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The nature of epilepsy in the general population. I. Characteristics of patients receiving medication for epilepsy.

The study is a community-based study carried out in the UK to determine the characteristics of patients receiving treatment for epilepsy, with particular reference to the duration, nature and severity of epilepsy. 119 participating general practitioners distributed questionnaires to 2528 patients taking medication for epilepsy. Information requested included the age and sex distribution of the patients, seizure type, duration of epilepsy and frequency of seizures, employment status of the patients, and state benefits received. 1628 patients replied. The prevalence of people receiving treatment for epilepsy was estimated at 4.5/1000. Fourteen per cent were under the age of 20 years and 23% were aged 60 or more. Seventy per cent had had epilepsy for at least 5 years, while 8% had been diagnosed in the previous year. Fifty-three per cent had had one or more seizures in the year prior to the survey, and 20% had seizures at least monthly on average. Most patients had seizures of partial origin. The rate of unemployment and receipt of social security benefits was higher than in the general population. The study indicates that the majority of patients receiving treatment for epilepsy in the community have long-standing epilepsy, often intractable to medical treatment, and associated with considerable social handicap. Health care planning should take this into account.

Adolescent↗

Outcome assessment in epilepsy: available rating scales for adults and methodological issues pertaining to the development of scales for childhood epilepsy.

During the past decade, several scales have been developed to improve the assessment of outcome in epilepsy. These scales were developed for adults and their reliability, validity and usefulness have been established. However, there is also a need for alternative measures of outcome in childhood epilepsy, especially a measure of seizure severity (SS) and measures pertaining to quality of life (QoL). Four of these adult scales are reviewed and compared to examine their applicability in childhood epilepsy. Two important methodological differences between them are discussed: (a) patient self-report vs. physician-based scales and (b) generic vs. disease-specific instruments. QoL in epilepsy is briefly reviewed. Severity of seizures and severity of side-effects are relatively neglected areas of importance to QoL in epilepsy. The existing instruments for adults are not appropriate for children in their present form. Some specific methodological issues, which are relevant for the development of scales for children with epilepsy, are subsequently discussed. New scales pertaining to physical and psychosocial aspects of QoL in childhood epilepsy are being developed. In the near future, data on their reliability, validity and usefulness will become available. A combination of scales focusing on specific aspects of QoL, including SS and severity of adverse effects, and more traditional clinical data may provide a more complete assessment of outcome in childhood epilepsy.

Adult↗

Prevalence of epilepsy and health status of adults with epilepsy in Georgia and Tennessee: Behavioral Risk Factor Surveillance System, 2002.

Behavioral risk factors associated with comorbidity in people with epilepsy are largely unknown. We studied a population-based sample of 8057 adults through the 2002 Behavioral Risk Factor Surveillance System, in Georgia and Tennessee, ascertaining a lifetime epilepsy prevalence of 2.1% in this population. This structured interview revealed that those with epilepsy had significantly worse self-reported fair or poor health status (39% vs 17% in adults without epilepsy), significantly greater cigarette smoking (38.8% vs 24.9% in other adults), and high rates of obesity (34.1% vs 23.7% in adults without epilepsy). Large percentages of adults with epilepsy reported currently symptomatic asthma and recent joint pain. Adults with epilepsy had lower educational attainment and lower household incomes, but a higher rate of medical insurance coverage, than did other adults. This type of population-based survey can serve to identify health disparities, behavioral risk factors for other chronic diseases, and unmet health care needs in individuals with epilepsy, and to track changes in these measures over time.

Adolescent↗

Postpartum depression in women with epilepsy versus women without epilepsy.

The goal of this study was to determine if there is a significant difference in the rate of postpartum depression among patients with epilepsy and healthy controls. All patients were recruited from the Epilepsy Center and the Department of Obstetrics and Gynecology, University of Milan, St. Paolo Hospital (Milan, Italy). Thirty-five pregnant women with epilepsy and an equal number of pregnant women without epilepsy were assessed with the Edinburgh Postnatal Depression Scale (EPDS), a clinical interview used to screen for postpartum depression (PPD), and a sociodemographic questionnaire. The rate of PPD in patients with epilepsy was statistically significantly higher than that of the controls (P<0.05). PPD was present in 29% of the patients with epilepsy and 11% of the controls. In conclusion, it is very important to point out that in our pilot study, the rate of PPD was higher among women with epilepsy than among women without epilepsy. In this regard, women at higher PPD risk can be identified earlier and treated as soon as possible to alleviate their symptoms and improve their quality of life.

Adult↗

Teachers' perceptions in central Turkey concerning epilepsy and asthma and the short-term effect of a brief education on the perception of epilepsy.

This study was aimed at assessing and comparing schoolteachers' perceptions of epilepsy and asthma in central Turkey. Two-hundred seventy-five schoolteachers completed a questionnaire on their knowledge, attitudes, and practice. There were 18 pairs of questions on the questionnaire (one question in each pair pertained to epilepsy, and the other to asthma). One month later, following a brief education program on epilepsy, participants completed the epilepsy section of the questionnaire a second time. Among the teachers, initial rates of acceptance of children with epilepsy were significantly lower than the rates for asthma. Children with epilepsy were less encouraged by teachers to play with others and were perceived as more aggressive. More teachers were worried about other parents objecting to having a child with epilepsy in the class. Following the brief education program, there was a significant positive change in the teachers' perception of epilepsy.

Adult↗

Neuroimaging in the evaluation of children and adolescents with intractable epilepsy: II. Neuroimaging and pediatric epilepsy surgery.

The costs of epilepsy encompass all aspects of life, including medical, educational, and psychosocial. Adults with intractable epilepsy who undergo epilepsy surgery and have seizure-free outcomes still have significant barriers in the attainment of improved quality of life. For this reason, there is increasing interest in the recognition of children and adolescents with intractable epilepsy who might be epilepsy surgery candidates. This is Part II of an article on the role of neuroimaging in the evaluation of children and adolescents with intractable epilepsy. Part I addressed the role of MRI in detecting the substrates of epilepsy (Pediatr Neurol 1997;17: 19-26); Part II elaborates on the selection process of pediatric patients who might benefit from epilepsy surgery. Although EEG remains the cornerstone of the evaluation process, MRI, SPECT, and PET can play a pivotal role in the identification of the underlying epileptogenic focus and minimize the need for invasive EEG monitoring. Magnetic resonance spectroscopy and magnetoencephalography are also innovative, noninvasive techniques which may aid in the localization of the epileptogenic focus. Functional MRI scans may soon replace invasive technologies in the identification of eloquent cortex that should not be a part of the surgical resection.

Adolescent↗

Are what people know about their epilepsy and what they want from an epilepsy service related?

This study set out to investigate the level of knowledge about epilepsy in general and in relation to the patients' own condition, in patients attending a tertiary referral epilepsy outpatient clinic. It also sought to investigate patient satisfaction with the service and whether knowledge acquired about epilepsy related to that satisfaction. Seventy out of 94 patients responded to the Epilepsy Knowledge Profile Questionnaires (general and personal knowledge of epilepsy) and a questionnaire assessing service satisfaction. Patients were found to know more about epilepsy in general than about their own condition. In particular some patients were unable to give accurate indications of their drug regimes. Over 91% were satisfied with the serviced they received but this bore no relation to information they had acquired or wanted about epilepsy. Multidisciplinary services were requested by a sizeable percentage of patients but especially access to a specialist nurse in epilepsy. The study highlights the need for clinicians to check patients' knowledge about their condition and for further work to clarify the source of patients' satisfaction with service delivery.

Adolescent↗

Effect of epilepsy on psychomotor function in children with uncomplicated epilepsy.

This study assessed the impact of epilepsy on motor function in children. We aimed to analyze the effect in uncomplicated epilepsies (cryptogenic partial and idiopathic generalized epilepsy). A group of 87 children with epilepsy (47 males, 40 females; mean age 8y, standard deviation [SD] ly 9mo, range 4y 11mo to 12y 11mo), but without learning disability* or other neurological comorbid disorders, was compared with a control group of 107 children (76 males, 31 females; mean age 8y 4mo, SD 2y 2mo, range 4y 7mo to 12y 2mo). The differences in main motor skills and psychomotor speed were analyzed using the Movement ABC and computerized measures for simple reaction times and finger tapping. No significant difference in motor function was found. The overall psychomotor development of children with epilepsy is comparable to controls. However, a significant slowing of psychomotor speed in the group with epilepsy was reported. No relation with antiepileptic drug treatment was demonstrated and no difference in psychomotor speed between different types of epilepsy was found. The existence of a condition leading to excessive neuronal discharge leads to a general cortical inhibition. The slowing of psychomotor speed in children with uncomplicated epilepsy may be the behavioural presentation of this inhibition.

Child↗

Neuroepidemiological study of childhood epilepsy by application of international classification of epilepsies and epileptic syndromes (ILAE, 1989).

A population-based survey of childhood epilepsy was made in 1975 on the total population of children aged < 10 years living in Okayama Prefecture (n = 2,378 patients). Using the data obtained, we attempted to reclassify the various types of epilepsy according to the international classification (ILAE, 1989). Reclassification was possible in 1,872 (78.7%) of the 2,378 cases. The 1,872 cases consisted of 1,045 (55.8%) with localization-related epilepsies, 824 (44.0%) with generalized epilepsies, and 3 (0.2%) with epilepsies undetermined whether focal or generalized. Classification of the epilepsies in a population-based survey using the international classification involves difficulties, because both clinical and EEG findings are essential. However, if an appropriate area is selected, classification of epilepsies and epileptic syndromes in a population-based survey is possible by referring to all medical records stored at every hospital and practitioner's clinic that administers treatment to patients with epilepsy in the area.

Age Factors↗

Behavioural abnormalities and retention rates of anti-epilepsy drugs during long-term treatment of epilepsy: a clinical perspective.

Vigabatrin is among the most promising of the new anti-epilepsy drugs, but two unrelated complications have been noted in patients receiving vigabatrin for chronic refractory epilepsy. These are, transient behavioural abnormalities and falling numbers of patients continuing to take vigabatrin (decreasing retention rate) during long-term treatment. Psychotic reactions have been carefully documented both in patients receiving standard and new anti-epilepsy drugs, and in up to 6% of patients after epilepsy surgery. As many psychotic episodes are associated with a dramatic cessation of seizures (i.e. high efficacy of treatment), it is not surprising that behavioural abnormalities are more often seen in patients receiving higher, more effective doses of vigabatrin, particularly those taking more than 3 g/day. A gradual dose increase and slow withdrawal is recommended for possible prevention of behavioural abnormalities, at least in some patients. During treatment with standard anti-epilepsy drugs, approximately 50% of patients remain on randomized treatment with the same drug for several years. For oxcarbazepine, a new drug, the figure is 60%, and for vigabatrin it is about 50%. Although comparative trials are not available, the retention rate for vigabatrin does not appear to be strikingly different to that reported for conventional anti-epilepsy drugs. In summary, behavioural abnormalities and falling retention rates appear to be general features of chronic epilepsy, and are certainly not exclusively associated with the use of vigabatrin. Controlled trials are required to establish the specific contribution, if any, of vigabatrin in development of these complications of intractable epilepsy.

Anticonvulsants↗

The misdiagnosis of epilepsy in children admitted to a tertiary epilepsy centre with paroxysmal events.

AIMS: To determine the proportion of children admitted with difficult to treat paroxysmal events to a tertiary epilepsy centre who did not have epilepsy. METHODS: In an observational retrospective study, all case notes of 223 children admitted in 1997 were examined. The referral was made from the local paediatric department in 51% of cases, other departments in 27%, and from general or specialist practitioners in 22%. Doubt regarding the diagnosis of epilepsy was expressed in the referral note in 17%. On admission, 86% were on antiepileptic drug treatment. During admission all children were subjected to a comprehensive intensive observation and 62% had EEG monitoring. RESULTS: In total, 39% (87/223) were found not to have epilepsy. In 30% of children (55/184) referred without any doubts about the epilepsy diagnosis, the diagnosis was disproved. Of the 159 children admitted for the first time, 75 (47%) were discharged with a diagnosis of non-epileptic seizures. Of 125 children admitted for the first time with no doubts about the diagnosis of epilepsy, 44 (35%) did not have epilepsy. Staring episodes were the most frequently encountered non-epileptic paroxysmal event. Psychogenic non-epileptic seizures were found in 12 children. A total of 34 (15%) had their medication tapered off; a further 22 (10%) had tapered off medication before admission. CONCLUSION: The present study supports the view that misdiagnosis of epilepsy is common. The treating physician should be cautious in diagnosis, especially of staring episodes. A diagnostic re-evaluation should be undertaken in difficult cases with continuing paroxysmal events in order to avoid unnecessary drug treatment and restrictions on the child's lifestyle.

Adolescent↗

Northern epilepsy syndrome: an inherited childhood onset epilepsy with associated mental deterioration.

A new autosomal recessively inherited disease of the central nervous system involving childhood epilepsy and mental deterioration is described. Twenty three patients (11 males and 12 females) belonging to 11 families from northern Finland have been identified. A common ancestor has been found for nine families. The mean age of onset of epilepsy was 6.7 years (range 5-10 years) and the epilepsy was characterised by generalised tonic-clonic seizures increasing in frequency up to puberty. One third of the patients also had complex partial seizures during childhood. During young adulthood the epileptic activity began to decrease, but complete remission did not occur. Electroencephalography showed progressive slowing of the background activity with relatively scanty epileptiform activity. Out of four ictal recordings the paroxysmal activity was initiated focally in two cases. Clonazepam and sodium valproate had some antiepileptic effect, clonazepam being the more beneficial of the two. Mental development, which was originally normal, began to deteriorate two to five years after the onset of epilepsy, and the deterioration continued during adulthood in spite of good epilepsy control, leading to mental retardation by middle age. The pathogenesis of the disorder, called the Northern epilepsy syndrome, is unknown. Linkage analysis using DNA markers linked to the EPM1 gene for progressive myoclonus epilepsy of Unverricht-Lundborg type showed that the Northern epilepsy syndrome is not allelic to EPM1.

Adolescent↗

Epilepsy genes and the genetics of epilepsy syndromes: the promise of new therapies based on genetic knowledge.

Treatment strategies based on the molecular biology of the epilepsies may soon become a reality. Critical steps in this process are identifying molecular genetic defects in specific epilepsies, understanding of the neurobiologic consequences of those defects, and developing methods to correct the molecular defects or their downstream consequences. Identification of molecular defects is easier in single-gene epilepsies than in those with complex inheritance, although the latter are more common. A number of epilepsies have been mapped and, in two cases, specific genes have been identified. Unverricht-Lundborg disease is caused by defects in the cystatin B gene, with absence of the gene product. Autosomal dominant nocturnal frontal lobe epilepsy in some families is caused by mutations in the alpha4-subunit of the nicotinic acetylcholine receptor gene. In vitro studies suggest that the mutations lead to impaired function of the acetylcholine receptor, raising the possibility of cholinergic therapy for this condition. Advances in the molecular biology of the epilepsies are likely to change our understanding radically and to allow opportunities to develop innovative new treatments for epilepsy.

Animals↗

Gamma knife radiosurgery as a primary treatment for prolactinomas.

OBJECT: The purpose of this study was to estimate the efficacy of gamma knife radiosurgery (GKS) in controlling tumor growth and endocrinopathy associated with prolactinomas. METHODS: Between 1993 and 1997, 164 of 469 patients with pituitary adenomas treated by GKS harbored prolactinomas. The dose to the tumor margin ranged from 9 to 35 Gy (mean 31.2 Gy), and the visual pathways were exposed to a dose of less than 10 Gy. The mean tumor diameter was 13.4 mm. The mean follow-up time for 128 cases was 33.2 months (range 6-72 months). Tumor control was observed in all but two patients who underwent surgery 18 and 36 months, respectively, after GKS. Clinical cure was achieved in 67 cases. Clinical improvement was noted with a decrease in the hyperprolactinemia after GKS. Nonetheless, in 31 (29%) of 108 patients who were followed for more than 2 years no improvement in serum prolactin levels was demonstrated, although this could be normalized by bromocriptine administration after treatment. Nine infertile women became pregnant 2 to 13 months after GKS and all gave birth to normal children. There was no visual deterioration related to GKS. Five women experienced premature menopause. In these patients there was subtotal disappearance of the tumor and an empty sella developed. CONCLUSIONS: Gamma knife radiosurgery as a primary treatment for prolactinomas can be safe and effective both for controlling tumor growth and for normalization of prolactin hypersecretion. A higher margin dose (> or = 30 Gy) seemed to be associated with a better clinical outcome. Gamma knife radiosurgery may make prolactinomas more sensitive to the bromocriptine.

Humans↗

The coexistence of idiopathic generalized epilepsy and partial epilepsy.

PURPOSE: To describe the clinical, EEG, and imaging data of a series of patients with features of both idiopathic generalized epilepsy (IGE) and partial epilepsy. METHODS: A computerized database of all patients attending the regional epilepsy clinic was used to identify all patients with IGE. Case notes were reviewed, and cases with clinical evidence of co-existing partial epilepsy identified. RESULTS: Nine cases with clinical features of a partial and generalized-onset epilepsy and with electrophysiological evidence of IGE are presented. This represented <1% of the overall IGE population in the clinic. Five (55.6%) patients are currently in remission, with successful epilepsy surgery in one patient. CONCLUSIONS: The coexistence of IGE and partial epilepsy is uncommon, and if appropriate treatment with a broad-spectrum antiepileptic drug is given, then the prognosis can be good. Surgery should be considered for those cases with medically intractable partial-onset seizures as part of this syndrome.

Adult↗

Language-induced epilepsy, acquired stuttering, and idiopathic generalized epilepsy: phenotypic study of one family.

PURPOSE: Language-induced epilepsy involves seizure precipitation by speaking, reading, and writing. Seizures are similar to those of reading epilepsy (RE). The nosologic position of language-induced epilepsy is not clear. We performed a clinical and neurophysiological study in a multigenerational family with the association of idiopathic generalized epilepsy (IGE) with ictal stuttering as a manifestation of reflex language-induced epilepsy. METHODS: Nine members on three generations were studied. All patients underwent video-polygraphic EEG recordings (awake and during sleep). A standardized protocol was applied to test the effect of language and non-language-related tasks. RESULTS: Six patients presented language-induced jaw jerking that mimicked stuttering and corresponded to focal myoclonus involving facial muscles. This was associated with an IGE phenotype in four of these patients. Focal EEG spikes were found in all six patients by visual analysis and/or back-averaging techniques. The focal spikes were either asymptomatic (when followed by a slow wave) or symptomatic of facial myoclonia (when isolated). Levetiracetam, used as add-on or monotherapy in four patients, suppressed ictal stuttering. One additional case only had a phenotype of IGE without focal features. CONCLUSIONS: This family study demonstrates the phenotypic heterogeneity of the association of IGE phenotype with ictal stuttering (language-related reflex seizure). Our data suggest that this particular form of reflex epilepsy related to language has more similarities with generalized epilepsies than with focal ones. Neurophysiological investigations should be performed more systematically in patients with acquired stuttering, especially if there is family history of IGE.

Adolescent↗

Do children with benign rolandic epilepsy have a higher prevalence of migraine than those with other partial epilepsies or nonepilepsy controls?

PURPOSE: Prior studies have given conflicting data concerning the association of benign rolandic epilepsy of childhood (BREC) and migraine but were limited by lack of sensitive, diagnostic criteria for childhood migraine. By using revised International Headache Society (IHS-R) criteria, we compared the prevalence of migraine in children with BREC with that of those (a) with cryptogenic/symptomatic partial epilepsy and (b) without epilepsy. METHODS: Three cohorts of children, gender and age matched (within 1 year) were identified: (a) BREC, (b) cryptogenic/symptomatic partial epilepsy, and (c) no history of seizures. Parents were queried in a standardized interview about migraine and migraine equivalents in their child, and in either biologic parent. Migraine was defined by using the IHS-R (for children) and IHS criteria (for parents). Children with headache were divided into definite (meeting IHS-R criteria), probable (recurrent, throbbing headaches with nausea, vomiting, photophobia or phonophobia, not meeting IHS-R criteria), possible (recurrent headaches with throbbing character or associated nausea/vomiting), or nonmigraine groups. chi(2) analysis was used to determine whether the cohort with BREC had a higher prevalence of definite, definite or probable, or definite, probable, or possible migraines or migraine equivalents than the other two cohorts. RESULTS: Each cohort consisted of 53 children (mean age, 9.8-9.9 years, M/F ratio, 35:18). Those with BREC had higher rates of definite and probable (p = 0.05), of definite, probable, and possible migraine (p = 0.05), and of migraine equivalents excluding motion sickness (p < 0.005) than did those without seizures; however, they did not differ significantly from the cryptogenic/symptomatic partial epilepsy cohort. CONCLUSIONS: Partial epilepsy, regardless of etiology, is associated with higher rates of migraine in children. The pathophysiologic link between epilepsy and migraine is unknown.

Child↗

Familial occurrence of febrile seizures and epilepsy in severe myoclonic epilepsy of infancy (SMEI) patients with SCN1A mutations.

PURPOSE: The role of the familial background in severe myoclonic epilepsy of infancy (SMEI) has been traditionally emphasized in literature, with 25-70% of the patients having a family history of febrile seizures (FS) or epilepsy. We explored the genetic background of SMEI patients carrying SCN1A mutations to further shed light on the genetics of this disorder. METHODS: We analyzed the occurrence of FS and epilepsy among first- and second-degree relatives (N = 867) of 74 SMEI probands with SCN1A mutations (70 de novo, four inherited) and compared data with age-matched and ethnically matched control families. Familial clustering and syndromic concordance within the affected relatives in both groups were investigated. RESULTS: The frequency of FS or epilepsy in relatives of SMEI patients did not significantly differ from that in controls (FS: 13 of 867 vs. 12 of 674, p = 0.66; epilepsy: 15 of 867 vs. six of 674, p = 0.16). Different forms of epilepsy were identified in both relatives of SMEI probands and controls. Twenty-eight relatives with FS and epilepsy were distributed in 20 (27%) of 74 SMEI families; among the controls, 18 affected relatives were clustered in 13 (18.5%) of 70 families. No pedigree showed several affected members, including the four with inherited mutations. CONCLUSIONS: A substantial epileptic family background is not present in our SMEI patients with SCN1A mutations. These data do not confirm previous observations and would not support polygenic inheritance in SMEI. The investigation of the family background in additional series of SMEI patients will further shed light on the genetics of this syndrome.

Epilepsies, Myoclonic↗