Abstract Purpose: To demonstrate the clinical importance of using a high quality photic stimulator for recording EEGs to diagnose photosensitivity. Methods: We performed EEG examinations on 2 adult ...and 2 paediatric patients with a history of visually induced seizures; routinely we used a Grass PS 40 photic stimulator (rectangular Xenon lamp giving flashes of 10 μs duration, 0.7 J, 1–30 Hz, width 7 cm, length 12 cm). We repeated the IPS with a Grass PS 33 plus stimulator (round Xenon lamp giving flashes of 10 μs duration, 1 J, 1–60 Hz, diameter 14 cm). Results: Patients were affected by both benign and catastrophic epilepsies. They complained about episodes of dizziness (case 1), dizziness accompanied by a sensation in the arms and fear (case 2), absences (case 3), and myoclonic jerks (case 4). These symptoms occurred when working with neon lights, computers or ironing striped clothes (case 1), while driving (case 2), whenever there was sunlight (case 3 and 4). Only IPS performed with the Grass PS 33 plus stimulator evoked PPRs accompanied by their typical complaints. In all cases, the revised diagnosis led to changes in their treatment and the disappearance or diminishment of their complaints and PPR range. Conclusion: A PPR can occur in various types of epilepsy, can have a different meaning, and requires a different therapeutic intervention. Only an appropriate photic stimulator with diffuse white light and a flash intensity level of 1 J/flash, can reliably demonstrate whether a patient is photosensitive, or equally important exclude it.
Summary The comorbidity between epilepsy and migraine has been well known for a century, yet it is still not fully understood; the two disorders also share some risk factors, symptoms, and preventive ...drug therapy. A series of clinical observations and scientific data support the hypothesis of alteration of cortical excitability as a possible mechanism underlying their pathology, with both disorders characterized by transient paroxysmal neurological disturbance. So far, the numerous pathophysiological mechanisms responsible for neuronal hyperexcitability have only been studied in familial hemiplegic migraine (FHM), but they do suggest a link between migraine and epilepsy. Several studies support the hypothesis of a clinical continuum between some types of migraine and some types of epilepsies, with possibly even a complete overlap, representing, in particular cases, headache as the sole ictal manifestation of seizures. Taking into account the data in the literature, we hypothesize that several aetiopathological noxae (either environmental or genetics), such as Na+–K+ ATPase pump impairment, converging on a common final pathway represented by neuronal membrane hyperexcitability, could manifest as either epilepsy or headache/migraine, or both. The potential implications arising from this point of view include (a) a revision of headache/migraine diagnostic criteria as the sole ictal epileptic manifestation in international classifications of both epilepsies and headache disorders; (b) the careful follow-up of patients with headache/migraine as a residual feature, taking into consideration a revised concept of “complete seizure control” to avoid mistakes due to inopportune withdrawal of antiepileptic treatment. In addition, we suggest that headache is associated with other ictal-sensitive and motor features (more than those reported); these may be highly underestimated due to impairment of consciousness during complex partial seizures with or without secondary generalization.
Abstract Heritable EEG traits are often associated with epilepsy, and photoparoxysmal EEG response (PPR) is the most notable example of this observation in JME. Such EEG traits may be a subclinical ...expression of the defective mechanism that leads to epilepsy. Therefore, these traits can be used to map epilepsy genes by dissecting the complex epilepsy phenotype in endophenotypic sections that on their own have a presumed monogenic cause. Two characteristics make PPR particularly interesting as a useful endophenotype for epilepsy gene mapping. First, it shows an increased comorbidity with some but not all forms of epilepsy. Second, its mode of inheritance is compatible with a monogenic cause, which promises relative straightforward gene identification through positional cloning. Here, we summarize the current state of affairs. This article is part of a supplemental special issue entitled Juvenile Myoclonic Epilepsy: What is it Really?
Behavioral aspects of epilepsy Schachter, Steven C; Holmes, Gregory L; Kasteleijn-Nolst Trenite, Dorothée G. A
2008., 2007, 20071015, 2007-10-15
eBook
Covers the mechanisms underlying epilepsy and behavior, neurophysiologic function, neuropsychiatric and behavioral disorders in patients with epilepsy, the effects of treatments and surgery on ...behavior, pediatric and adolescent epilepsy, disorders associated with epilepsy that impact behavior, and more.
Highlights • Four members of one family had visual sensitivity combined with talking induced myoclonic jerks. • Visual sensitivity increased around age fifty with onset of talking induced jaw jerks. ...• All photosensitive family members shared a heterozygous R129C mutation in the SCNM1 gene. • SCNM1 mutation might lead to increased susceptibility to PPR and epilepsy.
Summary Purpose Carisbamate, a novel neuromodulatory agent with antiepileptic properties, was evaluated in patients with photoparoxysmal responses to intermittent photic stimulation (IPS) in this ...multicenter, non-randomized, single-blind, placebo-controlled, proof-of-concept study. Methods Eighteen Caucasian patients (14 females, 4 males) with a mean age of 30 years (range: 16–51 years) underwent standardized IPS under three eye conditions (during eye closure, eyes closed and eyes open) at hourly intervals for up to 8 h after receiving placebo (Day 1), carisbamate (Day 2) and placebo (Day 3). Carisbamate was given at single doses of 250–1000 mg. All patients received one or two concomitant antiepileptic drugs, most commonly valproate. Results Carisbamate produced a dose-dependent reduction in photosensitivity in the 13 evaluable patients, with abolishment of photoparoxysmal responses in 3 patients and clinically significant suppression of such responses in 7 additional patients. Photosensitivity was abolished or reduced in all five patients in the 1000-mg dose group. The onset of carisbamate occurred rapidly, with clinically significant suppression achieved before or near the time peak plasma drug levels were reached. The duration of action was dose-related and long-lasting, with clinically significant reductions of photosensitivity observed for up to 32 h after doses of 750 or 1000 mg. Carisbamate was generally well tolerated, with dizziness and nausea reported more frequently after active drug than placebo. Conclusion This study shows that carisbamate exhibits dose-related antiepileptic effects in the photosensitivity model. Randomized, controlled studies of carisbamate in epilepsy patients inadequately controlled by their existing AED therapy are warranted.
Although many observations in patients with this intriguing type of epilepsy have been described and detailed studies have been performed, only a few meet the current criteria of class 1 or 2 ...evidence-based studies. In general, the selection bias is due to studying a referral population instead of the general population, and to different age and sex distributions of the subjects under study. Comparing the various studies is often difficult, because of differences in the populations studied (single seizures, epilepsy centre population, etc.), but also because of different methods (photic stimulator, flash frequencies, eye conditions, etc.) and the terminology used.
Finally, and most crucial, in many studies there is often no information on how the data were actually obtained (EEG or clinical data or both?). The popular term “photosensitive” is used widely and applied to patients with a history of visually induced seizures, with and without a photoparoxysmal response (PPR), and to those with only a PPR. An overview of the “hard” data is given with future needs for a better understanding of this type of epilepsy and for improving the endophenotype for genetic research.
It is important to standardise the studies as much as possible and describe in detail the methodology of the study, taking at least the above variables into account.
Purpose: Cognitive deficits are one of the major limiting factors in the everyday life functioning of patients with focal seizures. Although cognitive rehabilitation methods have been successfully ...applied to patients with other central nervous system (CNS) lesions, these methods have not yet been evaluated in cognitively impaired patients with epilepsy. The present study evaluated the effectiveness of two commonly used methods for attention deficits: (a) the Retraining Method, aimed at retraining impaired cognitive functions; and (b) the Compensation Method, aimed at teaching compensatory strategies while taking neuronal loss for granted.
Methods: Fifty adult outpatients with focal seizures and attention impairments receiving carbamazepine (CBZ) monotherapy were randomly assigned to the Retraining Method, the Compensation Method, or to a waiting‐list control group. Established and self‐reported neuropsychological outcomes and self‐reported quality of life of these groups were evaluated at pretraining, posttraining, and at a 6‐month follow‐up measurement point and were completed by 44 patients.
Results: Neuropsychological outcomes related to training, self‐reported neuropsychological outcomes, and quality of life at the 6‐month follow‐up measurement point improved both in the Retraining Method group (n = 19) and the Compensation Method group (n = 17) relative to the waiting‐list control group (n = 8). The Compensation Method was more effective in improving self‐reported neuropsychological outcomes and quality of life, especially for patients with less education. The patients with active epilepsy benefited more from both methods than did the seizure‐free patients.
Conclusions: These data show that cognitive rehabilitation programs are effective for patients with focal seizures and attention deficits and should, therefore, be incorporated into comprehensive care programs.
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