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Knowledge of epilepsy among teachers in Khon Kaen Province, Thailand.

INTRODUCTION: Epileptic patients face social stigmatization due to negative attitudes and incorrect knowledge on epilepsy. OBJECTIVES: To evaluate knowledge of epilepsy among teachers in Khon Kaen province. MATERIAL AND METHOD: A self-administered questionnaire distributed to 102 teachers who attended the training lectures on epilepsy. The number of correct responses for each item were collected. The statistical analysis included the percentage of correct response and the means of the total scores. RESULTS: Most (78.4%) respondents understood that a seizure is an abnormal electrical discharge in the brain, while 54.9% thought it included a form of abnormal movement and 1% demonic possession. The generalized tonic-clonic seizure (GTCs) was the type of seizure with which most respondents were familiar (90.2%), while 23.5% had knowledge of absence seizures. The respondents identified the following as causes for epilepsy: 1) head injury (84%); 2) genetic disease (74.5%); 3) high fever (68%); and, 4) brain tumor (57%). A small minority associated epilepsy with eating pork (11%) and even fewer (2%) with a non-organic/non-physical cause. Only 16% of respondents thought epilepsy was incurable, and a quarter (27%) of the teachers thought epileptics required anti-epileptic drugs (AEDs) life long, while 20 and 9 percent believed patients would take AEDs for 3-6 months and only for episodes, respectively. About 57% of the teachers thought epileptics needed AEDs for 2-5 years. Most (77-79%) respondents thought epileptics were prohibited from using machinery or driving, and 63% thought alcohol would be prohibited. Almost two-thirds of the teachers (64%) thought they should try to place an object between the teeth of a person having an episode in order to prevent a biting injury to the tongue and 27% would restrain the person and perform chest compressions (CPR). The average total score for correct answers on the questionnaire was about 60% (29.26/50). Respondents generally understood that epilepsy is controllable (82%) and were able to identify a seizure (78.4%). The lowest scores were found in the section on identifying the types of seizures (37.8%). CONCLUSION: Teachers' knowledge of epilepsy was incomplete; thus, an epilepsy education campaign is needed and should emphasize the types of seizures, the causes of epilepsy, and management.

Developing Countries↗

[Controversies and problems in the diagnosis of benign occipital epilepsies in infancy, childhood and adolescence].

AIM: To review the changes introduced by the new proposal of diagnostic scheme for people with epilepsy made by the International League Against Epilepsy in the categorization of the idiopathic occipital epilepsies of childhood and the diagnostic challenges of these disorders. DEVELOPMENT: The term occipital paroxysms has disappeared and two major forms are distinguished: one with early onset or Panayiotopoulos type, officially recognized as a syndrome, and one with late onset or Gastaut type. To these entities we have to add the recently described idiopathic photosensitive occipital lobe epilepsy, also included in the new list of syndromes. Detailed electroencephalogram investigations are crucial to reveal all the neurophysiological diagnostic aspects of these disorders. These investigations may suggest a close relationship with rolandic epilepsies. The characterization of the Panayiotopoulos' syndrome as a focal occipital epilepsy has been questioned. The limits of the idiopathic photosensitive epilepsy with other photosensitive epilepsies are not well defined yet. The variant of Gastaut might be sometimes difficult to distinguish from migraine with visual aura. Its long term prognosis is not clear yet, in part because this overlap with other entities. The persistence of seizures beyond adolescence in some patients and the development of learning disorders and mild cognitive dysfunction cast some doubts about its good prognosis. CONCLUSIONS: The characterization of the idiopathic occipital epilepsies and its relationships with other childhood epilepsies are a fascinating area of development in epileptology. Further research is necessary to define the limits of these disorders and their long-term prognosis.

Adolescent↗

[The personality change in epilepsy patients].

UNLABELLED: Personality disorders in epilepsy patients are frequent, and cause behaviour that is quite often troublesome for social environment of patients. The peculiarity of the clinical picture and mechanism of disorder formation has caused great interest. The term 'characteropathy in epilepsia patient', with characteristic viscosity as its important basic symptom, was eliminated from present medical terminology. Current worldwide international disease classification systems (DSM-IV and ICD-10) do not distinguish the separate form of personality disorders related to epilepsy. THE AIM: of the work is to analyse personality change in epilepsy patients on the basis of information from the patients and their relatives by means of the Adjective Check List (ACL) by Gough and Heilbrun, with taking into consideration the incidence of depression and the phenomenon of 'out-of-body experience'. MATERIAL AND METHODS: 40 patients with idiopathic generalized epilepsy with tonic - clonic seizures in age 19-55 years (mean 28 years) and 30 persons of age - matched control groups were examined. Each examined person and relative filled in ACL form two times in order to answer a question about the features of actual and premorbid patient's personality. The change of personality was evaluated according to ACL criteria and the psychoterapeutic criteria. In analysis were also taken into account the incidence of depression and the phenomenon of 'out-of-body experience'. RESULTS: The personality change in epilepsy patients was more frequent than in the control group and more frequent concerned need scales of ACL (endurance, deference, self- and others understanding, and aggression scale). In the grave epilepsy group considerable behavioral and interpersonal relationships change (more subordination tendency), features of dementia, and often phenomenon 'out-of-body experience' were observed. The range of personality change in the mild epilepsy group, like in control group, included the adaptation of family functioning principles to the patients' needs, the change of patients' emotional reaction and the meaning of life. In this group depression was often, but the phenomenon 'out-of-body experience' hardly ever appeared. CONCLUSIONS: The personality change in epilepsy patients was found in more than 80% of patients and concerned need scales of ACL (endurance, deference, self- and others understanding and aggression scale), and thematic scales (self ideal, self-control, and self-confidence scale). In the grave epilepsy group considerable behavioral changes, features of dementia, and often the phenomenon 'out-of-body experience' were observed. In the mild epilepsy group the changes were limited to alter of conviction and emotional response, often depression appeared, but hardly ever the phenomenon 'out-of-body experience' was observed.

Adult↗

[Epilepsies in puberty and adolescence. Follow-up and drug therapy].

1. Puberty and adolescence mainly can have a bad effect on idiopathic generalized epilepsies. This can show up in the first onset of seizures in adolescence, e.g. as a form of idiopathic grand mal on awakening, juvenile absence epilepsy or juvenile myoclonic epilepsy (impulsive petit mal). Also, already diagnosed absence epilepsy can become worse, e.g. by recurring absence seizures or grand mal seizures or the new onset of grand mal on awakening. 2. Exogenous factors, like changing the time of sleeping and waking, lack of sleep and the abuse of alcohol, play an important role in these types of epilepsy. Cutting out these factors is just as important as the consequent drug treatment on a longterm basis. 3. Drug treatment of idiopathic generalized epilepsy will be done with valproate, barbiturates and ethosuximide. Regarding fatal complications of liver function, which are more frequent than previously thought of, valproate can be recommended as a drug of first choice, especially in early childhood and when a combination of drugs is used. 4. A very good effect of adolescence is seen in children with benign idiopathic focal epilepsy with a centro-temporal spike focus. Antiepileptic treatment with carbamazepine as an agent of first choice can be done less vigorously. 5. Menstruation usually only modifies but does not worsen the natural course of epilepsy. There is still no real therapeutical plan for this rare form of epilepsy. 6. It cannot be a general rule, not to change current medical treatment before or during puberty in order to avoid any worsening of seizures. A decision always has to be made regarding the form of epilepsy and the individual situation, which has to be discussed with the patient and perhaps his family.

Adolescent↗

Partial epilepsy manifesting atonic seizure: report of two cases.

PURPOSE: Atonic seizures are commonly seen in patients with generalized epilepsy but only infrequently in patients with partial epilepsy. Clinically generalized atonic seizures as a partial epilepsy have not been studied in detail with video/EEG monitoring. Here we describe the clinical and physiologic characteristics of atonic seizures due to partial epilepsy and discuss the underlying mechanism. METHODS: Two patients with partial epilepsy manifesting atonic seizures, one with frontal lobe epilepsy (FLE) and the other with parietal lobe epilepsy (PLE), were reported. The long-term video/EEG monitoring, magnetic resonance imaging (MRI), and interictal fluorodeoxyglucose-positron emission tomography (FDG-PET) were investigated in each patient. RESULTS: Paroxysmal diminution of muscle tone mainly involved the axial muscles in both patients. In contrast with the abrupt falls seen in patients with Lennox-Gastaut syndrome, the falls in these patients were slow, taking 2-5 s to fall down. Ictal EEG records showed low-voltage fast activity in the frontocentral area followed by repetitive spikes at the midline frontocentral area in the patient with FLE, and rhythmic spikes in the left central area in the patient with PLE. Interictal FDG-PET disclosed hypometabolic regions consistent with the clinical and EEG findings. CONCLUSIONS: Slow falls might be a feature of atonic seizures in partial epilepsy. Long-lasting atonia in partial epilepsy could be due to either one of the following two possible mechanisms: (a) epileptic activities arising from the negative motor area, of which 50-Hz electric stimulation causes motor inhibition, or (b) sustained atonia with successive electromyogram (EMG) silent periods caused by epileptic discharges arising from the inhibitory area of the primary sensorimotor area.

Adult↗

Adolescent seizures and epilepsy syndromes.

Specific epilepsy syndromes begin during adolescence and create a significant neurologic burden. Knowledge of these syndromes has important treatment and prognostic implications, which usually extend into adulthood. Little is known about the effect of menarche on seizures, even though a relationship of seizures to the menstrual cycle has been observed for many years. In general, puberty is not thought to influence seizure frequency. However, estrogen is thought to activate epileptiform activity; testosterone may decrease seizure activity; and progesterone decreases epileptiform discharges. These effects are mediated by effecting gammaaminobutyric acid (GABA) transmission. Idiopathic generalized epilepsies are the most frequent group with adolescent onset. These are probably polygenic in origin and represent a biologic continuum. Juvenile myoclonic epilepsy (JME) is the most common form. This contrasts with a variety of progressive myoclonic epilepsies that also are first seen in adolescence and have a genetic origin and specific treatments. Finally, although temporal lobe epilepsy associated with hippocampal sclerosis may have its origin in childhood, often the child does not come to surgical evaluation until adolescence or young adulthood. The characteristic clinical history, seizure semiology, and magnetic resonance imaging findings have allowed a discrete epilepsy syndrome to be established. Applying these same criteria to children and adolescents reveals that hippocampal sclerosis is the most common lesion responsible for their intractable temporal lobe epilepsy. Hippocampal sclerosis is probably underdiagnosed in children. The safety and efficacy of epilepsy surgery in the age group is excellent. Knowledge of the epilepsy syndromes that remit before adolescence, may persist into adolescence, or begin in adolescence is central to the treatment of this age group.

Adolescent↗

Remote memory in epilepsy.

PURPOSE: There is now a considerable amount of research relating to memory functioning in epilepsy. The majority of studies have focused on the retention of new information, and few reports have measured memory for past events. This study aims to redress this and measure the efficiency of remote memory in epilepsy. METHODS: A remote memory questionnaire was prepared and administered to three groups of patients with epilepsy and a control group without epilepsy. The questionnaire assessed knowledge of public events that occurred between 1980 and 1991, inclusive. The epilepsy groups comprised 33 patients with temporal lobe epilepsy (TLE), 33 with extratemporal epilepsy (ExTE), and 10 with primary generalized epilepsy (PGE). Thirty control subjects were tested. RESULTS: Patients with TLE performed significantly less well on the questionnaire than all other groups (p = 0.001), but no effect of laterality was recorded; patients with extratemporal or primary generalised epilepsy did not differ from controls. Performance on the questionnaire was not determined by verbal IQ, educational achievement, social class, or drug treatment, but was related to the number of generalised convulsions that had occurred since 1980. The strongest neuropsychological predictors of performance on this questionnaire were measures of verbal memory. CONCLUSIONS: The study demonstrated weak memory for past events in patients with TLE, thereby providing evidence of a broader memory disturbance in this group than has been previously highlighted. A test of remote memory, such as the one designed for this study, is easy to administer and provides clinically important information not available from conventional neuropsychological tests.

Achievement↗

Prevalence of active epilepsy in a rural area in South Tanzania: a door-to-door survey.

OBJECTIVE: A door-to-door survey was carried out to assess the prevalence of active epilepsy in the rural population within the catchment area of the Mnero Diocesan Hospital in Southern Tanzania. METHODS: A total of 4,905 individuals living in 1,047 households were enrolled in a study, consisting of a screening dialogue with a representative family member followed by a face-to-face interview with the affected subject. The diagnosis of epilepsy followed clinical guidelines proposed by the International League Against Epilepsy (ILAE). RESULTS: We detected 42 cases (21 males and 21 females) of active epilepsy (8.6/1,000; 95% CI: 6.0/1,000-11.1/1,000; age-adjusted prevalence ratio: 7.4/1,000). Thirty of them met the case definitions of generalized seizures, whereas 12 patients reported partial seizures. The peak prevalence was found in both sexes in the second decade of life (f: 16.9/1,000; m: 15.8/1,000; t: 16.4/1,000), with a second peak in the group aged 30-39 years (f: 10.4/1,000; m: 8.8/1,000; t: 9.7/1,000). Eleven patients (26.2%) were classified as "strongly suspected of symptomatic" epilepsy, the remaining 31 patients (73.8%) as possibly being idiopathic, symptomatic, or cryptogenic epilepsy cases. DISCUSSION: This study provides baseline data about the active epilepsy prevalence in a rural African population. Compared to western countries, our study confirmed a pattern toward higher prevalence of epilepsy in tropical countries. As shown in previous studies, we observed a higher prevalence of epilepsy in children and adolescents, followed by a steady decline with increasing age. Reasons for this type of frequency could be ascribed to malnutrition, trauma, genetic conditions, antenatal and prenatal care, diseases resulting in febrile convulsions, cerebral infections and may even result in death in specifically diseased individuals.

Adolescent↗

Prognostic factors in neocortical epilepsy surgery: multivariate analysis.

PURPOSE: Defining prognostic factors for neocortical epilepsy surgery is important for the identification of ideal candidates and for predicting the prognosis of individual patients. We use multivariate analysis to identify favorable prognostic factors for neocortical epilepsy surgery. METHODS: One hundred ninety-three neocortical epilepsy patients, including 91 without focal lesions on MRI, were included. Sixty-one had frontal lobe epilepsy (FLE), 80 had neocortical temporal lobe epilepsy (nTLE), 21 had parietal lobe epilepsy (PLE), and 22 had occipital lobe epilepsy (OLE). The primary outcome variable was patient status >or=2 years after surgery (i.e., seizure free or not). Clinical characteristics and the recent presurgical diagnostic modalities were considered as probable prognostic factors. Univariate and standard multiple logistic regression analyses were used to identify favorable prognostic factors. RESULTS: The seizure-free rate was 57.5%. By univariate analysis, a focal lesion on MRI, localized ictal onset on surface EEG, epilepsies other than FLE, localized hypometabolism on fluorodeoxyglucose-positron emission tomography (FDG-PET), and pathologies other than cortical dysplasia were significantly associated with a seizure-free outcome (p<0.05). Multivariate analysis revealed that a focal lesion on MRI (p=0.003), correct localization by FDG-PET (p=0.007), and localized ictal onset on EEG (p=0.01) were independent predictors of a good outcome. CONCLUSIONS: The presence of a focal lesion on MRI, correct localized hypometabolism on FDG-PET, or localized ictal rhythms on EEG were identified as predictors of a seizure-free outcome. Our results suggest that these findings allow the selection of better candidates for neocortical epilepsy surgery.

Adult↗

Prevalence of childhood epilepsy and distribution of epileptic syndromes: a population-based survey in Okayama, Japan.

PURPOSE: A population-based survey childhood epilepsy was undertaken in Okayama Prefecture, Japan, to determine the prevalence rate and the distribution of epilepsies and epileptic syndromes according to the International Classification (ILAE, 1989). METHODS: Information on patients younger than 13 years with active epilepsy was collected from medical records. Patients diagnosed with epilepsy according to clinical and EEG findings were put on the list even if those patients had had a single seizure or seizures occurring during febrile episodes. RESULTS: In total, 2,220 cases were identified from a background population of 250,997. The prevalence rate was 8.8 per 1,000. If we exclude patients who had experienced a single seizure or seizures occurring during febrile episodes to compare our results with previous reports, the prevalence rate was 5.3 per 1,000. Of the 2,220 cases, 2,030 (91.4%) were classified into three major categories by ILAE classification. They consisted of 1,556 (76.7%) with localization-related epilepsy, 453 (22.3%) with generalized epilepsy, and 21 (1.0%) with undetermined epilepsy. Of the 2,030 cases, 309 (15.2%) were classified into epileptic syndrome categories, and 84.8% of the total were nonspecific types of epilepsy. CONCLUSIONS: The prevalence rate of childhood epilepsy was distributed from 5.3 to 8.8 per 1,000. The appearance rate of various types of epileptic syndromes was low. Most cases could not be classified into the detailed categories of the International Classification (ILAE, 1989).

Age Factors↗

Prevalence of epilepsy in a rural district of central Lao PDR.

PURPOSE: To assess the prevalence of epilepsy in a rural district of Lao PDR and to describe the clinical and epidemiological profile of the disease. METHODS: Door-to-door screening was performed on the entire population of 8 randomly selected villages in the Hinheub district, using an internationally validated and standardized questionnaire. Additional passive case detection was performed through village key informants. Suspected epilepsy patients identified by the questionnaire were revisited and examined by an experienced neurologist on two follow-up visits. The confirmation of epilepsy was based only on an in-depth clinical examination. Electroencephalograms were performed at the district health care center. RESULTS: In the 8 villages, 277 suspected cases of epilepsy were identified among 4,310 interviewed subjects; 194 of whom (70%) underwent a clinical examination by a neurologist during the first visit while 65 of 83 remaining suspected cases were seen on the second confirmation visit. Twenty-seven persons with epilepsy were identified. Six additional patients were diagnosed in 219 self-referred subjects. An overall prevalence of 7.7 cases of epilepsy per thousand inhabitants was calculated (95% CI 5.3-10.7). Generalized epilepsy (21 cases, 63.6%) was commoner than partial epilepsy (9 cases, 27.3%). The remaining 3 cases (9.1%) were not-classifiable as either generalized or partial. EEG abnormal findings were found in 12 of the 24 patients (50.0%) who had an EEG registration. CONCLUSIONS: This is the first study in Lao PDR to estimate the prevalence of epilepsy. Compared to Western countries it shows a pattern towards a higher prevalence.

Adolescent↗

The genetics of human epilepsies.

INTRODUCTION: Genetic causes contribute to many different forms of human epilepsies. Genetic analyses obviously represent a very powerful tool to study this highly heterogeneous pathology. The identification of the genes involved in various epileptic syndromes could prove useful in elucidating the basic mechanisms of epilepsies. This review summarise the recent progress that have been made in the localisation and/or identification of many epilepsy genes. METHODS: The MEDLINE database as well as different other scientific sources were screened. RESULTS: While the genes responsible for several progressive epilepsies have now been identified, it is unlikely that they play a role in the aetiology of true idiopathic epilepsies. Although the majority of idiopathic epilepsies are inherited as complex traits, most studies performed to date have led to the identification of several genetic loci underlying rare epileptic syndromes inherited in a Mendelian pattern. Four genes encoding neurotransmitter- or voltage-gated ion channel subunits have been identified in various idiopathic epilepsies. The epilepsies could thus be considered as one of many paroxysmal disorders that are due to mutations in ion channel genes. CONCLUSION: Effort is still needed to identify the genes responsible for the large variety of other epileptic disorders inherited as Mendelian traits and evaluate the role of these genes in the more common and polygenic forms. Defining the genetic bases of the latter will also require that exhaustive association and sib-pair studies to be performed. These studies may help understand the pathophysiology of human epilepsies, will lead to better genetic counselling in affected families, and represent the first step towards the discovery of new therapeutic targets and methods.

Animals↗

Genetic predisposition to severe myoclonic epilepsy in infancy.

PURPOSE: To address genetic predisposition to febrile convulsions (FCs) and epilepsy as an etiologic background of severe myoclonic epilepsy in infancy (SMEI). METHODS: Familial antecedents of epilepsy and FCs were analyzed in four groups of patients with SMEI (65 cases), FCs (57 cases), childhood absences (67 cases), and a control group of patients with no neurologic problems (64 cases). RESULTS: Patients with SMEI and those with FCs had significantly increased incidence of FCs in their relatives compared with those with absence epilepsy and with the control group. The incidence of epilepsy in relatives of patients with SMEI and absence epilepsy was increased compared with that in the control group and reached statistical significance. Epilepsy in relatives with SMEI had the characteristics of idiopathic generalized epilepsy. CONCLUSIONS: A genetic predisposition could determine three types of epileptic syndromes: FCs, idiopathic generalized epilepsy, and SMEI.

Adolescent↗

Depression and Anxiety in Patients with Epilepsy.

The aim of this investigation was to study the interaction between depression/anxiety and epilepsy. One hundred fifty individuals with partial epilepsy, 70 with idiopathic generalized epilepsy, and 100 controls were administered two self-rating mood questionnaires (Zung and Stai) for the evaluation of depression and anxiety, respectively. The group with epilepsy was much more severely impaired than the controls according to both mood questionnaires; the patients with partial epilepsy, especially those with temporal lobe epilepsy (TLE), were more depressed and anxious than the patients with generalized epilepsy. The group with left TLE appeared to have the highest levels of depression and anxiety. The final results of our study confirmed that some mood disorders are common throughout the epilepsy population, especially in patients with left TLE. No correlation was noted between the frequency of seizures and onset of epilepsy and the results of the mood questionnaires. Moreover, no differences were found in depression and anxiety between males and females among both left focus and right focus epilepsy patients.

Journal Article↗

Idiopathic Generalized Epilepsy.

Successful treatment of idiopathic generalized epilepsy begins with accurate seizure classification. Seizure types, such as absence, myoclonic, and primary generalized tonic-clonic seizures (PGTCS), often can be classified based on a detailed history and inter-ictal electroencephalogram (EEG). Ideally, patients can be classified into specific epilepsy syndromes, such as childhood absence epilepsy, juvenile myoclonic epilepsy (JME), or generalized tonic-clonic seizures on awakening. Idiopathic generalized epilepsy should be distinguished from focal epilepsy with rapid secondary generalization. If this distinction is not clear after history, physical examination, and routine inter-ictal EEG, then ambulatory EEG, video EEG monitoring, or neuroimaging studies may be needed. Ethosuximide, valproate, or lamotrigine are all appropriate first-line choices in the treatment of childhood absence epilepsy. The specific medication should be chosen based on the side effect profiles, dosing formulations, and titration schedules of the medications. The available evidence best supports valproate as the first-line choice in the treatment of JME, although lamotrigine and topiramate may be appropriate choices in this setting. More data specific to JME are needed to clarify the role of medications such as levetiracetam and zonisamide in the treatment of JME. The available evidence to guide the treatment of PGTCS is limited, because most trials did not rigorously exclude patients with focal epilepsy with rapid secondary generalization. Available evidence suggests that valproate is an appropriate first-line choice for PGTCE. Lamotrigine or topiramate also may be appropriate choices. More data are needed to clarify the role of levetiracetam and zonisamide in the treatment of PGCTS. If it is unclear whether a patient has idiopathic generalized epilepsy or focal epilepsy with secondary generalization, then a broad-spectrum anticonvulsant, including valproate, lamotrigine, or topiramate, should be considered. More data are needed to support the broad-spectrum efficacy of levetiracetam and zonisamide.

Journal Article↗

Status epilepticus in childhood: a retrospective study of initial convulsive status and subsequent epilepsies.

A retrospective study was carried out on 261 patients with various epilepsies who had undergone convulsive status epilepticus prior to the subsequent onset of epileptic seizures. 1. Convulsive status epilepticus was found more in partial epilepsy and secondary generalized epilepsy at about the same rate, and evidently less in primary generalized epilepsy. On the average, three-fourths commenced their convulsive disorders with initial status epilepticus. 2. There were free intervals of years following initial status and preceding subsequent epilepsy. The interval was evidently shorter, less than two years, in a majority of patients with secondary generalized epilepsy, whereas the interval was mostly longer, more than six years, in patients with partial epilepsy. 3. The permanent deficient sequelae resulting from initial status were most closely associated with secondary generalized epilepsy. This was also exemplified by the higher rate of atrophic change on CCT. On the contrary, such permanent sequelae were less marked in partial epilepsy especially of complex seizure. 4. It was concluded that secondary generalized epilepsy resulted in cases with more severe brain damage within a relatively shorter interval, whereas complex partial seizure resulted from less severe damage with an obviously longer interval following convulsive status epilepticus.

Adolescent↗

Principles of treatment of epilepsy.

Epilepsy is a common chronic neurological condition with a prevalence of 4-8 per 1000. The present classification of epilepsy is based on: 1) the etiology, which distinguishes symptomatic epilepsies from those that are idiopathic and cryptogenic, and 2) the localization of the disorder in the brain, separating the generalized seizures from epilepsies with partial or focal onset. The majority of patients with epilepsy will go into remission and two-thirds will remain so 2 years after drug withdrawal. The impact of epilepsy on individual patients varies. Employment, driving and learning may constitute major problems. There is a small but definite increase in mortality in patients suffering from epilepsy. Treatment of epilepsy usually involves long-term medical treatment, with the ultimate aim being no seizures and no drugs. Before starting treatment, the diagnosis of epilepsy should be assured. Initiation of antiepileptic drug therapy needs a full and adequate discussion with the patient and the choice of the minimum effective dose of an appropriate monotherapy. Nonpharmacological treatments may be necessary at a relatively early stage if pharmacologic treatment is ineffective. In choosing between different anti-epileptic drugs, consideration should be given to the efficacy of the drug for an individual patient and the tolerability of the drug. There is good evidence from many studies that the chief factor determining relative effectiveness is likely to be the spectrum and incidence of adverse effects of antiepileptic drugs. Some 20% of patients developing epilepsy have a chronic disorder uncontrolled by drugs. In patients receiving and complying with optimal doses of a single antiepileptic drug, the addition of further agents is likely to result in a significant improvement in seizure control in only about 10% of patients, but inevitably it increases the risks of dose-related, idiosyncratic and chronic toxicity due to both pharmacokinetic and pharmacodynamic drug interactions. For this group of patients an appropriate aim may not be complete remission of seizures but a compromise of reduced seizure frequency with less severe seizures, to be achieved with one or, at most, two drugs. The management of these patients with unremitting seizures constitutes a treatment challenge for epileptologists.

Journal Article↗

Molecular genetics of human familial epilepsy syndromes.

Genetic defects have been recently identified in certain inherited epilepsy syndromes in which the phenotypes are similar to those of common idiopathic epilepsies. Mutations in the neuronal nicotinic acetylcholine receptor alpha4 and beta2 subunit genes have been detected in families with autosomal dominant nocturnal frontal lobe epilepsy. Both receptors are components of neuronal acetylcholine receptor, a ligand-gated ion channel in the brain. Furthermore, mutations of two K+ channel genes also were identified as the underlying genetic abnormalities of benign familial neonatal convulsions. Mutations in the voltage-gated Na+-channel alpha1 and beta1 subunit genes were found as the cause of generalized epilepsy with febrile seizures plus, a clinical subset of febrile convulsions. Mutation of a voltage-gated K+-channel gene can cause partial seizures associated with periodic ataxia type 1 and some forms of juvenile myoclonic epilepsy can result from mutations of a Ca2+ channel. This line of evidence suggests the involvement of channels expressed in the brain in the pathogenesis of certain types of epilepsy. Our working hypothesis is to view certain idiopathic epilepsies as disorders of ion channels (i.e., "channelopathies"). Such a hypothesis should provide a new insight into our understanding of the genetic background of epilepsy.

Adult↗