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• W2095406491 abstract "In ancient times, it was the common belief that epilepsy represented attacks by the gods or evil spirits, usually as punishment. Epilepsy was thus considered “the sacred disease” (Hippocrates, 1984). It is likely that the first major step toward a modern understanding occurred in about 400 BC, when Hippocrates taught and wrote that epilepsy is a disease of the brain that must be treated by diet and drugs, not religious incantations (Temkin, 1971; Engel & Pedley, 2008). Further advances in understanding epilepsy emerged very slowly over the subsequent 2,300 years, and the pace of discovery only really began to accelerate in about 1850 or so with the writings of Robert Bentley Todd (1855),Jean-Martin Charcot (1891) and, especially, John Hughlings Jackson. It is safe to say that between 1850 and 2008, there have been more fundamental discoveries related to epilepsy than in all the other centuries that had followed Hippocrates. Jackson’s (1870, 1881) contributions remain fundamental. Solely through clinical observations of focal motor seizures, he accurately inferred, first, that cortical motor representation was fixed in a predictable way within the rolandic cortex of the opposite hemisphere; and, second, that the motor cortex was organized in terms of movements, not muscles. The cortical stimulation experiments of Fritsch and Hitzig (1870) soon confirmed the localized cortical representation of motor function. Jackson (1899) also localized other epileptic manifestations to specific cortical areas, including a “dreamy state” to the medial temporal lobe (uncus). Finally, he hypothesized that the underlying physiologic abnormality for all cases of epilepsy was the “occasional, sudden, excessive, rapid and local discharges of gray matter” (Jackson, 1873), and that the particular clinical manifestations of such discharges depended on the specific areas of brain in which they occurred. Kaufmann (1912) working in Russia was the first to observe electrical changes in the brain during experimentally produced seizures. Unfortunately, he was unable to photograph or otherwise create a permanent record of them. The first photographs of electroencephalography (EEG) changes during experimental seizures were published 2 years later by Cybulski and Jelenska-Macieszyna (1914). The modern era of epilepsy investigation in humans can be dated to 1929 when Hans Berger published recordings of the brain’s electrical activity recorded at the scalp (Berger, 1929). Berger (1932) illustrated sequential postictal EEG changes following a generalized tonic–clonic seizure, and Berger (1933) published both the first example of interictal discharges as well as a “minor epileptic seizure” (probably an absence attack) with 3/s rhythmic waves in the EEG. Correlating EEG patterns with clinical seizure phenomena represented a major step in understanding and classifying epilepsy. Pioneering collaborative studies in Boston by Frederick and Erna Gibbs, frequently in collaboration with William G. Lennox, established the concept of clinical–electrographic correlations (Gibbs et al., 1935, 1936, 1937, 1943, 1948). Indeed, from about 1935 to 1960, these investigators provided a steady stream of major contributions that shaped many of our modern concepts related to EEG and epilepsy. At first it seemed that specific EEG patterns correlated with the major types of epilepsy, because different interictal and ictal abnormalities were seen in grand mal, petit mal, and psychomotor seizures. Of course, this proved to be a great oversimplification, but even as later classifications became increasingly refined, EEG findings have remained a physiologic mainstay of both diagnostic investigation and clinical management. The idea that epilepsy has a genetic component is an ancient one. Hippocrates considered epilepsy a hereditary disease both in humans and in lower animals, as did Galen. In modern times, the studies of Lennox in families of patients with epilepsy and, especially, in twin pairs (the great majority of whom he had personally evaluated), provided important and enduring documentation of the hereditability both of epilepsy and certain EEG patterns (Lennox, 1947, 1951; Lennox & Lennox, 1960. An important contribution was Lennox’s emphasis that environmental and hereditary factors interact in all patients with epilepsy, although one or the other may be relatively more contributory in individual patients. In a recent reanalysis of Lennox’s twin pairs using contemporary classifications, Vadlamudi et al. (2004) concluded that the Lennox data provided clear evidence of a strong genetic basis for idiopathic generalized seizures and syndromes, as well as for febrile seizures. Furthermore, there was good correlation between Lennox’s data, particularly for idiopathic generalized epilepsy, and the more recent findings from the Australian twin data set (Berkovic et al., 1998). Although Locock (1857) described using bromides to treat epilepsy, and Hauptmann (1912) introduced phenobarbital, the six papers related to the development of sodium diphenyl hydantoinate (phenytoin) published by Putnam and Merritt between 1937 and 1941 forever transformed the treatment of epilepsy and the development of antiepileptic drugs (Putnam & Merritt, 1937; Merritt & Putnam, 1938a, 1938b, 1939, 1940; Putnam & Merritt, 1941. Rowland (1982) summarized their monumental achievements as follows: They devised a simple but reproducible method to test drugs for anticonvulsant efficacy that utilized the threshold for convulsive seizures induced by electrical stimulation of the brain in cats. They demonstrated that anticonvulsant effects of drugs in this model accurately predicted efficacy in humans. Phenytoin became the first antiepileptic drug to be tested in animals before it was given to humans. They showed that anticonvulsant effects could be separated from sedative effects. The experience with bromides and phenobarbital had suggested that the two were necessarily linked. They showed that the efficacy of anticonvulsant drugs differed according to type of seizure. For example a drug that was effective in suppressing grand mal seizures could be ineffective against petit mal seizures, and vice versa. They discovered that phenytoin was a potent anticonvulsant drug, and it remained a cornerstone of epilepsy treatment for more than 50 years. Finally, their method of screening antiepileptic drugs opened the way for development of other effective antiseizure drugs. Wilder Penfield was one of the great pioneers in the development of excisional surgery as a treatment of choice for uncontrolled focal epileptic seizures, especially those arising from neocortical regions (Penfield & Steelman, 1947; Penfield & Flanigin, 1950; Penfield & Baldwin, 1952). Although Morris (1950, 1956) reported an early series of patients undergoing temporal lobe resections that included mesial structures, and Bailey and Gibbs (1951) were the first to use EEG guidance in performing temporal lobe surgery, it was the sustained and carefully documented work of Penfield and his colleagues in Montreal that gave a major impetus to the surgical treatment of epilepsy (Rasmussen, 1977). Penfield studied with Foerster, from whom he learned to remove glial scars from patients with posttraumatic epilepsy and to use electrical stimulation to map motor and sensory areas of the brain (Foerster & Penfield, 1930). During his many years in Montreal, Penfield’s interest in the surgical treatment of epilepsy was closely intertwined with his commitment to exploring and mapping the localization of different functions within the cerebral cortex. With the arrival of Herbert Jasper in 1937, Penfield acquired a sophisticated neurophysiologist to join him in localizing epileptogenic foci and in exploring cortical physiology and function. This partnership culminated in one of the great classics of neurology and neuroscience, Epilepsy and the Functional Anatomy of the Human Brain (Penfield & Jasper, 1954). Penfield’s experience of epilepsy surgery, documented in numerous papers, and that of his students, most of whom later established epilepsy surgery units of their own, offered clear evidence of the safety and efficacy of resective surgery as a treatment option for patients with uncontrolled focal epilepsy, and set a standard and scientific approach that remains a benchmark today. Finally, no discussion of epilepsy surgery can end without acknowledging Murray Falconer’s contributions, particularly his recognition of the importance of hippocampal sclerosis in temporal lobe epilepsy (Falconer, 1968). Epileptiform discharges in EEG are indicators of susceptibility to seizures. The reason for this is that they reflect a specific underlying cellular abnormality characteristic, to one degree or another, of neurons within an epileptogenic focus. Experimental studies in animals have demonstrated that EEG spikes or sharp waves are associated with synchronous paroxysmal depolarizing bursts occurring in cortical neurons (Matsumoto & Ajmone-Marsan, 1964; Prince, 1968a; Prince & Futamachi, 1968). The long-lasting hyperpolarization that typically follows each cellular burst results in the surface slow wave that commonly follows each spike or sharp wave (the spike-wave complex) (Prince, 1968b). The bursts also trigger inhibitory activity in the surrounding cortex (“surround inhibition”), which helps restrict the spread of epileptiform discharges (Prince & Wilder, 1967). Surround inhibition also probably contributes to the intermittent slowing often seen in an epileptic EEG focus. Subsequently, after an earlier report by Yamamoto (1972), Prince and his colleagues—most notably Schwartzkroin and Wong—used slice preparations successfully to extend studies of cellular phenomena of epileptic events, including the first such recordings from human cortex (Schwartzkroin & Prince, 1978; Wong & Prince, 1978, 1979; Prince & Wong, 1981). Prince is one of the great epilepsy physician–scientists of modern times. His laboratory has had a remarkable record of continuing major contributions to our understanding of the basic mechanisms of epilepsy and also of training several generations of epilepsy leaders, not only from North America but from around the world. One of the oldest and best-established clinicopathological correlations is between epilepsy and hippocampal sclerosis. Beginning with the initial observations of Bouchet and Cazauvieilh (1825) and Sommer (1880), modern concepts of hippocampal sclerosis have been greatly influenced by the careful pathologic studies of Margerison and Corsellis (1966) and Bruton (1988), and by the physiologic studies of Meldrum. Meldrum et al. (1973) showed that the traditional assumption that brain damage resulting from seizures was the result of hypoxia and/or ischemia was incorrect. Although systemic factors can exacerbate brain damage, it is the excessive excitatory activity associated with ictal events that produces the characteristic pattern of cell loss seen in the hippocampus and elsewhere. Furthermore, neuronal populations within the hippocampus are selectively vulnerable (e.g., CA1 and the hilus) or resistant (e.g., CA2 and the granule cell layer) to the effects of seizure-induced injury. This “epileptic brain damage” occurs even when systemic factors (temperature, oxygenation, and blood pressure) are controlled. Subsequently, more complex changes were described as consequences of seizures, including mossy fiber sprouting and synaptic reorganization (Houser et al., 1990; Sutula et al., 1988, 1989; Tauck & Nadler, 1985. It is likely that the Seizure Unit established by William G. Lennox at the Children’s Hospital in Boston was the forerunner of today’s comprehensive epilepsy centers (Lombroso et al., 1988). The Seizure Unit emphasized diagnosis and treatment, training of personnel necessary to provide comprehensive care (doctors, nurses, psychologists, social workers, and others), research, psychosocial rehabilitation, and education. Indeed, Dr. Frederick Gibbs (1961) predicted in his obituary for Lennox that the Seizure Unit would be the model for other centers, “wherever men are determined to have the best.” In the late 1960s and 1970s, it became increasingly recognized that the most effective treatment of patients with epilepsy required a multidisciplinary approach to address the many facets of optimal management—including accurate diagnosis and classification of seizures; identification and avoidance of precipitating factors; pharmacologic treatment that minimized both acute and chronic toxicities; appropriate surgical interventions in selected patients; and vocational, educational, and psychosocial rehabilitation (Dreifuss, 1980). With the indefatigable leadership of Kiffin Penry [then Chief of the Epilepsy Branch at the National Institute of Neurological Disorders and Stroke (NINDS)] and his colleagues Roger Porter and Fritz Dreifuss, and with the practical impetus of National Institutes of Health (NIH) funding, several comprehensive epilepsy centers were established in the 1970s. Research was an important component of these early centers, as was the use and integration of community resources. This has continued to be the case in the best centers today, which often serve as regional or even national resources for the management of patients with intractable seizures. Guidelines for these centers have been established by the National Association of Epilepsy Centers (2001). Neuropathologic examination of the nervous system has long played an important clinical and research role in understanding neurologic diseases, and the clinicopathologic conference is a time-honored educational forum. Until the 1970s, the study of neuroanatomy and the structural effects of diseases on the brain could only be done by direct examination of tissue obtained by biopsy or autopsy. The introduction of computerized tomography and, later, magnetic resonance imaging (MRI) dramatically changed that situation forever, as it became possible to study the brain in both health and disease in living individuals. Computed tomography (CT) provides images based on the differential absorption of x-rays by brain tissue. MRI depends on the differences in intensities of radiowave signals that arise from tissue in which the alignment of hydrogen atoms placed in a magnetic field has been displaced by a radiofrequency pulse. With both CT and MRI, additional information, particularly about the effects of pathologic processes on the blood–brain barrier, can be obtained by use of contrast agents. Brain imaging, especially MRI, has had an incalculable impact on the field of epilepsy. It is essential for the diagnosis and localization of epileptogenic lesions, as its ability to detect small epileptogenic lesions such as hippocampal sclerosis, cavernous malformations, cortical dysplasia, hamartomas, gangliogliomas, and dysembryoplastic neuroepithelial tumors is unsurpassed. Imaging is also important in planning epilepsy surgery, especially when intracranial electrodes are required, and in situations when coregistration of functional, metabolic, and anatomic modalities is desirable. Finally, brain imaging is integral to epilepsy research. Beginning in 1979, Noebels used mouse models of epilepsy phenotypes to study the effects of single gene mutations (“epilepsy genes”) (Noebels & Sidman, 1979). Such mouse models have proved to be a powerful tool in defining key sites of vulnerability in the brain’s careful regulation of cortical excitability. Leppert et al. (1989) reported the first linkage in an idiopathic human epilepsy syndrome to chromosome 20 in a family with benign familial neonatal convulsions. Lewis et al. (1993) described linkage to chromosome 8 in two other families with benign familial neonatal convulsions. In 1998, three reports defined the molecular basis at both loci as mutations in the voltage-gated potassium channel genes KCNQ2 (chr 20) or KCNQ3 (chr 8) (Biervert et al., 1998; Charlier et al., 1998; Singh et al., 1998) Three years earlier, however, Steinlein et al. (1995) were actually the first to report the molecular basis of a human idiopathic epilepsy syndrome when they identified a missense mutation in the α-4 subunit of the neuronal nicotinic acetylcholine receptor, CHRNA4, in patients with familial nocturnal frontal lobe epilepsy. Although many of the single-gene mutations identified in idiopathic epilepsies are channelopathies, the number of genes found so far is still small, and in some instances, may be family specific, not broadly syndrome specific. In autosomal dominant lateral temporal lobe epilepsy with auditory features, the molecular basis is a mutation in the leucine-rich glioma inactivated gene 1 (LGI1) on chromosome 10q, whose function remains largely unknown (Kalachikov et al., 2002). Furthermore, epidemiologic and family studies indicate that in the great majority of people with epilepsy, the role of genetic factors, although clearly evident, is both less certain and more complex. There are undoubtedly specific genetic determinants of the brain’s susceptibility to seizures and epilepsy following a particular injury, as well as other genetic factors that determine the occurrence of individual epilepsy syndromes. One of the greatest challenge facing investigators now is to identify and characterize those genes that alter an individual’s susceptibility to seizures in the presence of acquired brain pathology or as a reaction to acute or subacute cerebral dysfunction. Concepts about epilepsy, or indeed of any disease, are very much the products of the scientific beliefs of the time, and the tools that are available to investigators. Therefore, over the last century we have seen the focus of epilepsy research and, as a result, views of what epilepsy “is,” developing historically from anatomic, neurophysiologic, and neurochemical perspectives, to a more encompassing neurobiologic construct that itself has evolved from system to cell; from in vivo to in vitro models; and to studies of transmitters, receptors, and channels. Now, of course, we are firmly in the era of molecules and genes. As we celebrate Epilepsia’s centenary and begin its second century, there is an almost palpable excitement about the growing opportunities to make a difference for people with epilepsy and other neurologic disorders. The diversity with which human epilepsy expresses itself indicates that it is not a unitary problem and, therefore, that there is unlikely to be a singular solution to any of epilepsy’s many facets. Nonetheless, the potential of new investigative tools, especially those of brain imaging, molecular biology, and bioengineering, are providing unparalleled and rapidly accelerating progress in illuminating the mechanisms of epilepsy and epilepsy-related brain dysfunction, and offering greater hope than ever before for prevention, effective treatment, and even cure. I thank Dr. Daniel Lowenstein, University of California at San Francisco, for providing some of the information used in this article from material he assembled while preparing the Hoyer Lecture, which he presented at the 2006 Annual Meeting of the American Epilepsy Society. I confirm that I have read Epilepsia’s position on issues involved in ethical publication and affirm that this report is consistent with those guidelines. Disclosure: I have no conflicts of interest to disclose." @default.
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• W2095406491 title "Major advances in epilepsy in the last century: A personal perspective" @default.
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