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The National General Practice Study of Epilepsy. The syndromic classification of the International League Against Epilepsy applied to epilepsy in a general population.

In this prospective, population-based study of 594 cases of newly diagnosed epilepsy, proportions in categories as defined by the International League Against Epilepsy (ILAE) were as follows: (1) localization-related epilepsies: 1.1* idiopathic, 1.2%; 1.2* symptomatic, 16.2%; and 1.3 cryptogenic, 24.6%; (2) generalized epilepsies: 2.1* idiopathic (idiopathic generalized epilepsy) with 3-Hz spike and wave: absence epilepsy, 2.2%; juvenile myoclonic epilepsy, 1.5%; and nonspecific idiopathic generalized epilepsy, 5.6%; 2.3.1* symptomatic generalized epilepsies, 1.5%; 2.3.2* specific syndromes with generalized epilepsy, 0.3%; 3.2 seizures without unequivocal focal or generalized features, 32%; 4.1 situation-related syndromes, isolated seizures, 9.9%; seizures due to acute toxic or metabolic cause,* 4.5%. Only 33.6% were in diagnostic ILAE categories (asterisks) and many rare syndromes were not represented. The remainder (66.4%) were in various nonspecific categories. Only 24% of localization-related epilepsies could be clinically localized to a single ILAE-proposed site of origin and of these best localized cases, 14% had strongly discordant imaging or electroencephalograms. These major problems in applying the ILAE classification to epilepsy in the general population and its underemphasis of modern imaging techniques are discussed.

Cerebral Cortex

Prognosis of epilepsy in newly referred patients: a multicenter prospective study of the effects of monotherapy on the long-term course of epilepsy. Collaborative Group for the Study of Epilepsy.

A cohort of 280 previously untreated epilepsy subjects (159 men and 121 women aged 2-81 years) recruited in 14 Italian centers were treated with antiepileptic drug (AED) monotherapy and followed for a median period of 48 months to investigate the rates of seizure remission (i.e., complete control), in general and with reference to various prognostic factors. The cumulative probability of achieving 1-year remission was 62% by 1 year after onset of treatment, 81% by 2 years, 92% by 3 years, and 98% by 5 years. The corresponding figures for 2- and 3-year remission at 5 years were 92 and 78%, respectively. Sixty-two patients (22.1%) had no remission period with monotherapy. Remission rates were significantly lower among patients with two or more seizure types and were inversely correlated to the number of seizures before treatment. The rate of seizure relapses during the first year of follow-up appear to correlate to the risk of developing refractory epilepsy (i.e., with no remission).

Adolescent

Coeliac disease, epilepsy, and cerebral calcifications. The Italian Working Group on Coeliac Disease and Epilepsy.

There have been anecdotal reports of an association between coeliac disease and epilepsy with cerebral calcifications that resemble those of the Sturge-Weber syndrome. A series of patients who had epilepsy with calcifications, in whom coeliac disease (CD) was incidentally observed, prompted us to study this association. 43 patients (15 male, age range 4.6-30.7 years) were selected from two series. 31 patients with cerebral calcifications of unexplained origin and epilepsy (series A) underwent intestinal biopsy. 12 patients with CD and epilepsy (series B) underwent computed tomography. Antibodies to gluten, folic acid serum concentrations, were measured, and HLA typing was done in most patients. 24 of the series A patients were identified as having CD on the basis of a flat intestinal mucosa (15/22 with a high concentration of serum antigluten), and 5 series B patients showed cerebral calcifications, giving a total of 29 cases with the combination of CD, epilepsy, and cerebral calcifications (CEC). In 27 of these CEC patients, calcifications were located in the parieto-occipital regions. Only 2 of the series A patients had gastrointestinal symptoms at the time of intestinal biopsy; most patients had recurrent diarrhoea, anaemia, and other symptoms suggestive of CD in the first 3 years of life. The epilepsy in CEC patients was poorly responsive to antiepileptic drugs. Gluten-free diet beneficially affected the course of epilepsy only when started soon after epilepsy onset. Cases of "atypical Sturge-Weber syndrome" (characterised by serpiginous cerebral calcifications and epilepsy without facial port-wine naevus) should be reviewed, and CD should be ruled out in all cases of epilepsy and cerebral calcifications of unexplained origin.

Adolescent

Medicosocial aspect of people with epilepsy in Japan--a survey from standpoint of epilepsy center.

Medicosocial aspects of 2,000 patients with epilepsy were outlined on the basis of international classification of epilepsies and epileptic seizures. 1. The incidence of secondary generalized epilepsy was higher compared with that reported previously. It was possible that we have been dealing with rather intractable epilepsy. Non-convulsive epileptic seizures should be treated as intensively as convulsive attacks. 2. Contrary to primary generalized epilepsy, secondary generalized epilepsy and partial epilepsy, a part of which was complex partial seizures, were more prone to be associated with psychological difficulties. 3. The rate of normal mentality was apparently proportional to the actual ease of employment among adult patients. Fortunately, the general attitude of education toward younger people with epilepsy was seemingly generous. However, the marital status of both sexes was far from satisfactory. The stigma might have been felt more by adult patients than younger ones. 4. It appeared to be conceivable that two-thirds of the people with epilepsy are treatable to a certain extent by adequate regimens while one-tenth are obliged to stay in residential centers for a lengthy period of time. Thus, various rehabilitation activities are indicated for one-fourth of the patient population. Actual strategy for rehabilitation of epileptics should be individually designed in accordance with the natural history of each epilepsy.

Adolescent

Relative frequency of different types of epilepsy: a study employing the classification of the International League Against Epilepsy.

Approximately three-quarters of epileptic patients seen in consultation by specialists can easily be assigned to one of the diagnostic categories of the International League against Epilepsy. When patient age is taken into account, such a distribution shows that partial epilepsy is more common than generalized epilepsy (62% versus 38%) in patients of all ages, especially in those over 15 years of age (78% versus 22%). In contrast, generalized epilepsy is more frequent in subjects under 15 than partial epilepsy (55% versus 45%). In this younger age group the rarity of partial epilepsy is primarily due to the infrequency of temporal lobe epilepsy (21% of cases, as opposed to 56% in subjects over 15), whereas both primary and secondary generalized epilepsy contribute to the frequency of generalized epilepsy in this group.

Adolescent

Dipole tracing in childhood epilepsy with special reference to rolandic epilepsy.

To assess the clinical applicability of dipole tracing in childhood epilepsy, the location and stability of electric source generator of focal spikes seen in EEG of epileptic children were investigated using this method. The patients were divided into 3 groups; benign childhood epilepsy with centrotemporal spikes (BCECS, Group A, n = 14), other types of epilepsy with centrotemporal spikes (Group B, n = 15) and epilepsy with focal spikes in other areas (Group C, n = 13). The spike dipole in each group was analyzed using dipole tracing method. The following results were obtained. (1) The spikes of BCECS were characterized by constantly stable dipoles, compared to those of the other types of childhood epilepsy. The spikes of epileptic children with mental retardation mostly lacked the stability of dipoles. These seemed to suggest that the stability of dipoles was closely related to the prognosis and pathophysiology of epilepsy. (2) The dipoles of BCECS were localized strictly in the Rolandic area. Dipole tracing from EEG spikes was considered to contribute to the elucidation of the pathophysiology of childhood epilepsy.

Adolescent

Epilepsy and the quality of everyday life. Findings from a study of people with well-controlled epilepsy.

Epilepsy is a stigmatising disorder and available evidence suggests that its diagnosis can have important psychosocial consequences and severely reduce the quality of an individual's everyday life. A number of studies have examined the psychosocial aspects of living with epilepsy, but these have generally involved groups of patients with severe or intractable epilepsy, so that the prevalence of problems may be over-estimated. The present study examined psychosocial functioning in a group of people in whom epilepsy was well-controlled; the majority had been seizure-free for at least two years. In doing so, it drew upon a model of quality of life which incorporated physical, social and psychological domains. Among this group of people, psychosocial functioning and adjustment to epilepsy appeared high, with low reported levels of distress. This is an important finding, not least for people with epilepsy themselves.

Activities of Daily Living

Ontogeny of feline temporal lobe epilepsy, II: Stability of spontaneous sleep epilepsy in amygdala-kindled kittens.

We previously described a model of spontaneous "sleep epilepsy" in kindled kittens with temporal lobe epilepsy (TLE). We now describe the postkindling course of this model from preadolescence to maturity and suggest pathophysiologic mechanisms. Spontaneous epilepsy, particularly generalized tonic-clonic convulsions (GTCs), developed 1h to 4 months after amygdala kindling and persisted to adulthood. At first, GTCs were detected only in sleep; later, convulsions also occurred during wakefulness. Two factors were consistently associated with the sequential onset of sleep and waking GTCs: seizure clusters and anatomic seizure localization. (1) Seizure clusters. Cats with infrequent or unclustered GTCs continued to exhibit "sleep epilepsy," defined by convulsions occurring exclusively during sleep. In contrast, cats with frequent seizure clusters developed recurrent or terminal convulsive status in conjunction with GTCs during waking and sleep. Severe seizure manifestations therefore appeared to contribute to the dissociation of convulsions from the sleep-wake cycle. (2) Anatomical seizure localization. Focal seizure origin appeared to differentiate sleep from waking GTCs. Onset during sleep was first recorded in the kindled amygdala, whereas onset during waking was initially detected outside the temporal lobe. Findings thus suggest secondary "kindling" of multifocal epilepsy. Secondary epileptogenesis is consistent with "transsynaptic" kindling effects. This phenomenon is defined in mature animals by rapid secondary site kindling (transfer) and subtle morphologic changes distal to the stimulating electrode. Transfer may be accentuated by youth, because kittens developed spontaneous seizure foci in previously unstimulated tissue. Moreover, multifocal interactions and diffuse cell loss were implicated as possible mechanisms. Collectively, the findings indicate complications with early onset TLE in kindled cats. Onset during youth can have an unfavorable prognosis, reflected by recurrent status epilepticus and multifocal epilepsy with convulsions distributed throughout the sleep-wake cycle.

Amygdala

National general practice study of epilepsy: the social and psychological effects of a recent diagnosis of epilepsy.

OBJECTIVES: To determine the nature and extent of psychosocial problems in epilepsy and their associations. DESIGN: A postal survey was used drawing data from prospective consecutive cases. Data on demographic, medical, and social backgrounds were collected. A specially designed, validated attitude questionnaire examined 14 areas of psychosocial adjustment to epilepsy. SETTING: 124 primary care general practices. SUBJECTS: Adults (aged over 17) with a recent diagnosis of epilepsy (within previous 36 months). They were registered with the national general practice study of epilepsy and had a confirmed diagnosis according to the usual criteria. 216 subjects were approached for the survey by their general practitioners; 192 returned questionnaires. RESULTS: Problems in at least one area were experienced by 175 (91%) of the 192 subjects. Problems were generally mild, which contrasts strongly with findings in chronic cases. The areas of greatest concern were fear of seizures (80% of cases; 72% moderate or severe) and fear of stigma in employment (69% of cases; 40% moderate or severe). A highly significant relation was found between psychosocial effects and the frequency and recency of seizures. CONCLUSIONS: In the early stages of epilepsy psychosocial effects are closely related to the severity of the medical condition, suggesting that the argument for the stigmatising effect of the diagnosis by itself is less important than previously thought. The findings also suggest that problems may evolve as the condition becomes chronic.

Adaptation, Psychological

Experience with the International League Against Epilepsy classifications of epileptic seizures (1981) and epilepsies and epileptic syndrome (1989) in epileptic children in a developing country.

Four hundred eighty-three epileptic children attending the Pediatric Epilepsy Clinic at Bai Jerbai Wadia Hospital for Children, Bombay, India were classified according to the International League Against Epilepsy (ILAE) classification of epileptic seizures (1981) and epilepsies and epileptic syndromes (1989). The predominant seizures were partial (53.6), generalized (40.3%), and unclassifiable (6%). In epilepsies and epileptic syndromes, 55.3% were partial, 27% were generalized, 13.5% were undetermined, and 4.1% were special syndromes. Although our results were similar in many respects to those of other reported series, some differences were observed in the incidence of partial and generalized seizures, and partial and generalized epileptic syndromes and their subgroups, such as idiopathic, symptomatic, and cryptogenic partial syndromes, idiopathic generalized syndromes, and symptomatic specific syndromes. These differences are probably due to different age limits, methods of case ascertainment and inclusion criteria, different genetic and environmental factors, variable interpretation of clinical and EEG features, and lack of facilities for investigation in developing countries. Despite various limitations, we were able to classify most cases; the ILAE classification can be used in developing countries so that comparison can be made with other studies.

Adolescent

Benign myoclonic epilepsy of infancy: electroclinical symptomatology and differential diagnosis from the other types of generalized epilepsy of infancy.

Benign myoclonic epilepsy in infancy (BME) is characterized by the occurrence of brief myoclonic attacks in normal infants aged 4 months to 3 years. There is no prior personal history, although in some patients 1 or 2 isolated febrile convulsions may occur prior to the onset of myoclonias. A family history of epilepsy or febrile convulsions is present in 30% of cases. Myoclonic attacks are short and mild, they involve mainly the head and upper limbs. The psychomotor development continues normally after the onset of seizures. The EEG shows a normal background activity and generalized spike-wave or polyspike-wave discharges associated with the myoclonias. These abnormalities are activated by drowsiness and during the first stages of sleep. A clinical and EEG photosensitivity is present in one-third of the patients. Myoclonias can be easily controlled by valproate monotherapy. Rare grand mal seizures can occur during adolescence, after withdrawal of drug treatment. The psychomotor evolution is good if treatment is started early. When myoclonias begin during the first year of life, the diagnoses of cryptogenic infantile spasms and of non-epileptic benign infantile myoclonus must be eliminated. In cases with a later onset, the following diagnoses can usually be easily discarded: cryptogenic Lennox-Gastaut syndrome, myoclonic-astatic epilepsy and unclassified epilepsies with the association of myoclonias and other types of seizures.

Cerebral Cortex

Comparison of glutamine synthetases from brains of genetically epilepsy prone and genetically epilepsy resistant rats.

Since glutamine synthetase (GS) has been proposed as the primary enzyme in the regulation of glutamate metabolism in the central nervous system and since inhibition of the activity of this enzyme in vivo leads to seizures, it has been proposed that an abnormality in the structure or function of this enzyme could be responsible for the induction of seizures in epilepsy prone rats. To test this hypothesis the glutamine synthetases were purified from the brains of both genetically epilepsy prone rats (GEPR) and their progenitors, genetically epilepsy resistant rats (GERR). The enzymes were compared using both SDS-PAGE and isoelectric focusing. The immunoreactivities of equal amounts of protein were determined using the ELISA technique, and the regulation of the glutamine synthetase activities by Mn2+/Mg2+ ratios were compared. The only difference found between the glutamine synthetases from the two strains was a slightly lower specific activity of the enzyme from the epilepsy prone animals.

Animals

[Clinico-electroencephalographic analysis of wakeful epilepsy and sleep epilepsy].

Some results of a clinico-physiological analysis of epileptic patients with a different diurnal distribution of generalized convulsive seizures in the sleep-vigilance cycle are reported. It is demonstrated that in vigilant epilepsy there is an intensification of L-rhythms in the EEG recordings in the different functional states of vigilance, combined with a prevalence of delta-sleep in the sleep structure. In sleep epilepsy there is a predominance of the 2nd sleep stage in the sleep structure, besides an EEG desynchronization in the state of vigilance. These results indicate some differences in the activity of nonspecific (synchronizing and desynchronizing) structures in epilepsy with a different diurnal distribution of generilized convulsive seizures.

Adolescent

MRI in the presurgical evaluation of patients with frontal lobe epilepsy and children with temporal lobe epilepsy: pathologic correlation and prognostic importance.

We performed magnetic resonance imaging (MRI) using a high-field strength magnet (1.5 T) in two series of 53 patients with intractable partial epilepsy of frontal lobe or temporal lobe origin who subsequently received ablative surgery for their seizure disorder. In the first series of patients the pathologic correlation and prognostic importance of an MRI-identified lesion in the frontal lobe were assessed. Twenty-five percent of the patients with negative MRI studies and 67% of patients with neuroimaging abnormalities restricted to the frontal lobe, were seizure-free at a minimum duration of follow-up of 1 year. None of the patients with a multilobar MRI-detected abnormality was seizure-free postoperatively. In the second study the sensitivity and specificity of MRI-based hippocampal volumetry was determined in pediatric patients with partial epilepsy of temporal lobe origin unrelated to foreign-tissue pathology. Hippocampal formation atrophy in the epileptic temporal lobe was identified in 63% of patients. The sensitivity and specificity of hippocampal volumetry was 100% in patients with mesial temporal sclerosis. The presence of an MRI-detected epileptogenic lesion in the frontal lobe and hippocampal formation atrophy in the temporal lobe may correlate with the underlying pathology and affect the identification of potential candidates for epilepsy surgery.

Adolescent

Clinico-electroencephalographical study of focal epilepsy with special reference to "benign epilepsy of children with centro-temporal EEG foci" and its age dependency.

Childhood epilepsy with nocturnal seizure and rolandic (centro-temporal) discharge on EEG, had recently been noted by some French and other European authors. These types of epilepsy usually appeared in middle childhood and showed a favorable effect ot anticonvulsant therapy. The present investigation deals with analysis of seizures and of some characteristics of rolandic discharge, particularly with respect to the cerebral maturational process involved in the appearance of seizure discharge. The patients were selected out by the symptoms of epilepsy and features of EEG's, consisting of 20 children in total. Fifteen patients showed somatomotor attacks and five showed somatosensory as well as somatomotor attacks. As for the duration of seizure, those who had seizures before three and 10 years of age, had attacks for relatively short periods, whereas those who had attacks from four to nine years of age, usually had longer duration. The amplitude of "RD" remained lower than 50 muv up to three years of age, whereas it reached as high as 200 muv up to nine years, particularly during sleep, which became lower after the age of 10. It was concluded that there is an age dependency between the ages of four and nine.

Adolescent

Knowledge of epilepsy among relatives of the epilepsy sufferer.

A 23-item questionnaire was constructed to assess awareness of basic facts about epilepsy. Close family members of the epilepsy sufferer achieved significantly higher scores on the questionnaire than individuals from families without experience of the condition. The questionnaire may be useful in the identification of family members of the epilepsy sufferer whose knowledge of the condition is low and who may require appropriate counselling.

Adult

Status of follow-up among patients with epilepsy in epilepsy clinic.

Out of 238 consecutive patients entering our epilepsy clinic from October 1988 till March 1990, the status of follow-up was evaluated in October 1990. One hundred patients (42%) could adhere to the follow-up appointment and 33 patients (14%) visited the clinic irregularly. A total of 105 patients (44%) failed to return for the follow-up at least for 2 months dated backward from October 1990. Seventy-five percent of the patients lost the follow-up within 6 months. The main reasons of the dropout were poor seizure control, seizure free, denial of epilepsy, receiving operation in other hospitals. The appropriate strategies to aid in adherence to medical care among patients with epilepsy should be formulated through the analysis of factors contributing to the dropout, which might be strongly influenced by different medical and sociocultural backgrounds all over the world.

Adolescent

Epilepsy, antiepileptic drugs, and malformations in children of women with epilepsy: a French prospective cohort study.

We conducted a prospective study of teratogenic effects of antiepileptic drugs (AEDs) in pregnant women with epilepsy in southeast France, comparing malformation rates with those collected by a birth defects registry. We evaluated isolated microcephalies separately. Malformations were seen in 7% of infants of mothers with epilepsy (IME) and in 1.36% of the general population. No significant relationship was found between type and severity of epilepsy and occurrence of malformations or isolated microcephaly. Valproate and phenytoin were the most teratogenic (all malformations). None of the malformations observed in IME whose mothers received valproate, phenytoin, or phenobarbital was seen in IME not exposed to the respective AEDs. Phenytoin plus phenobarbital was more teratogenic than phenobarbital alone. Benzodiazepines, prescribed only in combinations, had a borderline, nonspecific effect on microcephaly.

Abnormalities, Drug-Induced