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Bromides were effective in intractable epilepsy with generalized tonic-clonic seizures and onset in early childhood.

Thirty-six children with epilepsy resistant to conventional treatment were treated with bromides in addition to the current therapy. Six out of 19 cases with prevailingly or exclusively generalized tonic-clonic seizures became seizure-free and in 9 cases a reduction in seizure frequency of more than 50% was achieved. Freedom from seizures could not be obtained in 13 cases, who had frequent minor seizures in addition to generalized tonic-clonic seizures. In some, minor seizures were even activated. Tonic and focal seizures showed no response. Side effects were observed in one-third of the cases (acne, loss of appetite, loss of weight, fatigue) but in no case they did become intolerable. Fifty to 80 mg potassium bromide per kg body weight seems to be an effective daily dose range. There is a preferential indication of bromides for patients suffering from early onset epilepsy with generalized tonic-clonic seizures and/or alternating hemi-grand mal, for whom other treatment is ineffective. This disorder is characterized by a high familial incidence of epileptic seizures, onset between 6 months and 3 years of age, normal development until the onset of seizures, generalized tonic-clonic seizures and often alternating hemi-grand mal, seizure precipitation by fever, and occasional combination with or transition to myoclonic-astatic and/or myoclonic seizures. EEG is often normal or shows slight slowing in the initial phase; later it shows theta rhythms and generalized spikes and waves. Especially, if the onset is during the first year of life, the course of the epilepsy is often unfavourable.

Adolescent↗

Ego functions in epilepsy.

Two groups of epilepsy patients (28 patients with temporal lobe epilepsy and 15 patients with primary generalized epilepsy) entered a study of personality traits related to epilepsy, based on a modification of Bellak's semistructured interview for assessment of ego strength. Two groups of subjects served as controls: 15 patients with a non-neurological but relapsing disorder, psoriasis, and 15 healthy volunteers. Compared with the group of healthy volunteers, a decreased adaptive level of ego functioning was found in the epilepsy groups, regardless of seizure types and EEG findings, and, to a lesser extent, compared with the psoriasis group. Areas of ego functioning most affected were "reality testing", "cognitive functioning", "integrative functioning" and "regulation and control of drives". Patients with more than one type of seizure were the most affected, as were patients who were younger than 15 years when the disease began. The number of anticonvulsants administered did not influence the results. No difference on adaptive level of ego functioning was found between the group with primary generalized epilepsy and the group with temporal lobe epilepsy. Similarly, the temporal lobe epilepsy group with predominantly right-sided and left-sided EEG changes, respectively, showed similar adaptive levels of ego functioning.

Adult↗

Regional reductions in serotonin 1A receptor binding in juvenile myoclonic epilepsy.

BACKGROUND: Juvenile myoclonic epilepsy (JME) is classified as primarily generalized epilepsy and as such is assumed to lack an anatomic substrate. Although neurochemical abnormalities are probable, few studies have investigated whether they exist in JME. Animal data and the high incidence of myoclonic seizures in serotonin-intoxicated patients suggest that the serotonin system may be disturbed in JME. OBJECTIVE: To test the hypothesis that JME is associated with a disturbed serotonin system and that this disturbance could be reflected in altered serotonin 1A receptor binding. DESIGN: The serotonin 1A receptor binding potential (BP) was measured with positron emission tomography and serotonin 1A receptor antagonist carbonyl-carbon 11-WAY-100635. The BP was calculated using a reference tissue model in several limbic and neocortical regions and the raphe nuclei. SETTING: Epilepsy clinics of the Karolinska University Hospital, Stockholm, Sweden. PATIENTS: Eleven patients with JME and 11 controls were studied. MAIN OUTCOME MEASURE: Serotonin 1A receptor BP calculated in a set of volumes of interest. RESULTS: The patients with JME showed a reduced BP in the dorsolateral prefrontal cortex, raphe nuclei, and hippocampus. CONCLUSIONS: The observed reductions in serotonin 1A receptor BP suggest that the serotonin system is affected in JME. Although the data give no definitive information about underlying mechanisms, they provide a strong argument for the view that not all brain regions are homogeneously involved in this condition, further questioning the current classification of primarily generalized epilepsy.

Adult↗

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↗

[Antiepileptic drug therapy for adult patients with intractable seizures].

Epileptic syndrome, antiepileptic drug (AED) therapy and mental disorder were studied in 223 patients with intractable epilepsy, who were admitted to our epilepsy center between 1992 and 2000. Symptomatic localization-related epilepsy was diagnosed in 86.1% of patients, symptomatic or cryptogenic generalized epilepsy in 7.6%, idiopathic generalized epilepsy in 1.8%, unclassifiable epilepsy in 3.1% and non-epilepsy in 1.3% on discharge. Only 6.3% had diagnoses on discharge that were incongruent with their diagnoses on admission. AED therapy during admission improved markedly in 50% of patients and moderately in 20%, however, 60% had seizures more frequently than 4 a month on discharge. Generalized tonic and clonic seizures were suppressed completely in 82.5% of patients. The number of AEDs used were 2 AEDs in 28.6%, 3 in 39.1% and 4 in 22.3%. Only 6.4% of patients were on monotherapy on discharge. Mental retardation was in 58.7%, schizophrenia-like psychosis in 8.5%, delusional disorder in 1.8%, mood disorder in 3.6%, AED-related disorder in 14.3% and psychogenic disorders in 21.5%. AED therapy is effective for intractable seizures, but it is limited in its effect. Mental disorders also coexisted in most of patients. Therefore comprehensive therapy of epilepsy is necessary for patients with intractable seizures.

Adult↗

Study on FIRDA and 3 Hz rhythmic slow wave bursts occurring in the frontal area of epileptic patients.

We classified epileptic patients with frontal-dominant rhythmic slow-wave bursts into a FIRDA group with frequencies of 1.5 to 2.5 Hz and a 3 Hz group with a frequency of 3 Hz. We compared the two groups in clinical background and EEG findings. The patients in the FIRDA group were older, and partial epilepsy was more common. In the 3 Hz group, idiopathic generalized epilepsy was more common. All of the partial epilepsy in the patients in the FIRDA group was symptomatic, and organic brain damage such as sequelae of brain tumor surgery, or cerebrovascular disorders, etc., were the most common primary diseases. There was no difference between the two groups in frequency of seizures, and VPA was the drug most often used to treat the patients in the 3 Hz group. No morphological differences between the two group, except the difference in frequency, were observed among the EEG findings. Accompaniment by localized spike waves or sharp waves was more common in the FIRDA group. When epilepsy was accompanied by rhythmic slow-wave bursts in the frontal area, its clinical significance depended on whether its frequency was 2.5 Hz or less or 3 Hz. When it was 2.5 Hz or less, symptomatic partial epilepsy was more common, suggesting the presence of large, prominent organic brain damage, such as sequelae of brain tumor surgery or cerebrovascular disorders. Idiopathic generalized epilepsy, on the other hand, was shown to be more common when the frequency was 3 Hz.

Adolescent↗

Evolution of childhood absence epilepsy, juvenile myoclonic epilepsy and epilepsy with grand mal on awakening.

Seventy-one Chinese children with idiopathic generalized epilepsy, from among 1931 pediatric epileptics, were reviewed clinically and electroencephalographically. They were classified under childhood absence epilepsy (CAE, 50/71), juvenile myoclonic epilepsy (JME, 7/71) and epilepsy with grand mal on awakening (GMA, 14/71). All the patients' family members with epilepsy were classified under idiopathic generalized epilepsy. Hyperventilation was the most common precipitating factor for CAE, and sleep deprivation was the most common one for JME and GMA. Electroencephalographically, fast generalized spike or polyspike-wave complex (> or = 3Hz) was the most common feature. Three JME (42%) and 4 GMA patients (28%) had been diagnosed as CAE about 5 to 7 years preceding JME and GMA, respectively. Sixteen CAE cases (32%) later evolved to be GMA. All JME patients had concomitant GMA and three GMA cases (21%) had concomitant JME at diagnosis. There is strong evolutional correlation among CAE, JME and GMA in this series.

Adolescent↗

[Epilepsy with myoclonic absences].

Within generalized epilepsy, the syndrome of epilepsy with myoclonic absences is considered as intermediate between idiopathic and symptomatic forms. This syndrome is characterized by developing in childhood with a male predominance. Critical EEG shows paroxysms of PO at 3 Hz, and in the polygraphic recording rhythmic 3 Hz myoclonus is observed with a strict correspondence between EEG spike and myoclonus. The response to therapy is generally poor, and 18% develop generalized symptomatic epilepsy. We report 3 patients with epilepsy with myoclonic absences and good outcome, to emphasize the importance of a precise diagnosis by means of the polygraphic recording of the attack, the fact the association of sodium valproate and ethosuximide is the most useful therapy and, finally, the possibility that some patients with epilepsy with myoclonic absence may develop Janz's juvenile myoclonic epilepsy.

Adult↗

Epilepsy: a general practice study of knowledge and attitudes among sufferers and non-sufferers.

The aim of this study was to examine epilepsy sufferers' attitudes to and knowledge of their condition, the effect of epilepsy on their lives and their views on the management they had received, and to compare knowledge and attitudes with those of a control group of non-sufferers. A questionnaire was completed by 29 patients with epilepsy and 32 control group subjects from two general practices. It was found that people with epilepsy knew little more than those without epilepsy regarding the nature of the condition, its aetiology and seizure precipitants. Those with epilepsy were concerned about the seizures and the effect these had on various aspects of their lives, and were concerned about long-term side effects of anti-epileptic medication. There were no significant differences between the two groups with respect to educational achievement, employment record and social activities. The findings are discussed and suggestions put forward for improving the care offered to epilepsy sufferers by both general practitioners and hospital clinics.

Adult↗

Idiopathic epilepsy with generalized tonic clonic seizures in Antioquia, Colombia: is the joint Amerindian and Negroid racial admixture the cause of its high prevalence?

Most Colombian populations stem from the admixture of Caucasians, Amerindians and Negroids. In the world, these two latter ethnical groups show a significantly higher prevalence of epilepsy than the former one. We tested the hypothesis that the high prevalence of idiopathic epilepsy with generalized tonic clonic seizures found in the Antioquian population (Paisas), from Colombia, is due to their possible joint Negroid and Amerindian ethnic components. We have previously demonstrated that inheritance is the principal factor for developing epilepsy in this community. Analyses of racial admixture, heterogeneity between populations, genetic distance, and phyletic relationships were performed among epileptic and non epileptic samples from the Antioquian community. Also Caucasians, Spaniards, Basques, Jews, Chileans, Negroids, Amerindians and Mongoloids were included in the analysis. Four highly polymorphic blood systems were used as genetic markers: RH, MNS, ABO and FY. They were chosen because of their high discriminant power in these ethnic groups. In the population affected with idiopathic epilepsy, the estimated Negroid and Amerindian rates of admixture were low (3% and 14%, respectively). Although, these degrees of admixture can be explained due to common ancestral origins, the estimated proportion of Amerindian admixture in the epileptic affected population, was significantly higher than the estimated for the Non affected Antioquian population. The latter finding is consistent with the analysis of heterogeneity between populations that discriminated epileptic population from non epileptic Antioquian population (p < 0.05). Epileptic and non epileptic Paisas clustered in topology with Caucasians, very close to Spaniards and Basques and highly distant from Negroids and Amerindians. Thus, far, the origin of the high prevalence of idiopathic epilepsy in the Antioquian (Paisa) population cannot be explained by the hypothetical joint Negroid and Amerindian ethnical admixture, but using additional genetic markers and other methods of racial estimation of admixture it is necessary to corroborate if the Amerindian admixture component is significantly higher in the epileptic population than in the non epileptic Paisa population.

Black People↗

Longitudinal EEG studies in a kindred with Lafora disease.

We reviewed 18 EEG studies in four members of a family with the Lafora form of progressive myoclonic epilepsy. Each patient was the product of a consanguinous marriage and presented as a teenager with progressive seizures, myoclonus, dementia, and ataxia, and had biopsy proven disease. The EEG early in Lafora disease has spike-wave activity resembling that seen in a primary generalized epilepsy; the background slowing is more typical of a secondary generalized epilepsy. With disease progression, there is increased epileptiform activity, and a striking change in the spike-wave complexes, with a marked increase in frequency up to 6-12 Hz, and many more short duration polyspike components. Unlike some other forms of secondarily generalized epilepsy, the EEG in Lafora disease is distinguished by an increased frequency of the spike-wave complexes with disease progression.

Adolescent↗

A survey of lamotrigine and vigabatrin treatment in children with severe epilepsy.

A retrospective survey was carried out of add-on treatment with lamotrigine (LTG) and vigabatrin (GVG) in 109 children with severe epilepsy, treated between 1987 and 1994, identified from a total population of 300 patients seen annually, in a tertiary referral outpatient clinic in Cardiff, Wales. Of 79 patient treatments with LTG and 86 with GVG, 42 patients were treated with add-on LTG, 52 with add-on GVG and 20 with both drugs simultaneously. A Kaplan-Meier curve, applied to each of the two index drugs, indicated that 71 and 62% of patients would be expected to continue taking LTG or GVG, respectively after 40 months. Improved seizure control (> or = 50%) at the time of audit was seen in 65% of LTG and 58% of GVG patient treatments for all epilepsy syndromes, but there was a higher proportion of patients with generalized epilepsy improved by LTG (28/41, 68%) than that improved by GVG (8/20, 40%), and only those with generalized epilepsy treated with LTG became seizure free (8/38, 21%). Similar proportions of patients discontinued LTG (16%) and GVG (15%) due to an adverse experience, but a higher proportion discontinued GVG (18%) compared with LTG (6%) because of lack of efficacy. This study supports the relative clinical effectiveness of LTG and GVG in the real world, where children with severe epilepsy are treated in clinical practice and serves to generate hypotheses to enable design of prospectively controlled trials, which should enable more rational use of these two drugs in the paediatric population with epilepsy.

Adolescent↗

Differential participation of some 'specific' and 'non-specific' thalamic nuclei in generalized spike and wave discharges of feline generalized penicillin epilepsy.

Extracellular single unit and electroencephalographic (EEG) activity during generalized spike and wave discharges (SW) induced by i.m. penicillin was recorded simultaneously in the cortex, in a 'specific' thalamic nucleus (n. lateralis posterior, LP) and in some 'non-specific' thalamic nuclei (n. centralis medialis, NCM; n. centrum medianum, CM; n. centralis lateralis, CL) Computer-generated EEG averages and histograms of single unit activity were triggered by either peaks of EEG transients or action potentials. The time at which cortical neurons (66/66) were most likely to fire was during the 'spike' of the SW complex while absence of firing was the rule during the 'wave'. Most LP neurons (23/26) showed a similar pattern, 3 cells firing preferentially during the 'wave'. In NCM only 17 of 39 neurons fired during the 'spike', 8 of 39 neurons during the 'wave' while the others showed no change in their firing pattern during SWs. Twenty-six of 30 CM and 20 of 24 CL neurons fired during the 'spike' of SW; the other cells in these nuclei did not change their firing pattern during SWs. When present, rhythmic fluctuations in firing linked to SW discharge were less prominent in these 'non-specific' thalamic nuclei than in cortex and LP. Furthermore, participation of NCM, CM and CL neurons in the SW rhythm occurred only after neurons in cortex and LP had become involved in it. Thus, as is the case for cortical neurons, the main firing pattern of thalamic cells during SWs consists of an oscillation between 'excitation' during the 'spike' and 'inhibition' during the 'wave' of the SW complex. However, the coupling between cortical and thalamic neuronal firing is less intimate for cells of the 'non-specific' thalamic nuclei than for a 'specific' nucleus such as LP. Thus, at least some 'specific' thalamic nuclei are more intimately involved in the mechanism of SW discharge than the midline intralaminar nuclei.

Animals↗

An audit of epilepsy in general practice.

A simple records audit of patients with epilepsy revealed some deficiencies in care and that regular follow-up was inadequate. Further information was obtained by a questionnaire. A new protocol of care was introduced and further audit is being undertaken.

Adolescent↗

[Level of myoglobin in serum of patients with epilepsy after generalized tonic-clonic seizure].

In 11 patients with epilepsy serum myoglobin level determinations were performed by the RIA method 10 minutes, 1 hour, 4, 12, and 24 hours after tonic-clonic seizure and were compared with myoglobin concentrations in a group of 30 healthy persons. A statistically significant increase of myoglobin concentration was shown already in the first hour after the seizure with maximal level, almost five times exceeding the baseline level in the fourth hour, and slightly lower--four times exceeding the baseline--12 hours after the seizure. The correlative studies, comparing the profile of myoglobinaemia with the clinical picture (duration of the disease, age and sex, presence or lack of EEG changes, type of therapy used) showed no statistical significance.

Adult↗

[The immunopathology and immunogenetics of some forms of pediatric epilepsy].

52 patients with primary and 42 with secondary generalized epilepsy at the age of 2-16 years were examined. In all the patients blood content of T- and B-lymphocytes as well as of their subpopulations (T-helpers, T-suppressors) was estimated with the use of monoclonal antibodies. Immunogenetic studies of allogenic lymphocytes were also performed in mixed lymphocyte cultures for proband and mother. There was an increase of CD4/CD8 ratio (T-helper/T-suppressor) as well as elevation of B-lymphocytes level in patients with secondary generalized form of epilepsy. A positive reaction of allogenic lymphocytes from mother and child in mixed cultures was observed in 46 patients with primary generalized epilepsy (88.5%), in 16 cases with progredient disease. In secondary generalized form of epilepsy it occurred in 7 cases only (16.7%). In 28 cases of epilepsy with progredient course not only antiepileptic drugs but also immunomodulators were used with positive effect in 14 patients.

Adolescent↗

Is the underlying cause of epilepsy a major prognostic factor for recurrence?

BACKGROUND: We investigated the prognostic value of the type of epilepsies and epileptic syndromes for seizure recurrence. In patients with partial epilepsy, we focused on the prognostic value of any structural brain abnormality and of the location of the epileptogenic region. METHODS: A total of 2,200 adult outpatients were included in a hospital-based observational survey, with a follow-up of 1 to 7 years. Twenty-two percent of the patients exhibited generalized epilepsy, 62% partial epilepsy, and 16% undetermined epilepsy. RESULTS: Seizure control (>1 year without seizure) was achieved in 82% of patients who had idiopathic generalized epilepsy, 35% of those with symptomatic partial epilepsy, 45% of those with cryptogenic partial epilepsy, and 11% of those with partial epilepsy associated with hippocampal sclerosis (HS). Temporal lobe epilepsy (TLE) was the most refractory partial epilepsy, with only 20% of such patients remaining seizure free, compared with 36% of extra-TLE patients. In partial epilepsy, HS, cerebral dysgenesis, and dual pathology (HS and another lesion) were associated with a low rate of seizure-free patients (11%, 24%, and 3%, respectively). No significant difference in seizure control was found between patients with extra-TLE and those with TLE and no HS. CONCLUSIONS: In adults, partial epilepsy is more difficult to treat than idiopathic generalized epilepsy. In patients who have partial epilepsy, the location of the epileptogenic zone does not seem to be a determining factor. Brain abnormalities--especially HS, either alone or associated with another lesion--are a major prognostic factor.

Adolescent↗

Classification of epilepsy syndromes and role of genetic factors.

In this report the types of epilepsy syndromes seen in children in a tertiary referral center in Beirut, Lebanon were studied and the importance of consanguinity and family history in the occurrence of these syndromes was investigated. Records of 230 pediatric patients evaluated during a 1-year period with the diagnosis of single seizure, febrile seizure, or epilepsy were reviewed. Each patient was classified according to the International League Against Epilepsy classification. The occurrence of consanguinity, of family history of febrile seizures or epilepsy, and of other variables was noted. Thirty-six percent of patients were diagnosed with localization-related epilepsy, 21.7% with generalized epilepsy, 11.7% with undetermined generalized or focal, and 24.3% with special syndromes. Twelve percent of patients were diagnosed with idiopathic, 15.1% with symptomatic, and 30.3% with cryptogenic epilepsies. Consanguinity was more common in patients with symptomatic and cryptogenic epilepsies than in patients with idiopathic epilepsies or with incidental seizures (P < 0.05). Family history of epilepsy was more common in patients with symptomatic, cryptogenic, and idiopathic epilepsies than in patients with incidental seizures (P < 0.05). Family history of febrile seizures but not consanguinity was more common in patients with febrile seizures (P < 0.05). We conclude that genetic factors are important not only in idiopathic epilepsies and febrile seizures but also in cryptogenic and symptomatic epilepsies.

Adolescent↗