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Course and prognosis of childhood epilepsy: 5-year follow-up of the Dutch study of epilepsy in childhood.

Knowing the prognosis of epilepsy will undoubtedly influence the treatment strategy. This study aimed to define the prospects of newly diagnosed childhood epilepsy, assess the dynamics of its course, identify relevant variables and develop models to assess the individual prognosis. Four hundred and fifty-three children with newly diagnosed epilepsy were followed for 5 years. Terminal remission at 5 years (TR5) was compared with terminal remission at 2 years (TR2) and with the longest remission during follow-up. Variables defined at intake and at 6 months of follow-up were analysed for their prognostic relevance. In multivariate analyses, combinations of variables were tested to develop reliable models for the calculation of the individual prognosis. Data on treatment, course during follow-up and epilepsy syndromes were also studied. Three hundred and forty-five children (76%) had a TR5 >1 year, 290 (64%) >2 years and 65 (14%) had not had any seizure during the entire follow-up. Out of 108 children (24%) with TR5 <1 year, 27 were actually intractable at 5 years. Medication was started in 388 children (86%). In 227 of these (59%), anti-epileptic drugs (AEDs) could be withdrawn. A TR5 >1 year was attained by 46% on one AED, on the second AED by 19%, and by 9% on all additional AED regimes. Almost 60% of the children treated with a second or additional AED regime had a TR5 >1 year. Variables predicting the outcome at intake were aetiology, history of febrile seizures and age. For intake and 6-month variables combined, sex, aetiology, postictal signs, history of febrile seizures and TR at 6 months were significant. The model derived from intake variables only predicted TR5 <1 year correctly in 36% and TR5 >1 year in 85% (sensitivity 0.65, specificity 0.64). The corresponding values for the model derived from intake and 6-month variables were 43 and 88% (sensitivity 0.69, specificity 0.71). The course of the epilepsy was constantly favourable in 51%, steadily poor in 17%, improving in 25% and deteriorating in 6%. Intractability was in part only a temporary phenomenon. The outcome at 5 years in this cohort of children with newly diagnosed epilepsy was favourable in 76%; 64% were off medication at that time. Almost a third of the children had a fluctuating course; improvement was clearly more common than deterioration. After failure of the first AED, treatment can still be successful. Models predicting the outcome have fewer misclassifications when predicting a long terminal remission than when predicting continuing seizures.

Adolescent↗

Complications of epilepsy surgery after 654 procedures in Sweden, September 1990-1995: a multicenter study based on the Swedish National Epilepsy Surgery Register.

OBJECTIVE: To present the first national multicenter study on complications after epilepsy surgery procedures to include all epilepsy surgery centers in Sweden: Göteborg, Linköping, Lund, Stockholm, Umeå, and Uppsala. METHODS: Every epilepsy surgery procedure in Sweden is reported to the Swedish National Epilepsy Surgery Register. The report includes relevant social and medical items and complications. A complication is defined as minor if it resolves within 3 months and major if it affects activities of daily living and lasts longer than 3 months. Follow-up data are recorded for 2 years after the operation. Intrinsic checkpoints and external revision validate the register data. RESULTS: During the inclusion period (September 1990-December 1995), 654 surgical procedures were performed (age range, 6 mo-67 yr). Of these, 205 were invasive electrode procedures (182 first investigations and 23 reinvestigations), and 449 were therapeutic procedures (375 first operations and 74 reoperations). After invasive electrode procedures, only minor complications were reported (6.3%). For all 449 therapeutic procedures (including reoperations), minor complications were reported in 8.9% and major complications in 3.1 %. Only one major complication was reported in a patient under the age of 35 years. CONCLUSION: This is the first national multicenter study on complications after epilepsy surgery. It demonstrates that these procedures as performed at six different centers in Sweden are safe, but also that risk is related to age. In patients younger than age 35 years, the risk for a major complication after invasive subdural strip electrode investigation and epilepsy surgery is low.

Adolescent↗

Anesthetic implications of epilepsy, status epilepticus, and epilepsy surgery.

Epilepsy is a clinical paroxysmal disorder of recurring seizures, excluding alcohol or drug withdrawal seizures or such recurring exogenous events as repeated insulin-induced hypoglycemia. Epilepsy has a profound impact on each individual diagnosed with this disease. Seizures have been and are thought to arise as a result of abnormalities in (a) neural circuits, (b) excitation/inhibition balance, (c) potassium, and (d) genetic abnormalities. Therapy for epilepsy is either medical, entailing the use of a variety of antiepileptic drugs, or surgical. An urgent approach to seizure control is indicated when status epilepticus occurs. When all standard therapy fails, general anesthesia can be used to control status epilepticus. Surgery is an option in the treatment of epilepsy and requires extensive preoperative evaluation. The primary concerns for the neuroanesthesiologist anesthetizing the patient with epilepsy are the capacity of anesthetics to modulate or potentiate seizure activity and the interaction of anesthetic drugs with antiepileptic drugs. Proconvulsant and anticonvulsant properties have been reported for nearly every anesthetic. If seizure spikes are to be evoked during seizure surgery, then light anesthesia with a proconvulsant anesthetic is used. Conscious analgesia can be used for awake seizure surgery. However, if electrocorticography is not planned, then a general anticonvulsant anesthetic maintenance regimen is used. The latter technique also may be useful in patients whose anesthetic management is complicated by an incidental history of epilepsy.

Anesthesia↗

Implementation strategies for a Scottish national epilepsy guideline in primary care: results of the Tayside Implementation of Guidelines in Epilepsy Randomized (TIGER) trial.

PURPOSE: To determine the effectiveness of two dissemination and implementation strategies to implement a national guideline for epilepsy management in primary care settings. METHODS: Three-arm cluster-randomized controlled trial. The participants were general practitioners from 68 practices in Tayside, Scotland, and 1,133 of their patients with self-reported epilepsy treated with antiepileptic medications (AEDs). Practices were randomized blind to a control, intermediate, or intensive intervention. CONTROL: Postal dissemination of a nationally developed clinical guideline. Intermediate intervention: Postal dissemination of the guideline supported by interactive, accredited workshops, and dedicated, structured protocol documents. Intensive intervention: Intermediate intervention plus a nurse specialist who supported and educated practices in the establishment of epilepsy review clinics. The primary outcome was the SF-36 health-related quality-of-life instrument. Secondary measures were a battery of prevalidated epilepsy-specific quality-of-life instruments. These were administered at baseline and after the intervention phase. Process of care was assessed by case-note review on number of review meetings and counseling sessions for epilepsy before and after the interventions. RESULTS: None of the intervention groups showed any change in the primary or secondary outcome measures or process-of-care measures. CONCLUSIONS: None of the intervention strategies led to improvements in patient quality of life or quality of epilepsy care. Further research is needed to discover why the interventions failed, to identify barriers to adoption of guidelines, and to develop strategies that might improve implementation and uptake in the future.

Adult↗

Is cognitive reserve applicable to epilepsy? The effect of educational level on the cognitive decline after onset of epilepsy.

PURPOSE: The aim of this study was to know the effect of education level (EL) on the cognitive change after onset of epilepsy. The rationale is that people with high EL may have more cognitive reserve and show resistance to damage brought about by epilepsy. METHODS: Patients were from an outpatient epilepsy clinic of a national university hospital. All received initial and repeated neuropsychological tests and the Cognitive Ability Screening Instrument (CASI), at an interval of 12 months. CASI consists of nine items, with a total score of 100. We compared the differences between the two tests, in terms of z-scores. The EL was divided into high and low with a cut-off at 12 years, and age at entry was divided with a cut-off at 38 years. Epilepsy severity was divided into intractable and nonintractable. RESULTS: A total of 64 patients completed the study, including 31 with high EL and 33 with low EL, with no differences in gender and epilepsy severity between groups. In general, patients had cognitive impairment in multiple domains. At the baseline, the effect of EL was found for all items except remote memory and orientation, whereas the effect of age was observed for remote and recent memory. At the 12-month follow-up, subjects with high EL showed deterioration in mental manipulation, whereas subjects with low EL showed improvement in verbal fluency but deterioration in attention. CONCLUSIONS: Patients with high EL have better cognitive functioning than those with low EL after epilepsy has developed, which supports the cognitive reserve theory. However, 1-year follow-up might be too short to see significant changes between groups.

Adult↗

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↗

Is epilepsy a progressive disease? The neurobiological consequences of epilepsy.

While primary, or idiopathic, epilepsies may exist, in the vast majority of cases epilepsy is a symptom of an underlying brain disease or injury. In these cases, it is difficult if not impossible to dissociate the consequences of epilepsy from the consequences of the underlying disease, the treatment of either the disease or the epilepsy, or the actual seizures themselves. Several cases of apparent complications of epilepsy are presented to illustrate the range of consequences encountered in clinical practice and the difficulty in assigning blame for progressive symptomatology in individual cases. Because of the difficulty in interpreting clinical material, many investigators have turned to epilepsy models in order to address the potential progressive consequences of recurrent seizures. The authors review experimental data, mainly from animal models, that illustrate short-, medium-, and long-term morphological and biochemical changes in the brain occurring after seizures, and attempt to relate these observations to the human condition.

Adult↗

Psychiatric profiles and patterns of cerebral blood flow in focal epilepsy: interactions between depression, obsessionality, and perfusion related to the laterality of the epilepsy.

OBJECTIVES: In a study of patients with focal epilepsy the hypothesis was explored that different measurements of psychopathology are related to specific distributions of cerebral perfusion. METHODS: Forty patients had SPECT performed with (99m)Tc-HMPAO. In addition, patients received a psychiatric evaluation with the following psychiatric questionnaires: the Beck depression inventory, the Leyton obsessionality inventory, the Bear-Fedio questionnaire, and the social stress and support interview. Patients were analysed in two groups according to the laterality of the epilepsy. Nine patients were excluded based on poor quality scans (n = 1), unlateralised epilepsy (n = 4), and left or ambidextrous handedness (n = 4). RESULTS: There were no overall differences between the left and right epilepsy groups on measures of psychopathology. Associations were found between scores on some of the rating scales and regional cerebral blood flow. Specifically, for patients with left sided epilepsy, higher scores on the Beck depression inventory were associated with lower contralateral temporal and bilateral frontal perfusion, and higher occipital perfusion. For patients with right sided epilepsy higher scores on the Leyton obsessionality inventory were associated with increased perfusion in ipsilateral temporal, thalamic, and basal ganglia regions and bilateral frontal regions. CONCLUSION: The results do not support the notion that lateralised epileptogenic lesions are associated with different levels of depression, obsessionality, or personality traits. They support the view that certain psychopathological symptom patterns are related to specific regional dysfunctions depending on the laterality of a hemispheric lesion.

Adolescent↗

Self-esteem, behavior, and concerns surrounding epilepsy in siblings of children with epilepsy.

Researchers document the emotional impact of epilepsy on the child with seizures. Minimal data are available examining the effects of epilepsy on the siblings of children with seizures. Twenty children whose siblings had either frequent seizures or infrequent seizures were matched by age, gender, and birth order to control subjects with no chronic illness. These three groups were compared. Self-esteem, behavioral and social functioning, and family stress were measured by the Piers-Harris Self-Concept Scale, Child Behavior Checklist, and Family Inventory of Life Events. The siblings of children with epilepsy completed the Sibling Concern About Seizure Scale to define and measure their concerns surrounding epilepsy. There is no statistical difference in self-esteem or social functioning among the three groups. There is a trend toward increased incidence of externalizing behavior in siblings of children with frequent seizures. Data indicate a trend toward siblings of children with frequent seizures having more concerns about epilepsy than siblings of children with infrequent seizures. There is significantly more stress in families of children with frequent seizures compared to families of children with infrequent seizures and families of children with no chronic illness. Although there were no significant differences in the self-esteem, behavior, socialization, and concerns between the siblings in the family when compared to the control group or to each other, there were trends in the results that may be of clinical significance. These issues, along with the level of family stress, should be considered when coordinating and providing care to families of children with intractable epilepsy.

Child↗

Driving in adults with refractory localization-related epilepsy. Multi-Center Study of Epilepsy Surgery.

OBJECTIVE: To examine the frequency of driving an automobile and characteristics associated with driving in individuals with refractory localization-related epilepsy. BACKGROUND: Driving is generally restricted and monitored in people with epilepsy. Little is known about the frequency of driving and subsequent accidents specifically in individuals with uncontrolled epilepsy. METHODS: In an ongoing, prospective, multicenter study of resective epilepsy surgery, individuals were interviewed when they presented for surgical evaluation. Analyses were conducted using chi-square, t-tests, and multiple logistic regression. RESULTS: Of 367 eligible participants, 115 (31.3%) had driven in the last year, most on at least a weekly basis. In a multivariable analysis, factors associated with an increased likelihood of driving were having a current license (OR = 10.71, p < 0.001) and ever having had a license (OR = 3.86, p = 0.003). Younger individuals were also more likely to drive. Lower levels of driving were found in women (OR = 0.31, p < 0.001), individuals who were self-described as disabled (OR = 0.20, p < 0.001), and those who were employed full-time (OR = 0.43, p = 0.03) or part-time (OR = 0.15, p = 0.005). At some point in the past, 144 individuals experienced one or more seizures while driving, and 98 experienced at least one accident because of a seizure. Of those who had accidents, 94% reported property damage, 32% had an injury, and 20% caused injury to others. CONCLUSION: Despite restrictions, almost one third of individuals with refractory epilepsy drive. Understanding why they do may help identify means of modifying this behavior or identifying services that, if provided, would help people with uncontrolled epilepsy forego driving.

Adult↗

[Causes of late-onset epilepsy in an epilepsy clinic of Santa Catarina--Southern Brazil].

OBJECTIVE: To identify the most common etiologies of late-onset epilepsy in our society. METHOD: Retrospective and descriptive study in the CME/SUS of Florianópolis from 1990 to 1998, evaluating 120 handbooks of patients with late-onset epilepsy, that is, those that had initiated epileptic seizures of the 18 years in ahead. The collected variables were: age of the first seizure, etiologies and morbid familial history. RESULTS: The prevalence rate found for late-onset epilepsy was 29.48%. Most of the sample had partial symptomatic epilepsy and the most frequent and important were: cysticercosis with 20%, head trauma with 15%, febrile convulsions in infancy with 5% and 35% were classified as idiopatic. CONCLUSION: Prevalence of late-onset epilepsy in our society is 29.48%, value slightly upper to the told one in developed countries (25%). Very probably, this difference is consequence of the epidemic character of cysticercosis among us. Moreover, head trauma and febrile convulsions are common etiologies. Prophylaxis of epilepsy is viable and urgently necessary in our society.

Adolescent↗

Mah-jong epilepsy: a new reflex epilepsy.

BACKGROUND: Flickering light and color patterns, reading, language, movement, decision making, eating, tapping and touching, hot water immersion and auditory stimulation can induce seizures in some patients. This is known as "reflex epilepsy". The mechanism of reflex epilepsy is not clear. Recently, we cared for 12 patients (11 men and 1 woman, age 41-74 years; mean age = 63.1 years) whose seizures were induced by playing mah-jong, with mean age at seizure onset of 48.7 years. Nine of the 12 patients had seizures exclusively while playing mah-jong. METHODS: We retrospectively reviewed the medical records of 12 epileptic patients who visited our neurologic clinic from 1987 to 1999, with the chief complaint of mah-jong induced seizures. All patients underwent examinations including electroencephalography, brain computed tomography or magnetic resonance imaging, analysis of clinical manifestation of seizures, family history and past medical history. All were given anticonvulsant therapy for preventing seizures. RESULTS: Clinically, 10 patients had generalized tonic-clonic seizures and two patients had partial seizures with secondary generalization. Interictally, normal results on EEG were found in six patients, three had focal temporal spikes, and three had intermittent slow activity in the frontotemporal regions. Neuroimaging studies of the brain were normal in seven patients, two had lacunar infarctions, one had generalized atrophy and one had focal left parietal lesions of an unclear nature. Only one patient had a family history of epilepsy. All 12 patients received anticonvulsant therapy, and of these, nine had good epilepsy control. CONCLUSIONS: "Mah-jong epilepsy," a new reflex epilepsy, is probably related to thinking and decision making while playing mah-jong. The easy control of seizures induced by mah-jong suggests a benign nature. In addition to anticonvulsant therapy, avoiding playing mah-jong may be essential in preventing seizures.

Adult↗

Pregnancy in women with epilepsy : preliminary results of Kerala registry of epilepsy and pregnancy.

Eighty-five women with epilepsy were followed up for reproductive functions under the registry of epilepsy and pregnancy. 32 of them had completed the pregnancy. Their mean age was 26 years and mean seizure frequency was 0.7 during current pregnancy. Nineteen of them (59.4%) had generalized epilepsy. Nine of them were not on any anti epileptic drugs (AED), 23 women were on various AEDs, 19 being on monotherapy. Only 40% of the women were taking folic acid during pregnancy. Pregnancy ended as spontaneous abortion in one patient. Nearly one third required cesarean section. Majority (87.5%) had term babies. Three (10.7%) babies had birth asphyxia. Six babies (21.4%) had low birth weight. Congenital malformations were detected in four cases (12.5%). Malformations included neural tube defects, talipes equinovarus and other minor anomalies. These babies were exposed to sodium valproate, carbamazepine or phenobarbitone. The risk of malformation was significantly greater (p<0.05) when the mother had generalized epilepsy. The odds ratio for risk of malformation was much higher with sodium valproate (6) than that with carbamazepine (1.2) or phenobarbitone (0.8). Majority of women with epilepsy had safe pregnancy and childbirth without any aggravation of epilepsy.

Adult↗

[Epidemiology in epilepsy. Epilepsy primarily affects small children and the elderly].

Epilepsy is one of the commonest neurological/disorders, with peak incidences among the elderly and among children in the first year of life. Approximately 60,000 (0.7 per cent) of the Swedish population, 50,000 adults and 10,000 children, have active epilepsy. Stroke is the aetiology most commonly identified. The majority of those with a first-ever unproven seizure suffer no further seizures. Of those who do have further seizures (i.e., who develop epilepsy), 65-85 per cent eventually become free from seizures, and many of them are able to cease antiepileptic medication. Epilepsy is a heterogeneous disorder. The larger proportion of patients who are otherwise healthy, and who respond readily to antiepileptic treatment and manifest no side effects or only mild ones, are characterised by the best prognosis and are able to terminate treatment within 2-5 years without recurrence. At the other extreme is the smaller group of patients with onset of epilepsy very early in life, and characterised by multiple severe neurological defects, severe daily seizures, resistance (more or less) to all available treatment, and high mortality. In many cases of epilepsy, however, severity and prognosis lie somewhere between these two extremes.

Adolescent↗

[Treatment of childhood epilepsy with lamotrigine. An evaluation of efficacy in different types of epilepsy].

Fifty-two children with intractable epilepsy received lamotrigine as add-on therapy on a compassionate basis. The results were reviewed after three and six months of treatment in order to evaluate the efficacy in different epilepsy syndromes. Mental retardation was present in 60% of the children. Ictal EEG was obtained in 38 children. At three months the median monthly seizure frequency was reduced from 46 to 14 in the 37 children that still received lamotrigine (p < 0.01). Seven children were seizure-free. Seizure reduction was most impressive in generalized epilepsy, since 63% of these had more than 50% seizure reduction compared to 18% in partial epilepsies (p < 0.05). This difference was unchanged after six months of treatment. Side effects were reported in 18 of the children. In 13 children the parents reported an improved wellbeing. Lamotrigine seems to be an efficient antiepileptic drug-especially in generalized epilepsy.

Adolescent↗

Employment Concerns of People with Epilepsy and the Question of Disclosure: Report of a Survey of the Epilepsy Foundation.

This article presents the results of a survey of the Epilepsy Foundation affiliates in the United States and Puerto Rico, regarding the employment-related questions and concerns of people with epilepsy. The survey also explored how Epilepsy Foundation affiliates advise people with epilepsy about disclosing epilepsy status in the employment-seeking process. Some of the major employment-related questions and concerns reported by the affiliates included identifying resources to help in finding a job, questions about disclosure of epilepsy status in job applications, questions about job-related discrimination or Americans with Disabilities Act (ADA) violations, questions about the ADA, and questions about how to deal with co-workers or supervisors. Implications and design limitations are discussed.

Journal Article↗

Long-term prognosis in two forms of childhood epilepsy: typical absence seizures and epilepsy with rolandic (centrotemporal) EEG foci.

Findings in children seen between 1955 and 1965 during the year of onset of typical absence seizures (90 patients) or rolandic epilepsy (79 patients) were analyzed by actuarial methods. One hundred and eighteen patients were followed for more than 15 years. Rolandic epilepsy is a true benign epilepsy ending with puberty. Although school and family problems are common during the acute stage of the disease, the social adaptability of such patients is excellent. We considered only typical absences occurring as a first epileptic sign in normal children. Myoclonic or atonic absences have a poor prognosis. Many patients with simple and automatic absences experience remission 15 years after withdrawal of medication. The overall cessation rate in those experiencing absences was only 57.5%, however, and 36% of patients developed tonic-clonic seizures. Social adaptability was often inadequate. Simple and automatic absences (constituting a homogeneous group) are not truly a benign form of epilepsy, even though prognosis for those afflicted is better than that for those with other forms of primary generalized epilepsy.

Adolescent↗

Does epilepsy surgery lower the mortality of drug-resistant epilepsy?

Drug-resistant epilepsy has proved to be associated with an increased standardized mortality ratio (SMR), primarily due to seizure-related fatalities including sudden unexpected death (SUDEP). Recent studies have suggested that the surgical cure of temporal lobe epilepsy (TLE) was likely to normalize the SMR of patients suffering from refractory TLE. However, these studies raise a number of methodological issues, which have not always been fully addressed. Some conclusions have relied on previously reported data, indicating a SMR of approximately 5, and a SUDEP incidence of 9/1000 patient-years in drug-resistant epilepsy. In fact, as shown in this review, SMR varied considerably, from 2 to 16, in the various series of patients with refractory epilepsy, whereas the average SUDEP incidence in the same populations was calculated at 3.7/1000 patient-years. Other conclusions were based on the comparison of either surgically and medically treated patients, or cured and non-cured operated patients. In both situations, the two groups included a different proportion of excellent and poor surgical candidates. The biological differences that distinguish these two populations might explain part of the differences observed in their mortality rate, regardless of the effect of surgery. In particular, temporal-plus epilepsies involving the insula, the frontal orbital, or the frontal operculum region, might favour ictal arrhythmias, central apnoea and secondary generalization, which in turn would increase the risk of SUDEP. Future studies are thus warranted to specifically address these issues.

Age Factors↗