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• W1489330186 abstract "To the Editors: We are very grateful to Drs. Wong, Jozwiak, and Franz for elaborating upon and extending some of the critical issues raised in our recent review of tuberous sclerosis complex (TSC). Dr. Wong raises many important points as to whether tubers are necessary to derive useful mechanistic and clinical information from animal models of TSC. These issues were also discussed at length at the 2004 workshop, and participants concluded that a model that included tubers would be ideal but that species differences in brain structure and development would make such a TSC model unlikely. Nevertheless, as Dr. Wong mentions, with appropriate caveats and hypothesis-driven experiments, valuable insights can be obtained from nontuber models. We appreciate Dr. Wong's update regarding informative approaches currently being investigated in his and other laboratories. Dr. Jozwiak and colleagues propose a radical concept that is, at least at first glance, heretical in epileptology—treatment of epilepsy before epilepsy begins. The standard teaching has long been to treat seizures—not the possibility of seizures, not interictal spikes, but seizures. Perhaps head trauma is a case in point—there is no evidence that prophylactic treatment of head injured patients prevents the later development of epilepsy (Temkin, 2001). However, this philosophy may be giving way, and rightly so, for a number of reasons. It is increasingly being recognized that seizures are only a part of epilepsy, and sometimes a rather small part. Often, the accompanying cognitive dysfunction, mood abnormalities, antiepileptic drug side effects, and numerous other psychological, social, and vocational factors impair the lives of patients with epilepsy as much or more than seizures themselves. In addition, some forms of epilepsy appear to be progressive, with accumulating deficits over time (Sutula, 2004). The latent period between an initial brain insult and the development of epilepsy (epileptogenesis) is now a subject of intense scrutiny, with the presumption that understanding the neurobiological changes during the latent period will allow formulation of a targeted intervention to slow or halt progression to fully manifested epilepsy. Finally, the possibility that interictal neuronal activity in the form of interictal spikes might facilitate epileptogenesis, another radical concept, is receiving increasing attention (Staley and Dudek, 2006). With these issues in mind, Jozwiak's clinical approach deserves careful consideration. By prospectively identifying infants at risk for TSC (by fetal echocardiography to look for cardiac rhabdomyomas, careful physical examinations and genetic studies early in life, and EEGs prior to seizure onset), they have defined a population at extremely high risk for epilepsy. In these at-risk babies, would treatment with an antiepileptic drug prevent epileptogenesis or reduce seizure burden and cognitive/behavioral consequences? This is an intriguing idea, but the first question is what agent would be most effective. Currently available antiepileptic drugs have few if any convincing antiepileptogenic effects. Jozwiak cites a case report in which prophylactic vigabatrin treatment of a child with TSC appeared to stave off abnormal EEGs and onset of seizures, a beneficial effect that dissipated once the drug was stopped (due to concerns about visual field restriction). Other treatments that have shown some promise of an antiepileptogenic action include topiramate, valproate, levetiracetam, and the ketogenic diet. While vigabatrin and other treatments have shown acute seizure suppression effects in animal models of epilepsy (Vinogradova et al., 2005), we should heed a lesson learned from recent neuroprotection studies in stroke and head injury in which promising results in animal models did not translate into efficacy in large clinical trials (Savitz and Fisher, 2007; Temkin et al., 2007). Nevertheless, given the frequent poor cognitive outcome in TSC, with degree of severity paralleling seizure frequency, TSC represents a unique “natural culture” in which the above questions can be tested. An important advantage of studying antiepileptic therapy in TSC would be the limited number of patients necessary to determine if the intervention prevented the onset of epilepsy. Unlike disorders such as head trauma in which the risk of developing epilepsy is fairly low, the risk of epilepsy in TSC is very high. If one assumes that 60–80% of diagnosed infants with TSC develop seizures and that a drug with antiepileptogenic properties would reduce risk of epilepsy by 50%, a sample size of 27 in treated and nontreated groups would have an 80% power to detect a 50% decrease of developing epilepsy with a significance level (α) of 0.05 (two-tailed). Since the onset of epilepsy in TSC is typically within the first few years of life (Cross, 2005), the study duration could be limited to 1–2 years. Studying more than one dosage or drug would necessarily require more patients. However, even with multiple agents or doses, double-blinded, placebo-controlled study could be done rather quickly and efficiently. In view of the severe nature of epilepsy in TSC we believe that many families of children with TSwould be eager to participate in such a study. As discussed in our review, several of the molecular signaling mechanisms that have gone awry in TSC could be targeted for preventative intervention. We agree with Dr. Jozwiak that a longitudinal prospective study of early antiepileptic therapy in infants with TSC before the appearance of clinical seizures is not only reasonable but also necessary. The additional therapies targeting the mTOR pathway discussed by Dr. Franz provide even more opportunities for much needed clinical investigation." @default.
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• W1489330186 date "2007-08-01" @default.
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• W1489330186 title "Can Preventative Antiepileptic Therapy Alter Outcome in Infants with Tuberous Sclerosis Complex?" @default.
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• W1489330186 doi "https://doi.org/10.1111/j.1528-1167.2007.01178_4.x" @default.
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