Socrates and Temporal Lobe Epilepsy: A Pathographical Diagnosis 2400 Years Later
Osamu Muramoto and Walter G. Englertp
Socrates was one of the greatest philosophers of the Western world. However some of his enigmatic remarks and behaviors have been a subject of debate among scholars. We investigated the possibility of underlying epilepsy in Socrates by analyzing pathographical evidence in ancient literature from the viewpoint of the current understanding of seizure semiology. In 399 BCE, Socrates was tried and executed in Athens on the charge of “impiety.” His charges included the “introduction of new deities” and “not believing in the gods of the state,” because he publicly claimed that he was periodically and personally receiving a “divine sign,” or daimonion, that directed him in various actions. We found textual evidence that his daimonion was probably a simple partial seizure (SPS) of temporal lobe origin. It was a brief voice that usually prohibited Socrates from initiating certain actions. It started when he was a child, and it visited Socrates unpredictably. Moreover, we also found at least two descriptions of Socrates’ unique behavior that are consistent with complex partial seizure (CPS). The fact that Socrates had been experiencing both SPS and CPS periodically since childhood, makes the diagnosis of temporal lobe epilepsy (TLE) likely. We hypothesize that Socrates had a mild case of TLE without secondary generalization. This is the first report in 2400 years to present a pathographical diagnosis of TLE in Socrates based on specific diagnostic features in the ancient textual evidence. Our study demonstrates that the knowledge of modern epileptology could help understand certain behaviors of historical figures. Epilepsia 2006;47(3).EEG Features in Idiopathic Generalized Epilepsy: Clues to Diagnosis
Luiz Eduardo Betting, Susana Barreto Mory, Iscia Lopes-Cendes, Li M. Li, Marilisa M. Guerreiro, Carlos A. M. Guerreiro, and Fernando Cendes
Idiopathic generalized epilepsies (IGE) are frequently-encountered age-related epilepsies in people with genetic predispositions. Recognition of IGE is important for the choice of the antiepileptic drug. IGEs are divided in sub-syndromes, according to the main seizure type and age of onset. The electroencephalogram (EEG) is an important exam used for evaluation of epilepsies. When EEG shows generalized discharges it supports the diagnosis of IGE. However, in some patients with IGE, EEGs can be normal or disclose focal abnormalities. The objectives of this study were to investigate the EEG profile and its contribution for diagnosis and management in a group of patients with IGE, in 180 consecutive patients with IGE. The EEGs were classified as typical (generalized discharges), atypical (with focality or asymmetry) and normal. A total of 493 EEG exams were analyzed. The first EEG was normal in 45% of the 180 patients. Only 33% had typical abnormalities. Comparisons between IGE subsyndromes showed differences in the EEG profile. In some subsyndromes, the typical generalized discharges were more likely to be found compared to others. These findings support previous recommendations that IGE patients should be treated with appropriate therapy based on clinical history. Waiting for a typical abnormal EEG pattern can generate a delay in the correct diagnosis and treatment of these patients. In patients with long-term epilepsy, the diagnosis may be difficult. Furthermore, serial EEGs can help to elucidate the syndromic diagnosis, especially in patients with tonic-clonic seizures only and adult onset idiopathic generalized epilepsy. Epilepsia 2006;47(3).
Epileptogenicity of Supratentorial Medullary Venous Malformation
Takato Morioka, Kimiaki Hashiguchi, Shinji Nagata, Yasushi Miyagi, Fumiaki Yoshida, Futoshi Mihara, Ayumi Sakata, and Tomio Sasaki
Abnormal blood vessels in the brain, called vascular malformations, are common causes of epilepsy. Vascular malformations come in several types. Arteriovenous malformations (made of arteries and veins) and cavernous angiomas (made of capillaries) are known to produce seizures. The role of venous malformations is less clear. The purpose of this study was to evaluate the epileptogenicity of a type of venous malformation called a medullary venous malformations (MVM). Special consideration was given to any associations with intracerebral hemorrhage with or without other vascular malformations, including cavernous angioma (CA). A total of 10 patients with angiographically or histologically verified MVM were examined. The patients were divided into two groups with or without intracerebral hemorrhage, and their clinical, neuroradiological, interictal and ictal EEG findings were reviewed retrospectively. Although three of five patients in the non-hemorrhagic group presented with epilepsy, there was no concordance between the location of the MVM location and the EEG seizure focus. On the contrary, four of five patients in the hemorrhagic group developed epilepsy, and concordance between the hemorrhagic MVM location and the EEG focus was noted. One patient with a hemorrhagic MVM and an associated CA in the hippocampus had an electro-clinical picture of intractable medial temporal lobe epilepsy on this side. Although a supratentorial MVM itself is not epileptogenic, the development of an intracerebral hemorrhage may cause epilepsy. In particular, an associated CA may be highly epileptogenic. Epilepsia 2006;47(2).
Epilepsy in Colombia: Epidemiological Profile, Classification of Epileptic Seizures and Syndromes
Alberto Velez and Jorge Eslava-Cobos
A national study was performed in Colombia to determine the general and regional prevalence of Epilepsy, clinical profiles, seizure types and clinical syndromes. Based on the National Epidemiological Study of Neurological diseases (EPINEURO), we evaluated and followed for one year all the subjects with epilepsy from the National Sample. Clinical profiles were further assessed. Seizure types and Epileptic syndromes were established according to the international classifications. This paper presents the clinical and epidemiological data. General prevalence was found to be 11.3 per 1,000 with little variation among regions, except the eastern region where prevalence was 23 per 1,000; prevalence for active epilepsy was 10.1 per 1,000. Women have a slightly greater – not statistically significant – risk. Most seizures are focal (partial), often with secondary generalization. The most frequently encountered epileptic syndrome was Partial Symptomatic/Cryptogenic (80%). Epilepsy onset in Colombia occurs most commonly in childhood. Prevalence rates of Epilepsy in Colombia are similar to those reported in nations with comparable developmental status, and the rates have diminished over time. This study presents the distribution of seizures and syndromes for epilepsy in Columbia, with the most frequent types being focal epilepsy syndromes. Epilepsia 2006;47(1).
Somatic Comorbidity of Epilepsy in the General Population in Canada
José F. Téllez-Zenteno, Suzan Matijevic, and Samuel Wiebe
Patients with epilepsy often suffer from other chronic conditions (comorbidity) that contribute to poorer health, lower quality of life and impaired seizure control. Previous studies have explored comorbidity in patients attending clinics or at referral centers. We explored the magnitude of comorbidity in the general population, focusing on non-psychiatric illnesses. We examined the prevalence of epilepsy and 19 chronic, non-psychiatric conditions in two door-to-door Canadian health surveys, the National Population Health Survey (49,000 people) and the Community Health Survey (130,882 people). We obtained a ratio of chronic conditions in people with epilepsy versus in people without epilepsy, defined as the Prevalence Ratio (PR). For example, a condition with a PR of 1.5 indicates that it is one and a half times more frequent in people with than without epilepsy. Most chronic conditions were significantly more prevalent in people with than without epilepsy. Particularly frequent conditions (PR ?2.0) include stomach/intestinal ulcers (PR 2.5 - 2.7), stroke (PR 3.9 - 4.7), urinary incontinence (PR 3.2 - 4.4), bowel disorders (PR 2.0 - 3.3), migraine (PR 2.0 - 2.6), Alzheimer’s disease (PR 4.3), and chronic fatigue (PR to 4.1). The important message is that non-psychiatric comorbidity is common in people with epilepsy. Clinicians caring for people with epilepsy need to be keenly aware that these patients are ill in many ways and require a more comprehensive and integrated approach. Also, because chronic comorbid conditions may cause epilepsy, result from epilepsy, or share mechanisms with epilepsy, their recognition provides opportunities for investigating new causes and treatments of epilepsy. Epilepsia 2005;46(12).
The Impact of Epilepsy on Health Status Among Younger and Older Adults
Mary Jo V. Pugh, Laurel A. Copeland, John E. Zeber, Joyce A. Cramer, Megan T. Amuan, Jose E. Cavazos, and Lewis E. Kazis
New-onset epilepsy is most common among the elderly, yet our understanding of epilepsy in the elderly is based on inferences from studies of younger adults. We used data from national and survey databases from the Veterans Health Administration to examine the impact of epilepsy on patients’ perception of health status in adults of all ages. We compared scores on measures of physical and mental health status for patients in three age groups: young adults (18-40 years), middle-aged adults (41-64 years), and older adults (65 years and older). Because the time since epilepsy onset may influence these outcomes, we compared scores for patients with new-onset epilepsy, chronic epilepsy and no epilepsy, adjusting scores for patient characteristics that may also affect health status. Young adults with new-onset epilepsy reported poor general health and worse mental health, but high levels of physical function and physical activity. Middle-aged patients with epilepsy scored lowest in all domains. While older adults with epilepsy had the lowest scores on measures of physical functioning, once the effects of other disease states were controlled, their scores on physical functioning were higher than those of middle-aged adults with epilepsy.
While older adults have fewer physiological reserves, they appeared most resilient in facing this chronic illness, and middle-aged adults fared the worst. It is possible that societal expectations for middle-aged adults make adaptation to epilepsy more difficult than for older adults. Interventions to improve quality of life among patients with epilepsy should be tailored to age and time since epilepsy onset. Epilepsia 2005;46(11).Prevalence and Characteristics of Epilepsy in the Aeolian Islands
Giuseppe G. Gallitto, Salvatore S. Serra, Paolino P. La Spina, Paolo P. Postorino, Angela A. Laganà, Fortunata F. Tripodi, Santo S. Gangemi, Salvatore S. Calabrò, Rodolfo R. Savica, Raoul R. Di Perri, Ettore E. Beghi, and Rosa R. Musolino
In most developed countries, the prevalence of active epilepsy (proportion of persons with at least one seizure in the previous five years) usually ranges between 5 and 10 cases per 1,000 population. However, the rates reported in the general population of different geographical areas are widely varying. In Italy, prevalence rates were from 3.3 in the south (Sicily) to 6.2 in the North of the peninsula.
We investigated the prevalence and characteristics of epilepsy on a particular setting, the Aeolian archipelago, made up of seven small islands off the northern Tyrrhenian coast of Sicily (13,431 inhabitants). The subjects were identified from all available medical information sources by the neurologists of our group. They were then evaluated by the epileptologists using a standardized questionnaire and reviewed by the whole research team to confirm the diagnosis. For a more detailed definition, some patients underwent instrumental investigations. At the specified time (prevalence day), 1 June 1999, the rate of active epilepsy (point prevalence) was 3.1. Epilepsies with partial seizures, related to documented or supposed brain damage, were the most common types. The prevalence of epilepsy in the Aeolian Islands, as well as in Sicily, is lower than in other developed areas, including northern Italy, perhaps due to local factors, both health care and cultural. In the archipelago, no special services are available and geographical constraints limit the possibility of using medical facilities. Prejudice regarding the disease is common, and there is a tendency to hide the disease, due the possible social disadvantages. Epilepsia 2005;46(11).
Focal Semiological and Electroencephalographic Features in Patients with Juvenile Myoclonic Epilepsy
Naotaka Usui, Prakash Kotagal Riki Matsumoto, Christoph Kellinghaus, and Hans Otto Lüders
Juvenile myoclonic epilepsy (JME) is an idiopathic generalized epilepsy that typically presents with generalized tonic-clonic, myoclonic, and/or absence seizures. Typically, the seizures are bilaterally symmetric, and EEG shows generalized interictal and ictal epileptiform activity. We examined focal clinical and EEG features in 26 patients diagnosed with JME on the basis of seizures recorded during inpatient video-EEG monitoring. Their age at seizure onset was 0 to 22 (mean 12.3) years, and age at time of monitoring was 12 to 44 (mean 26.5) years. Three patients had a combination of focal epilepsy and JME. Focal features were noted in seizure semiology in 12/26 (46%) patients. Six patients had focal myoclonic seizures and two had Figure 4 sign:- one with version (twisting) to the left, and another had left version followed by Figure 4 sign, and left arm clonic seizure. Their ictal EEGs was generalized at the onset but with a lateralized evolution over the right hemisphere. In the patient with JME and left parieto-occipital epilepsy, right arm clonic seizure and Figure 4 sign were seen during a generalized EEG seizure. Interictally, one patient had temporal sharp waves and another had run of spikes in the right frontal region. In conclusion, fourteen of 26 patients (54%) with JME exhibited focal semiological and/or electroencephalographic features. Video-EEG was essential in reaching a correct diagnosis and choosing an appropriate treatment regimen. Epilepsia 2005;46(10).The Prognostic Value of 18F-FDG PET in Non-refractory Partial Epilepsy
Lars Weitemeyer, Christoph Kellinghaus, Matthias Weckesser, Peter Matheja, Tobias Loddenkemper, Gerhard Schuierer, Otmar Schober, E. Bernd Ringelstein, and Peter Lüdemann
18F-Fluor-desoxyglucose (FDG) is a radioactive metabolite used to measure glucose uptake and metabolism with positron emission tomography (PET). Regions of diminuished glucose metabolism in the brain are a significant help in localizing the seizure onset zone in patients with epilepsy unresponding to anticonvulsant medication. In these patients, the presence of such a hypometabolic region increases the chances of successful epilepsy surgery. This study was carried out to determine whether regions of diminuished glucose metabolism do have prognostic value also in patients not yet unresponsive to anticonvulsant medication. Ninety of those patients where investigated with FDG-PET relatively early in the course of their epilepsy. Magnetic resonance tomography was used to exclude visible brain lesions. In addition, all relevant clinical data was recorded. At least two years later, the patients where contacted again using a standardized telephone interview to determine their current seizure frequency and anticonvulsant therapy. Multiple logistic regression was carried out to analyze whether regional abnormalities of brain glucose metabolism were associated with a lesser chance of seizure freedom later on. It turned out that information from standard data alone (seizure symptoms, EEG, anticonvulsive treatment at the time of the PET investigation) were the only significant factor associated with the chance of seizure freedom after two years. However, patients with regional PET abnormalities more frequently were found with worsening of their seizures in spite of anticonvulsant treatment. As a consequence, PET should not be used as a routine screening tool to sort out epilepsy patients with a poor prognosis. However, if PET data are available, they could help in identifying patients with a less benign course. Epilepsia 2005;46(10).Association Analysis of Malic Enzyme 2 Gene Polymorphisms with Idiopathic Generalized Epilepsy
Kirsten P. Lenzen, Armin Heils, Susanne Lorenz, Anne Hempelmann, and Thomas Sander
Linkage disequilibrium mapping revealed allelic and haplotypic associations between single nucleotide polymorphisms (SNPs) of the gene encoding the malic enzyme 2 (ME2) and adolescent-onset idiopathic generalized epilepsy (IGE). Homozygote carriers of the associated ME2 haplotype had a six-fold higher risk for IGE compared with any other genotype. The present population-based association study tested whether genetic variation of the ME2 gene confers susceptibility to common IGE syndromes in the German population. The study included 666 German healthy control subjects and 660 German IGE patients (IGE group), of which 416 patients had an age of onset in adolescence (IGEado group). Genotyping was performed for six SNPs and one dinucleotide repeat polymorphism, all located in the ME2 region. Neither allele nor genotype frequencies of any ME2 polymorphism differed significantly between the controls and the IGE groups (P > 0.22). There was no hint for an association of the putative risk-conferring haplotype, when present homozygously, in both IGE groups compared with controls (P > 0.18). The present results do not support previous evidence that genetic variation of the ME2 gene predisposes to common IGE syndromes. Thus, if there is a recessively inherited ME2 mutation then the size of the epileptogenic effect might be too small or not frequent enough to detect it in the present IGE sample. Epilepsia 2005;46(10).Subcortical Nuclei Volumetry in Idiopathic Generalized Epilepsy
Margitta Seeck, Sylvain Dreifuss, Goran Lantz, Pierre Jallon, Giovanni B. Foletti, Paul-Andre Despland, Jacqueline Delavelle, and Francois Lazeyras
Idiopathic generalized epilepsy (IGE) is characterized by a normal neurological examination, a normal brain scan or MRI, and a particular type of abnormal EEG pattern. The exact anatomical and neurophysiological correlates are not well understood but the thalamus, a structure in the center of the brain that transmits almost all of the in- and outgoing information, has been invoked as the responsible factor. Diffuse changes in the ratio of grey matter (cortex) vs white matter (subcortical structures, to which the thalamus belongs) have been described but no actual thalamic anatomical abnormalities have yet been detected. We were interested to see if subcortical structures other than thalamus were different from normal in patients with IGE. We therefore measured the volume of several distinct structures, i.e. caudate nucleus, putamen, pallidum as well as the thalamus, in 11 patients with IGE and compared these values with those of 15 healthy control subjects. Similar to previous reports, the thalamic volumes were not different. However, smaller putamen in both hemispheres were found in the IGE patient group. The putamen mainly modulates the information to and from the frontal cortex, including the motor and certain neuropsychological functions, and it is speculated that discrete abnormalities of this structure contribute to the clinical picture of IGE. Epilepsia 2005;46(10).
Monozygous Twin Brothers Discordant for Photosensitive Epilepsy: First Report of Possible Visual Priming in Humans
Gerrit-Jan de Haan, Dorothee Kasteleijn–Nolst Trenité, Hans Stroink, Jaime Parra, Rob Voskuyl, Marian van Kempen, Dick Lindhout, and Ed Bertram
There is no indication that epileptic seizures have permanent effects on the human brain. This study describes a 16-year old boy that had an epileptic seizure after repeated exposures to disco lights. Since then he had jerks in the shoulders and eyelids when exposed to strong lights. His monozygous twin brother had none of these symptoms, but he had not been exposed to the particular strong lights. Both twins had generalized epileptic responses in their EEGs provoked by visual stimulation, but only in the affected boy the response was accompanied by clinical symptoms. This study suggests permanent effects of repeated exposures to strong lights in a visual sensitive person. Epilepsy can be primed in certain rodent models by repeated stimulations, but the effect has not been described in men or in primates. Epilepsia 2005;46(9).Predicting Posttraumatic Epilepsy with MRI: Prospective Longitudinal Morphological Study in Adults
Anna Messori, Gabriele Polonara, Flavia Carle, Rosaria Gesuita, and Ugo Salvolini
Posttraumatic epilepsy (PTE) is a well-recognized complication of head injury, and there is open discussion on its pathogenesis. Focal brain lesions documented in the acute stage represent accepted risk factors, but the relation between PTE and posttraumatic brain abnormalities as revealed by magnetic resonance imaging (MRI) has not been established. We prospectively evaluated morphological risk factors for PTE by using a panel of MRI sequences in serial assessments up to 2 years after trauma. MRI abnormalities indicative of gliosis and hemosiderin, respectively, were assessed in the images of the 135 adults who completed the study; clinical follow-up was 5-10 years. Statistical analysis of the results showed that sequelae of focal brain lesions that required surgical treatment (subdural hematomas-contusions) were a significant PTE risk factor, as were some, but not any sequelae of nonsurgical hemorrhagic contusions. With the combined use of different MRI sequences, we identified three patterns of morphological appearance of these, and those showing hemosiderin dregs completely surrounded by gliosis since early follow-up MRI (50% of the total number of lesions) were not, at difference with those showing gliosis incompletely surrounding hemosiderin dregs and time-related changes from incomplete to complete gliosis wall around hemosiderin. Although our results require confirmation from further MRI studies because of several limitations, it would appear that follow-up MRI examination in the early chronic stage can differentiate among low, intermediate and high-risk sequelae of head injury, and that follow-up MRI studies can yield new evidence to the still open debate on posttraumatic epileptogenesis. Epilepsia 2005;46(9).Photic- and Pattern-induced Seizures: A Review for the Epilepsy Foundation of America Working Group
Robert S. Fisher,Graham Harding , Giuseppe Erba, Gregory L. Barkley, and Arnold Wilkins
This report summarizes background material presented to a consensus conference on visually-provoked seizures, convened by the Epilepsy Foundation of America. A comprehensive review of literature was performed. Photosensitivity, an abnormal EEG response to light or pattern stimulation, occurs in approximately 0.3 – 3% of the population. The estimated prevalence of seizures from light stimuli is approximately 1 per 10,000, or 1 per 4,000 individuals 5-24 years old. People with epilepsy have a 2-14% chance of having seizures precipitated by light or pattern. In the Pokemon cartoon incident in Japan, 685 children visited a hospital in reaction to red-blue flashes on broadcast television. Only 24% who had a seizure during the cartoon had previously experienced a seizure. Photic or pattern stimulation can provoke seizures in predisposed individuals, but such stimulation is not known to increase the chance of subsequent epilepsy. Intensities of 0.2 – 1.5 million candlepower are in range to trigger seizures. Frequencies of 15-25 Hz are most provocative, but the range is 1-65 Hz. Light-dark borders can induce pattern-sensitive seizures, and red color also is a factor. Seizures can be provoked by certain TV shows, movie screen images, videogames, natural stimuli (e.g, sun on water), public displays, and many other sources. Recommendations on reducing risk of seizures have been developed by agencies in the UK, Japan, and the International Telecommunications Union, affiliated with the UN. The Epilepsy Foundation of America has developed a consensus of medical experts and scientists on this subject, reported in an accompanying work. Epilepsia 2005;46(9).Photic- and Pattern-induced Seizures: Expert Consensus of the Epilepsy Foundation of America Working Group
Graham Harding, Arnold J. Wilkins, Giuseppe Erba, Gregory L. Barkley, and Robert S. Fisher
In August 2004, the Epilepsy Foundation of America convened a workshop to begin to develop an expert consensus on photosensitive seizures. Literature and data were reviewed, and consensus derived from discussion. A flash is a potential hazard if it has luminance at least 20 cd/m2, occurs at a frequency of least 3 Hz, and occupies a solid visual angle of at least 0.006 steradians (about 10% of the central visual field or 25% of screen area at typical viewing distances). A transition to or from saturated red also is considered a risk. A pattern with the potential for provoking seizures contains clearly discernible stripes, numbering more than five light-dark pairs of stripes in any orientation. When the light-dark stripes of any pattern collectively subtend at the eye from the minimum expected viewing distance a solid angle of more than 0.006 steradians, the luminance of the lightest stripe is greater than 50 cd/m2, and the pattern is presented for 0.5 seconds or longer, then the pattern should display no more than five light-dark pairs of stripes, if the stripes change direction, oscillate, flash or reverse in contrast; If the pattern is unchanging or smoothly drifting in one direction, no more than eight stripes. These principles are easier to apply in the case of fixed media, for example, a pre-recorded TV show, which can be analyzed frame-by-frame, as compared to interactive media. A consensus view of stimuli likely to provoke visually-evoked seizures can be developed. Epilepsia 2005;46(9).
Socioeconomic Status is a Risk Factor for Epilepsy in Icelandic Adults but not in Children
Dale C. Hesdorffer, Hong Tian, Kishlay Anand, W. Allen Hauser, Petur Ludvigsson, Elias Olafsson, and Olafur Kjartansson
Two earlier population-based studies provide conflicting information on the association between low socioeconomic status (SES) and risk for epilepsy. Seizure etiologies (e.g., head injury, stroke) associated with low SES were not addressed in prior analyses. We set up a surveillance system in Iceland in order to identify all incident unprovoked seizure or first diagnosis of epilepsy throughout Iceland (N=418) during the study period. Each case was matched to two controls selected from the Icelandic population registry as the next two same sex births alive, and without a history of unprovoked seizure on the date of the case’s incident seizure (N=835). We found an association between epilepsy and SES among adults, but not among children. Among adults, low education increased the risk for epilepsy 2.29-fold (95% CI=1.21-4.34) and home ownership was protective (OR=0.63, 95% CI=0.43-0.92). When analyses were repeated by seizure etiology, the association remained only in the group with epilepsy of unknown cause, even after adjusting for alcohol consumption. Low SES, indexed by low education or lack of home ownership, is a risk factor for epilepsy in adults, but not in children, suggesting a cumulative effect of SES on risk for epilepsy. This association is not explained by established risk factors for epilepsy (e.g., head injury, stroke). Additionally, we find no evidence of a downward social drift among cases whose parents had epilepsy. Epilepsia 2005;46(8).Post Stroke Epilepsy – Occurrence and Predictors: A Long-term Prospective Controlled Study
Morten I. Lossius, Ole M. Rønning, Geir D. Slapø, Petter Mowinckel, and Leif Gjerstad
Stroke is by far the most common known cause of epileptic seizures in the elderly, and there are about 700,000 new strokes every year in the United States. However, the risk for development of epilepsy after a stroke varies a lot in the literature. We studied the frequency of epilepsy after ischemic stroke (caused by blood clotting) and factors that may influence this frequency in a long-term study (7-8 years after stroke). In particular, we wanted to investigate if treatment in stroke units could reduce the frequency of epilepsy after stroke by comparing the frequency of epilepsy in those treated in stroke units to those treated in general medical ward. We found that 3.1% of the stroke patients developed epilepsy, i.e., recurrent seizures more than one week after the stroke. We found no significant reduction in the frequency of epilepsy in stroke patients treated in stroke units, compared to general medical ward. Stroke severity measured by the so-called Scandinavian Stroke Scale was, however, a significant predictor for post-stroke epilepsy. These findings suggest that present treatment in stroke units does not prevent the development of epilepsy after stroke. Modern treatment of stroke with thrombolysis, which immediately dissolves the blood clot, or new drugs that protect the brain cells (neuroprotective drugs), may decrease the frequency of post-stroke epilepsy. New studies are needed to study how to prevent post-stroke epilepsy. Epilepsia 2005;46(8).Epileptogenesis due to Peripheral Injury as a Cause of Focal Epilepsy
Allison E. Spiller, Alan Guberman, Fabrice Bartolomei, Benjamin Zifkin, and Frederick Andermann
This paper describes 3 patients who developed focal seizures after painful soft tissue injuries to one hand.
The seizures began soon after injury and started in the injured hand. All the subjects had normal MRIs. Although there was no evidence of structural brain damage, one had an indication on PET scanning of an
area of functional abnormality, which may have been a factor predisposing to seizures. All three cases had some finding, clinical, electroencephalographic, or imaging consistent with the possibility of contralateral cortical reorganization. The seizure patterns also argue for the involvement of cortical mechanisms. The idea that peripheral injuries can modify brain function is not new. There are many examples of central neuronal plasticity and reorganization that result from either peripheral stimulation or injury in animals and in humans, but it is not clear how cortical reorganization occurs or why it should lead to seizures in some cases. Presumably, the injuries led to plastic changes in the sensory/motor cortex resulting in increased excitability and ultimately in seizures. These findings suggest the possibility that epileptogenesis (the process of creating epilepsy in the brain) can occur in response to painful peripheral stimuli. Epilepsia 2005;46(8).Familial Temporal Lobe Epilepsy as a Presenting Feature of Chorea Acanthocytosis
Abdullah Al-Asmi, An C. Jansen, AmanPreet Badhwar, François Dubeau, Donatella Tampieri, Chaim Shustik, Suha Mercho, Ghislaine Savard, Carol Dobson-Stone, Anthony P.Monaco, Frederick Andermann, and Eva Andermann
Chorea-acanthocytosis (CHAC) is an inherited disorder caused by mutations in the gene called VPS13A located on chromosome 9q21. The disease is characterized by involuntary movements including chorea (fine dancing movements of the limbs), abnormal movements of the mouth or the tongue, tics, slurred speech, difficulty swallowing, absent reflexes, seizures and psychiatric problems. Blood smears show red cells that have an abnormal “spiky” shape and are called acanthocytes. Seizures are common in CHAC, but have not been well characterized in the literature. We report two CHAC families in which patients presented with epilepsy. Six patients in three sibships presented with seizures. Age at seizure onset ranged from 22 to 38 years. Seizures preceded other clinical manifestations of CHAC by up to 15 years, delaying diagnosis. Attacks could start with a sensation of déjà-vu, fear, hallucinations, palpitations or dizziness. Treatment of epilepsy in CHAC patients represents a challenge, since seizures may at times be difficult to control, requiring multiple anti-epileptic drugs, and some anti-epileptic drugs may worsen the involuntary movements. The diagnosis should be considered in patients who have both seizures and a movement disorder, have one and later develop the other, or have a family history of both movement disorders and epilepsy. Affected individuals in both families were missing a part of the VPS13A gene, as well as a part of the GNA14 gene that lies next to VPS13A on the same chromosome. Epilepsia 2005;46(8).Characterizing the Patterned Images that Precipitate Seizures, and Optimizing Guidelines to Prevent Them
Arnold J. Wilkins, John Emmett, and Graham F.A. Harding
Television can induce seizures in patients with photosensitive epilepsy partly because of the rapid flicker from the screen but also because of programs such as advertisements that include flickering images and prominent geometric patterns. In Britain and Japan, the use of guidelines to prevent the broadcast of material of this kind has reduced the incidence of seizures. We estimated the proportion of patients likely to be affected by flicker and patterns and used this information to develop a set of easy-to-use rules that keep the level of risk to a consistent minimum. We propose that striped patterns lasting longer than 0.5 seconds, and having bright stripes that occupy more than one quarter the area of the television screen should be restricted. If the stripes are static, then no more than 8 on screen at a time should be permissible, and if the stripes flash, no more than 5 should be allowed. Adherence to these guidelines would protect at least two thirds of susceptible patients. Epilepsia 2005;46(8).Pattern Reversal Visual Evoked Potentials in Patients with Newly Diagnosed Epilepsy
Bülent O. Genc, Emine Genc, Figen Güney, and Nurhan Ilhan
Evoked potential tests measure electrical activity in certain areas of the brain in response to stimulation of specific sensory nerve pathways. These tests can indicate dysfunction along these pathways that is too subtle to be noticed by the person or to show up on neurologic examination. Visual evoked potentials evaluate the visual nervous system from the eye to the brain. In order to measure visual evoked potentials, wires are placed on the scalp over the areas of the brain being tested. The patient sits before a screen on which an alternating checkerboard pattern is displayed and the patient’s response to this specific visual stimulation is recorded. In this study, VEPs were recorded in newly diagnosed, and previously untreated epilepsy patients. These were idiopathic epilepsy patients in whom no structural abnormality of the brain was demonstrated by computerized tomography or MRI. Patients who were under treatment were not included because antiepileptic medications can influence evoked potentials. Results obtained from epileptic patients demonstrated that nerve impulses were either slowed or accelerated when compared to those obtained from healthy volunteers. Slowed responses were observed in idiopathic epilepsy patients who were not sensitive to photic stimulation whereas accelerated responses were observed in patients who were sensitive to photic stimulation. These changes might be the result of a disorder of one or more neurotransmitters, including GABA which is the most important inhibitor neurotransmitter in the brain, or of minor morphological changes in the brain called “microdysgenesis”. Epilepsia 2005;46(8).
Epilepsy and Neurocysticercosis in Rural Bolivia: A Population-based Survey
Alessandra Nicoletti, Alessandro Bartoloni, Vito Sofia, Filippo Bartalesi, Josè Rosado Chavez, Rimberto Osinaga, Franco Paradisi, Jean-Luc Dumas, Victor C.W. Tsang, Arturo Reggio, and Andrew J. Hall
Neurocysticercosis has been reported as the most frequent parasitic infection of the central nervous system, and a major cause of epilepsy in several developing countries. We estimated the frequency of NCC in a well defined population of epileptic patients living in a rural area of the Cordillera Province, Bolivia. We carried out a door-to-door neuroepidemiological survey in a sample of 10,124 subjects to estimate the prevalence of epilepsy. A standard screening instrument for neurologic diseases was adopted; subjects positive at the screening phase underwent a complete neurological examination. Epilepsy was diagnosed according to the definition proposed by the ILAE in 1993 and seizure types were identified on the basis of the classification proposed by ILAE in 1981, considering both electroencephalographic and clinical data. In order to evaluate the frequency of NCC, epileptic patients identified during the survey underwent CT scan and serological evaluation to detect antibodies against T. solium by enzyme-linked immunoelectrotransfer blot. Neurocysticercosis was diagnosed according to the diagnostic criteria proposed by Del Brutto in 2001.
We detected 124 defined prevalent epileptic patients of whom 66 patients (53.3%) presented partial seizures. Of the 124 patients, 105 underwent CT scan while a serum sample was taken in 112 patients; for 97 patients both neuroradiological and serological data were available. Considering radiological, serological and clinical features, of the 124 patients 34 (27.4%) fulfilled the criteria for NCC. Of these 34 patients 24 (70.6%) presented partial seizures. Our data confirm an high frequency of NCC among a well defined prevalent cohort of epileptic patients. Epilepsia 2005;46(7).
Gender Differences in Epilepsy
Jakob Christensen, Marianne Juel Kjeldsen, Henning Andersen, Mogens Laue Friis, and Per Sidenius
Gender differences were noted in two large Danish epilepsy populations of 2,170 adult outpatients registered in the Danish National Epilepsy Database (EpiBase) at the Department of Neurology, Aarhus University Hospital. In this database, 318 children and adults were registered in the Danish Twin Registry. Women were more likely than men to develop idiopathic generalized epilepsy, and men were more likely to develop symptomatic focal epilepsy. The male preponderance of symptomatic localization-related epilepsy is probably related to the higher risk of damage to the central nervous system from tumors and trauma. The reason behind the female preponderance in idiopathic epilepsy is largely unexplained, but may relate to the effects of sex-hormones. Further analyses in EpiBase showed that localization-related epilepsies without obvious cause (cryptogenic) were more frequent in women. Thus, in general it therefore seems that non-symptomatic epilepsy (both idiopathic generalized and cryptogenic localization-related epilepsy) more often occur in women in contrast to symptomatic localization-related epilepsy that has preponderance in men. The results suggest a gender susceptibility to the development of specific epilepsy subtypes and should be further explored. Epilepsia 2005;46(6).
A Comparison of Epilepsy Patients in a Traditional Ambulatory Clinic and a Telemedicine Clinic
Karen A. Rasmusson and Jeanette C. Hartshorn
Telemedine is medical care at a distance, assisted by modern communication and computer technology. Patients are attended by a local nurse, technician or non-specialist physician, and the video image and sound is sent in near-real-time to a specialty clinic. A joint project between the UTMB Telehealth Center, the Epilepsy Foundation of Southeast Texas, and the Texas Department of Health, compared the outcomes of care from a traditional epilepsy clinic in the ambulatory care center of a large academic medical center with the outcomes in a telemedicine epilepsy clinic.
Demographic and outcome data were collected on patients in both clinics for three months in 2004. The authors gathered data on the number of seizures, hospitalizations, and emergency room visits. In addition, medication compliance was also measured by self-report and medication levels. There were no significant differences in any of the demographic data or outcome measures between the two groups.
Telemedicine is an acceptable alternative to in-person clinics for the provision of care to adults with epilepsy. Since telemedicine programs bring medical care closer to where patients live, these clinics provide an excellent alternative to provide consistent, specialty care in rural and geographically isolated areas. Additional studies are needed to investigate the potential costs associated with telemedicine as well as the potential for cost savings over time as patients are more able to access specialty care. Epilepsia 2005;46(5).Development of a Validated Clinical Case Definition of Generalized Tonic-Clonic Seizures for Use by Community-based Health Care Providers
Krishnan Anand, Satish Jain, Eldho Paul, Achal Shrivastava, Sirazul Ameen Sahariah, and Suresh K. Kapoor
Epilepsy is a disease characterized by sudden movement of body and limbs due to discharge of electrical impulse in the brain. There are no proven confirmatory laboratory tests for its diagnosis and doctors often rely only on the detailed history to arrive at the diagnosis. It is estimated that almost 80% of epilepsy patients are not being treated. If the diagnosis of epilepsy is made simpler then it is possible that this treatment gap could be minimized. The type of epilepsy called as Generalized Tonic-Clonic Seizures (GTCS) are amenable to easy diagnosis by their presentation. This paper tries to identify points in the detailed history used by neurologists which can be used by community based health care providers after training to come to a conclusion as to whether a patient coming to them with a symptom of rigidity or jerkiness of limbs or body has GTCS. The study compares the reported symptomatology of patients with GTCS and non-GTCS patients with seizures in India. It identifies six questions based on the data analysis and clinical experience that can help in this decision-making . These six questions are: absence of stress, presence of frothing, event occurring during sleep, incontinence during the episode, unconsciousness and injury during the episode. A case definition consisting of giving one point to an affirmative answer for each of the six questions was tested in five countries of the South East Asia Region. At a cut-off point of four out of six, this case definition rightly identified 72% of GTCS cases and all non-GTCS cases. The success of this simple question screen may make it easier to identify people with epilepsy in areas lacking sophisticated medical testing and systems. Epilepsia 2005;46(5).
Epilepsy and Neurocysticercosis in Atahualpa: A Door-to-Door Survey in Rural Coastal Ecuador
Oscar H. Del Brutto, Rocío Santibáñez, Luis Idrovo, Silvia Rodríguez, Enrique Díaz-Calderón, Carlos Navas, Robert H. Gilman, Fausto Cuesta, Aurelio Mosquera, Armando E. Gonzalez, Victor C.W. Tsang, and Héctor H. García
Cysticercosis results from a microscopic worm derived from being in contact with infected bad pork, or sections from an infected human. The worm can spread to the brain and cause seizures. Seizures may persist even after the worm has died. Cysticercosis is one of the most important causes of epilepsy in the world, especially the developing world. The authors carried out a door-to-door community survey to determine the prevalence of epilepsy and the role of neurocysticercosis in the occurrence of epilepsy in Atahualpa, a rural population of coastal Ecuador. During the first phase of the study rural doctors interviewed subjects aged 15 years or older using a validated questionnaire to detect subjects with epileptic seizures, and obtained a blood sample of consenting individuals to point to prior infection with cysticercosis. In the second phase, neurologists examined all suspected people in the community with cysticercosis, as well as a sample of non-infected individuals to determine the actual number of persons with epilepsy. Then, patients with epilepsy, as well as age- and sex-matched controls, underwent a computed tomography (CT) scan of the head. The questionnaire was answered by 2,415 of 2,548 residents of Atahualpa, and cysticercosis blood testing was performed in 1,687 individuals. Overall, cysticercosis tests in blood indicative of prior infection was 8.6%. Neurological examination confirmed 24 patients with epilepsy (crude prevalence of 9.9 per 1,000 population, and 10.8 per 1,000 when adjusted to the US population). A positive cysticercosis blood test was strongly associated with epilepsy. In addition, CT findings compatible with neurocysticercosis were more frequent among patients with epilepsy than in controls. It is concluded that neurocysticercosis is associated with one third of cases of epilepsy in Atahualpa, and may be a major contributory factor for the excess fraction of epilepsy seen in this population. Epilepsia 2005;46(4).Epileptic Seizures and Epilepsy: Definitions Proposed by the International League Against Epilepsy (ILAE) and the International Bureau for Epilepsy (IBE)
Robert S. Fisher, Walter van Emde Boas, Warren Blume, Christian Elger, Pierre Genton, Phillip Lee, and Jerome Engel, Jr.
The International League Against Epilepsy (ILAE) and the International Bureau for Epilepsy (IBE) have come to consensus definitions for the terms “epileptic seizure” and “epilepsy.” An epileptic seizure is a transient occurrence of signs and/or symptoms due to abnormal excessive or synchronous neuronal activity in the brain. Epilepsy is a disorder of the brain characterized by an enduring predisposition to generate epileptic seizures, and by the neurobiological, cognitive, psychological, and social consequences of this condition. The definition of epilepsy requires the occurrence of at least one epileptic seizure. These definitions eventually should help to standardize scientific, medical and social discussions of populations of individuals who have seizures. Epilepsia 2005;46(4).
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