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Cognition-induced epilepsy associated with specific emotional precipitants.

A 32-year-old woman with primary generalized epilepsy described absence events precipitated by talking about childhood sexual abuse, her epilepsy, or her father's difficulties with schizophrenia. She was referred for assessment of suspected psychogenic nonepileptic events. During simultaneous video/EEG monitoring, 30 absence seizures were recorded, 28 of which occurred while discussing one of these three specific precipitants. No nonepileptic events were seen. This finding highlights the importance of thorough investigation of ambiguous events, and confirms that specific emotional precipitants can induce seizures in susceptible people with epilepsy.

Adult↗

Effects of lamotrigine on absence seizures in rats.

Lamotrigine, a novel antiepileptic drug thought to inhibit the excitatory neurotransmitter release and indicated for convulsive seizures, was tested for its efficacy in modulating non-convulsive seizures of the absence type of epilepsy. This was done in a rat model for absence epilepsy: the WAG/Rij strain of rats. In side-effect free doses, lamotrigine did not inhibit absence seizures in these animals. Only in a high dose a reduction of spike-wave activity was noticed, but in that dose side-effects such as abnormal locomotion, were also seen. These findings underline the differential pharmacological profile of convulsive and non-convulsive generalized epilepsy.

Animals↗

Polygenic control of idiopathic generalized epilepsy phenotypes in the genetic absence rats from Strasbourg (GAERS).

PURPOSE: Generalized nonconvulsive absence seizures are characterized by the occurrence of synchronous and bilateral spike-and-wave discharges (SWDs) on electroencephalographic recordings, concomitant with behavioral arrest. The GAERS (genetic absence rats from Strasbourg) strain, a well-characterized inbred model for idiopathic generalized epilepsy, spontaneously develops EEG paroxysms that resemble those of typical absence seizures. The purpose of this study was to investigate the genetic control of SWD variables by using a combination of genetic analyses and electrophysiological measurements in an experimental cross derived from GAERS and Brown Norway (BN) rats. METHODS: SWD subphenotypes were quantified on EEG recordings performed at both 3 and 6 months in a cohort of 118 GAERS x BN F2 animals. A genome-wide scan of the F2 progenies was carried out with 146 microsatellite markers that were used to test each marker locus for evidence of genetic linkage to the SWD quantitative traits. RESULTS: We identified three quantitative trait loci (QTLs) in chromosomes 4, 7, and 8 controlling specific SWD variables in the cross, including frequency, amplitude, and severity of SWDs. Age was a major factor influencing the detection of genetic linkage to the various components of the SWDs. CONCLUSIONS: The identification of these QTLs demonstrates the polygenic control of SWDs in the GAERS strain. Genetic linkages to specific SWD features underline the complex mechanisms contributing to SWD development in idiopathic generalized epilepsy.

Action Potentials↗

Brainstem triggers absence seizures in human generalized epilepsy.

Simultaneous analysis of brainstem auditory evoked potentials (BAEPs) with reference to electroencephalography (EEG) was designed to examine the brainstem function corresponding to the EEG event. With this method, we investigated the brainstem function pre- and during the paroxysmal discharge in human absence seizures classified as primary generalized epilepsy (PGE). Two types of functional change in the lower brainstem were revealed as parameters of wave-III components (amplitude and area) of BAEPs without significant change in the upper brainstem. One was long-range biphasic fluctuation (acceleration followed by abrupt deceleration with the maximum -6.4+/-3.2 s before the seizure onset), and the other was rhythmic oscillation with 3 Hz. The latter, synchronized with the cortical spike-and-wave complex, imposed on the descending slope of the former. One important point is that both preceded the onset of cortical paroxysmal discharge. The results reappraise the classical hypothesis of "centrencephalic system" on seizure generating mechanism in human PGE. The results prove the primary triggering role of the lower brainstem that is independent of sleep-related synchronizations. The method is applicable to other types of EEG event for the investigation of brainstem involvement.

Adolescent↗

[Observation of an absence status in the EEG without clinically detected psychopathology].

It is reported that a patient suffering from generalized idiopathic epilepsy accidentally showed continuous epileptic activity as spike-wave pattern during an electroencephalographic lead without observing any clinical changes to whom. This state having been ended by a grand mal, the patient could give unspecific subjective complaints for the period of the "bioelectrical petit mal state", but he was fully able to remember. It is referred to that the phenomenon of the clinical inconspicuous appearance and the missing amnesia are special features.

Adult↗

Anoxic-epileptic seizures: home video recordings of epileptic seizures induced by syncopes.

Occasionally, but more often than has been reported, true epileptic seizures are triggered by non-epileptic syncopes. This combination of syncope and epileptic seizure has been called an anoxic-epileptic seizure. A few examples of such anoxic-epileptic seizures, including the induction of status epilepticus, have been reported in books and medical journals, but no video-recordings have been published. We show here home video recordings of the first three known examples of the transition from the triggering syncope and anoxic seizure, to the subsequent epileptic seizure. In the first two children, a neurally-mediated syncope, probably mediated by prolonged expiratory apnoea (so-called breath-holding spells), induces a long, clonic epileptic seizure with some features of myoclonic absence. In the third example, a compulsive Valsalva in an older autistic child provokes a vibratory tonic epileptic seizure. In addition, we show two further video clips of the most usual type of epileptic seizure induced by syncopes in very young children. In one, the video recording begins after the end of the triggering syncope and shows a rhythmic clonic seizure that includes repetitive vocalizations. The final recoding is of a spontaneous epileptic seizure with features of myoclonic absence: this child had both epilepsy and identical episodes induced by syncopes, that is, anoxic- epileptic seizures. Not only paediatricians and paediatric neurologists, but also adult neurologists and epileptologists in general, should be aware of the important clinical scenario of true epileptic seizures induced by syncopes. This phenomenon is not considered in any international classification. (Published with videosequences)

Adolescent↗

[An absence].

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Adolescent↗

Typical absence seizures associated with localization-related epilepsy: a clinical and electroencephalographic characterization.

INTRODUCTION: This paper describes the characteristics of patients with typical absence seizures associated with localization related epilepsy (LRE) and compares electroclinical features of absences occurring in these patients with those having childhood absence epilepsy (CAE). METHODS: Consecutive patients presenting with both LRE and typical absences in their epilepsy history were included in the study (Group 1). Clinical assessments and EEG investigations were conducted during the follow-up. Patients observed during the same period, but with typical absences fulfilling the CAE diagnostic criteria, were assigned to a second group (Group 2). RESULTS: Fourteen patients were included in Group 1. These patients had a mean age at their last visit of 11.3 years (range 7.2-16.8), with a mean follow-up period of 6.8 years. In all patients LRE was the first type of seizure to occur at median age of 4.95+/-2.1 years (range 1.9-8.8). Typical absences appeared at median age of 7.5+/-2.5 years (range 4.5-12.5), and were well controlled by therapy. Ictal EEG and semiology features of typical absences did not show any distinctive features when compared to those of Group 2 represented by 53 patients affected by CAE. However, age at onset was significantly higher in Group 1, as was the number of patients who underwent polytherapy, and the number with relapses after drug discontinuation. None of patients in Group 1 showed terminal remission. CONCLUSION: Although clinically heterogeneous and rare, the association of LRE with typical absences may be more than coincidental. In these patients, typical absences responded well to therapy, but terminal remission rates were lower than for CAE patients.

Adolescent↗

Absence seizures in patients with localization-related epilepsy.

We studied the clinical features of 12 patients with localization-related epilepsy (LRE) associated with absence seizures (AS). AS did not appear in any patients before partial seizures (PS) were first observed. The interval between the onset of PS and AS ranged from 1 month to 7.2 years (mean 2.11 years). The duration of AS (mean 5 months) was short compared with that of PS (mean 3.8 years). Carbamazepine (CBZ) was used in seven patients at the onset of AS. It was discontinued in five but continued in the other two. AS was initially treated with valproate in ten patients. Three of them needed additional antiepileptic drugs: clonazepam in two patients and ethosuximide in one. All patients became free from AS after treatment for AS was started, whereas PS was relatively intractable. Generalized spike-and-waves were often observed before the onset of AS. The interval between the first appearance of generalized spike-and-waves and the onset of AS ranged from 1 to 53 months (mean 20 months). AS in patients with LRE will be relatively benign and transient, and will respond well to antiepileptic drugs.

Adolescent↗