided detailed knowledge of the spectrum of visible PNES manifestations. However, little is known about how patients or seizure witnesses experience PNES, although many diagnoses in seizure clinics are made on the basis of self-reported information rather than video-EEG observations. This study describes the range of PNES manifestations as they are reported by patients or seizure witnesses. ⋯ These findings based on the self-report of patients with well-characterized PNES and witnesses of their seizures demonstrate why it can be difficult to distinguish descriptions of PNES from those of epilepsy on the basis of factual items. The differences between patient and witness reports suggest that clinicians have to take note of the source of information they use in their diagnostic considerations. The intra- and interindividual variability of reported PNES manifestations demonstrates the clinical heterogeneity of PNES disorders. The positive correlation of symptoms of dissociation and anxiety in these patients may reflect psychopathologic differences between subgroups of PNES patients.