Prevalence of Epilepsy in Adults in Northern Sweden

A multisource medical register review identified persons with active epilepsy in northern Sweden. Seven hundred thirteen persons aged greater than or equal to 17 years with epilepsy were determined on the prevalence day, December 31, 1985. The overall prevalence was 553 in 100,000 (566 in 100,000 if adjusted to the 1980 U.S. population). The ratio of males to females was 1.1, with a male prevalence of 575 and a female prevalence of 530 in 100,000. Age-specific prevalences varied between 530-644 in 100,000 except in persons aged greater than or equal to 70 years, for whom the prevalence was 321 in 100,000. Partial seizures were most common, 333 in 100,000, of whom the majority (250 in 100,000) had seizures that occasionally were secondarily generalized. Mental retardation was the foremost coexistent disorder, noted in 23%. The mean yearly seizure frequency was higher in persons with mental retardation than in nonretarded persons. Seventeen percent had seizures during the last week, 57% during the last year, whereas 16% had greater than or equal to 5 years' freedom from seizures. Most had onset of epilepsy before age 20 years. A presumed etiology was noted in 35%, more often in men than in women. Cerebrovascular disease was the most commonly identified presumed cause. Other nonepileptic diseases/disabilities were noted in 47%.     

Epilepsy in a population of mentally retarded children and adults

All mentally retarded (MR) subjects in a northern Swedish county were assessed for the occurrence of active epilepsy on a prevalence day. Active epilepsy was found in 299 subjects (20.2% of those with MR) corresponding to a crude prevalence rate of 1.2/1000 inhabitants. The age-specific prevalence for 0-9 years was higher for females than for males, while in other age groups it was slightly higher for males or showed no difference between the sexes. Epilepsy and MR were the only disorders in 129 subjects (43.1%). Cerebral palsy was the most common associated disorder and occurred in 100 (33.4%). A presumable etiology for epilepsy and MR was identified in 73.2% and 71.9%, respectively. The presumable etiological factors which caused MR occurred prenatally in 35%, perinatally in 10% and postnatally in 9%. The pathogenetic period was unknown in 31%. In 15%, the etiological events occurred during more than one of the above periods. The presumable causes were responsible for both epilepsy and MR in all except 7 cases. MR individuals with epilepsy were significantly more retarded than those without epilepsy. The first seizure occurred during the neonatal period in 11.6% and before 1 year of age in 27.7%. Generalized tonic-clonic seizures were the most common type and occurred in 204 subjects (68.2%). Seventy-one of these also had partial seizure manifestations. Daily to weekly seizures occurred in 26.8% and 32.0% had been seizure-free for the past year.     

Prevalence of Epilepsy in a Rural Community of Guatemala

Summary: We conducted a cross-sectional epidemiological study to assess the prevalence and patterns of epilepsy in a small rural village of Guatemala (population 2,111); 1,882 subjects (97.3%) were surveyed. By administering the World Health Organization (WHO) standard questionnaire and performing neurological examinations, we detected 16 cases of epilepsy. The crude prevalence rate for this community was 8.5 in 1,000 general population. Eleven cases were further classified as active epilepsy, giving a prevalence rate of 5.8 in 1,000 general population for this form. The most common type of seizure was generalized tonicclonic seizures (GTCS, 50%), followed by complex partial seizures (CPS, 37.5%), simple partial seizures (SPS, 6.2%) and generalized atonic seizures (6.2%). The age-specific prevalence ratio was highest among the group aged 20–29 years, although the difference between that group and the other age groups was not statistically significant ( z < 2, p > 0.05). Fourteen persons (87.5%) had sought medical care for their seizures at least once in their lifetime, 5 (31.25%) were receiving an antiepileptic drug (AED), and 9 (56.25%) had previously received treatment and 2 (12.5%) had never been treated for their illness. Phenobarbital was the most common AED prescribed; 7 persons had positive family history of epilepsy, 5 reported a history of significant head trauma, 4 had history of central nervous system disease, and 1 had a history of chronic alcohol intake.     

Influence of Epilepsy on Mortality in Mental Retardation: An Epidemiologic Study

Summary: Purpose : A cohort consisting of all persons with known mental retardation (MR) and living in a Swedish province on December 31, 1985, was followed for 7 years (1987–1992) to study the mortality pattern.
Methods : A file of the cohort was linked to the cause-of-death pattern of the general population in the study area.
Results : One hundred twenty-four deaths (8.4%) occurred among the 1,478 persons with MR. Thirty deaths (10.1%) occurred among the 296 persons with epilepsy and MR. The standardized mortality ratio (SMR) in those with only MR was significantly increased as compared with that of the general population: 1.6 [95% confidence interval (CI) 1.3–2.01; MR and epilepsy, 5.0 (CI 3.3–7.5); and MR, epilepsy, and cerebral palsy (CP), 5.8 (CI 3.4–9.7). Mortality was increased both in patients with partial seizures without seizures secondarily generalized (SMR 3.7, CI 1.0–13.6) and in patients with seizures secondarily generalized (5.0, CI 2.3–11.0). The highest mortality occurred in patients who had seizures that were always generalized from the onset: 8.1 (CI 5.7–11.5). Mortality increased with increasing seizure frequency during the year preceding the prevalence date. In patients with epilepsy and MR, pneumonia was the most common cause of death and a seizure was the probable cause of death in 6.7%.
Conclusions : Epilepsy is associated with a significantly increased mortality in persons with MR. The increase is related to seizure type and seizure frequency. Death in persons with epilepsy and MR is seldom directly due to seizures. Other impairments associated with epilepsy and MR are important causes of death.     

Unilateral motor epileptic seizures

Unilateral motor epileptic seizures occurred in 118 of 1902 persons with seizure disorders referred to a consultant neurological practice over a 30 year period. The unilateral motor seizures were nearly always manifestations of partial (focal, localisation related) epilepsy but, particularly if they occurred on different sides on different occasions, could be due to generalised onset epilepsy (10.2% of cases). The risk of cerebral tumour in those with unilateral motor seizures (10.2%) was greater than that for those with all other forms of seizure disorder (relative risk 3.56 times; 95% C.I. = 1.95 to 6.49 times). Unlike the situation in younger persons, if unilateral motor seizures began after the age of 20 years the chance of an underlying cerebral tumour (23.4%) was significantly higher than in other persons with adult onset partial seizures (relative risk 2.62 times; 95% C.I. = 1.44 to 4.77 times) and was much greater than that in all other seizure sufferers (relative risk 8.35 times; 95% C.I. = 4.66 to 14.9 times).     

Seizures in childhood ischemic stroke in Taiwan

In this retrospective study, we collected clinical and radiographic data on children (age range, 1 month to 18 years) with symptoms and radiographic confirmation of seizure after ischemic stroke for the period of January 1996 to July 2006. Thirty-nine out of 94 children with ischemic stroke had poststroke seizures. Thirty-three out of 39 children with poststroke seizures had new onset seizures but only data of 28 were available. Infection was the most common etiology in the early poststroke seizure group (52.4%) but not in the late poststroke seizure group (0%). Infarction involving arterial ischemic stroke of anterior circulation were the most common in both the early poststroke seizure (61.9%) and the late poststroke seizure group (57.1%). Epilepsy was the most common sequelae in both the early poststroke seizure (38.1%) and late poststroke seizure group (100%). Children who had initial focal neurological sign (100% vs. 38.1%; P=0.007) or the focal cortical dysfunction on EEG (85.7% vs. 33.3%; P=0.029) were prone to develop late poststroke seizures. Late poststroke seizures had a high risk of developing poststroke epilepsy (100% vs. 38.1%; P=0.007). We conclude that seizures commonly occur in childhood ischemic stroke. Most poststroke seizures developed at an early stage. Infection was the most common etiology that caused early poststroke seizures in childhood ischemic stroke. Initial focal neurological signs and focal cortical dysfunction on EEG are risk factors for developing epilepsy. Poststroke seizures did not affect mortality, but there was a significant difference in normal outcome and epilepsy between those with or without poststroke seizures.     

Risk Factors for Burns as a Consequence of Seizures in Persons with Epilepsy

Summary: To explore the risk factors and circumstances for burns in a population of persons with epilepsy, we surveyed 244 University of Colorado Health Sciences Center Seizure Clinic patients. Twenty-five had at least one seizure-related burn requiring medical attention (12 were hospitalized), 21 reported burns requiring medical attention not related to seizure activity, and 199 reported no burns. All patients with seizure-related burns had alteration of consciousness during most seizures. Statistical models with logistic regression indicated that a useful model for assessing risk of seizure-related burns in this population of patients could be constructed using three significant variables: lifetime total number of seizures experienced by the patient, the presence of interictal neurologic impairment, and gender. The remainder of the variables studied (percentage of seizures that occurred nocturnally, percentage of seizures preceded by a simple partial seizure (SPS) warning, self-care status, number of seizures in adult life, seizure type, patient age, age of onset of seizure disorder, and presence of burns not associated with seizure) were either not significantly correlated with seizure-related burns or did not significantly improve the success of the statistical model in light of the other variables studied. Most injuries occurred in the home during activities that might have been avoided. They included 10 burns incurred while cooking, 5 incurred while showering (with a plumbing system that permitted exposure to scalding water), and 3 caused by exposed room heaters. A subset of epileptic patients are at increased risk for burns and should minimize situations in which they can be burned.     

Clinical presentation of epilepsy in six villages in an onchocerciasis endemic area in Mahenge,Tanzania

Aims. To describe the clinical manifestations of epilepsy and access to antiseizure treatment in Mahenge in Central Tanzania, an onchocerciasis endemic area with a high prevalence of epilepsy. Methods. A door‐to‐door epilepsy prevalence survey was conducted in four rural and two sub‐urban villages. Trained community workers used five screening questions to identify persons suspected to have epilepsy. Such individuals were interviewed and examined by a neurologist or a medical doctor with additional training in epilepsy, and were tested for Onchocerca volvulus antibodies. Results. A total of 221 out of 8,062 (2.74%) surveyed individuals were confirmed to have epilepsy. The median age at seizure onset was 12 years (interquartile range: 7–16). Seventy‐nine persons with epilepsy (PWE) (36.1%) had a family member with epilepsy, which was a sibling in 52.1%. Tonic‐clonic seizures (142 individuals; 64.2%) were the most common seizure type. Nodding seizures were reported in 12.7% of PWE; the majority of them living in rural villages. Persons with nodding seizures reported more frequent seizures, presented with more psychiatric symptoms, and more often had onchocerciasis antibodies than those with other seizure types. The high rate of individuals with a seizure onset at between seven and 16 years is characteristic of onchocerciasis‐associated epilepsy (OAE). Of the PWE, 77.9% met the criteria for the clinical case definition of OAE. Eighty‐three PWE (37.6%) were not taking any antiepileptic medication. Phenobarbital was the antiepileptic drug most commonly prescribed in 76.1% of treated PWE. Conclusion. The high prevalence of epilepsy in rural villages in Mahenge most likely is related to the high prevalence of OAE. To prevent children developing OAE, strengthening the onchocerciasis elimination programme in Mahenge is urgently needed. Moreover, a decentralised epilepsy treatment programme is also needed to provide uninterrupted access to affordable antiepileptic drugs for the many PWE living in rural villages in the Mahenge area.     

Seizures and epilepsy in elderly patients of an urban area of Iran: clinical manifestation, differential diagnosis, etiology, and epilepsy subtypes

The incidences of seizures and epilepsy in the population show a peak after 60 years of age. Due to the lack of reported clinical aspects of seizure and epilepsy in the older patients in our region in Iran, this study was conducted to describe the clinical manifestation, etiology, differential diagnosis, and epilepsy subtypes of epilepsy and seizure. A cross-sectional retrospective study was performed on all consecutively elderly seizure and epilepsy patients, referred to the Epilepsy Association in the city of Qom, Iran over a 10-year period. A total of 466 patients aged >60 years were admitted. 31 % of the patients had epilepsy or seizure and 69 % of them had non-epileptic events. The most prevalent differential diagnoses in the beginning were syncope and cardiovascular disorders. The most frequent clinical symptom of epilepsy was generalized tonic–clonic seizures (75 %). The most common cause of seizure was systemic metabolic disorder (27 %). In epileptic elderly patients, no cause was ascertained for 38 % and the most frequently observed pathological factors were cerebrovascular diseases, which accounted for 24 %. The most common type of epileptic seizure was generalized epileptic seizures (75 %). 10 % of elderly epileptic patients suffered from status epilepticus, which was primarily caused by anoxia. Despite the rising rate and potentially profound physical and psychosocial effects of seizures and epilepsy, these disorders have received surprisingly little research focus and attention in Iran. Referring older patients to a specialist or a specialist epilepsy center allows speedy assessment, appropriate investigation and treatment, and less likely to miss the diagnosis.     

Systematic Literature Review of Psychiatric Comorbidities in Adults with Epilepsy

Background and PurposeMental illness is disproportionately common in people with epilepsy (PWE). This systematic literature review identified original research articles that reported the prevalence of psychiatric comorbidities based upon clinical assessments in a sample of PWE and assessed the clinical features of the populations found in studies included in our review of mental health comorbidity.MethodsThe included articles were written in English and published from 2008 to 2018, and focused on adults aged ≥18 years who had psychiatric diagnoses determined in clinical assessments, such as those found in medical records, clinician psychiatric evaluations, structured diagnostic interviews, and mental health screening questionnaires specific for a psychiatric disorder. The primary outcome was the prevalence of psychiatric comorbidities as a percentage of the total sample of PWE. Additional data included the overall sample size, mean age, epilepsy type, study design, and method of diagnosis. A modified Newcastle Ottawa Scale was used to assess the quality of the studies. All 23 articles that were consistent with the inclusion criteria were related to observational studies.ResultsMood disorders and anxiety disorders were the most common psychiatric comorbidities, with prevalence rates of 35.0% and 25.6%, respectively. Major depressive disorder was the most common mood disorder, with a prevalence of 24.2%. Post-traumatic stress disorder (PTSD) had the highest reported prevalence among anxiety disorders, at 14.2%, followed by general anxiety disorder at 11.1%. Other comorbidities included psychosis (5.7%), obsessivecompulsive disorder (3.8%), schizophrenia (1.7%), bipolar disorder (6.2%), and substance abuse (7.9%). The pooled prevalence of suicidality, as reported for two studies, was 9.3%. Temporal lobe epilepsy (TLE) was associated with higher levels of psychiatric comorbidity. Two (8.7%) of the 23 studies compared psychiatric comorbidities in TLE with that of extratemporal lobe epilepsy (ETLE), and one of these two studies found that depression was more common in TLE (53.8%) than in ETLE (25%). Regarding seizure types, partial seizures were associated with a higher prevalence of depression vs generalized seizures.ConclusionsThis systematic literature review of recent original research found a relatively high prevalence of mental health comorbidities in PWE. Mood and anxiety disorders are the most common comorbidities, while psychotic spectrum conditions such as schizophrenia and bipolar disorder are much rarer. The prevalence of comorbidity may vary with the epilepsy type and treatment responsiveness. These findings suggest that screening tools for depression and anxiety should be included as part of the training for epilepsy care, while resources for other relatively common conditions such as PTSD and substance abuse disorders should be readily available to neurology specialists who treat PWE.     

Epilepsy in patients with congenital cytomegalovirus infection

Patients with congenital cytomegalovirus (CMV) infection were at high risk for postnatal seizures, but little is known about epilepsy associated with congenital CMV infection. To define the features of epilepsy, we retrospectively reviewed the clinical, laboratory and neuroradiographic findings in 19 children (male 9) with congenital CMV infection. Seven (37%) patients had developed epilepsy (partial seizure 5 and epileptic spasms 2) at a mean age of 20 months (range 2–37 months). During the clinical course, West syndrome occurred in only three patients. The most common seizure type in our series was partial seizure. At the time of last follow-up (mean 96 months), seizures remained uncontrolled in six patients. Neonatal clinical manifestations (gestational age, gender distribution, birth asphyxia or symptoms at birth) were not predictive of the development of epilepsy. On the contrary, some neuroradiographic findings (ventricular dilatation and migration disorder) were significantly associated with the development of epilepsy.     

Long term course of childhood epilepsy following relapse after antiepileptic drug withdrawal

OBJECTIVE: To explore the course of epilepsy following relapse after antiepileptic drug (AED) withdrawal. METHODS: Forty two patients were identified with onset of epilepsy in childhood in whom AEDs had been withdrawn after at least 2 years of seizure freedom, and in whom a relapse had occurred. Two patients were lost to follow up. RESULTS: Median follow up after AED withdrawal was 5.9 years (range 1.6-13.2 years). Relapse occurred in more than half of the patients within 6 months of AED withdrawal. At the end of follow up, 12 patients (30%) were seizure free for at least 1 year (mean 10.4 years) without medication; 16 (40%) were seizure free for at least 1 year (mean 5.3 years) with ongoing medication; and 12 patients (30%) were seizure free for less than 1 year with medication. No status epilepticus occurred in any patient after withdrawal. Age at onset, if over the age of 5, combined with normal intelligence were predictive of an excellent outcome; presence of a neurological disorder, and hence symptomatic aetiology, was predictive of poor outcome after a relapse. CONCLUSIONS: Fears that premature withdrawal of AEDs might result in uncontrollable seizures were unsubstantiated in this study. The current practice of withdrawing AEDs in children who have been seizure free for 2 years can be beneficial to most of these patients.     

Body part asymmetry in partial seizure

Clinically differentiating between localisation related and generalised epilepsy is important because it carries significant implications for planning diagnostic management strategy. Asymmetry of body parts such as toes, popliteal crease levels, thumbs, cubital crease levels, and forehead and facial structures, are common in patients with localisation related epilepsy syndromes. We retrospectively studied 337 patients with seizure disorders. Body part asymmetry was routinely documented. Fifty-six were excluded because of non-epileptic seizures, pure psychiatric disorders, non-epileptic neurological disorders, brain tumours and strokes. The relationship between clinically detectable body asymmetry (BA) and the electro-anatomic characteristics of their epilepsy was explored. Body asymmetry was found in 88 out of 282 cases, in which 64 (73.5%) suffered from localisation related epilepsy. Among localisation related epilepsy, BA were found in 41.5% (n=64/154) of patients. In contrast, only 18.75% (n=24/128) of patients with generalised seizure disorders showed similar findings (P<0.0001). Among patients with partial onset seizures, lateralisation of BA was concordant with their seizure origin in 75.9% (n=41/54) and discordant in 24.1% (n=13/54). Investigation results of 10 partial epilepsy cases were non-lateralising at the time of study. Peak age of onset of concordant case was 0-5 years old while discordant group was 6-15 years old. We conclude that BA in patients with seizure disorder is a useful clue to diagnosis of localisation related seizure and may provide clues for lateralising seizure origin in partial onset seizures.     

Outcome after prolonged convulsive seizures in 186 children: low morbidity, no mortality

Prolonged convulsive seizures are a common neurological emergency and a potential cause of neuronal damage and functional sequelae. We explored the role of seizure duration and various background factors for neurological sequelae in children with prolonged convulsive seizures. The population-base of this study was all children (age < 16 years) who had been admitted to the Tampere University Hospital, Finland between 1993 and 1999 with convulsive seizures lasting more than 5 minutes. Patients were followed up individually (mean length of follow-up 2 years 1 month, range 0 to 7 years 8 months). All available data on the prolonged seizure episodes and clinical follow-up were analyzed retrospectively by a detailed review of all medical charts and records. In 186 children (94 males, 92 females; mean age 4 years 5 months, SD 3 years 10 months, range 1 month to 15 years 4 months) there were 279 separate convulsive seizure episodes lasting over 5 minutes, yielding an annual incidence of 47.5 out of every 100000 episodes. Seizure aetiology was idiopathic in 26.2% of episodes, febrile in 41.9%, remote symptomatic in 28%, and acute symptomatic in 3.9% of episodes. Mean duration of all seizure episodes was 42.5 minutes (SD 46.1 minutes) and was significantly correlated with the aetiology: shortest in the febrile group (mean 35.4 minutes) and longest in the acute symptomatic group (mean 88.6 minutes; p < 0.001). There was no mortality related directly to these acute seizure episodes. The most common sequela was an onset of epilepsy in 40 children (22%). Permanent neurological sequelae were noted in only four patients (2.2%; mean seizure duration 16 minutes) and non-permanent sequelae in six patients (3.2%; mean seizure duration 38 minutes). Neurological sequelae of prolonged convulsive seizures in children are rare and are related to aetiological factors rather than the duration of a single seizure. The role of acute seizures in the evolution of epilepsy in children remains obscure.     

Epilepsy and antiepileptic drug therapy in juvenile neuronal ceroid lipofuscinosis

PURPOSE: To survey the characteristics of epilepsy in patients with juvenile neuronal ceroid lipofuscinosis (JNCL) and determine the antiepileptic drug (AED) treatment most suitable for these patients. METHODS: The study included 60 patients with JNCL; their mean age was 16.5 years (range 5-33). The age at onset of epilepsy, type of seizures, effect of the first AED on seizures, and the current seizure frequency and AED therapy were studied. The side effects of the AEDs were also clarified. RESULTS: Fifty of the 60 patients had epilepsy. Patients' first epileptic seizure occurred at a mean age of 10.0 years (range 5-16), the most common type being generalized seizures. As the first AED tried, valproate (VPA) and lamotrigine (LTG) appeared equally effective, with 80% of the patients responding to these AEDs. During the study year, the median seizure frequency was four seizures a year (range 0-120), and 72% of the patients had good or satisfactory seizure control (0-6 seizures a year). In the different AED therapy groups, the proportion of patients with good or satisfactory seizure control ranged from 25% to 100%. LTG in monotherapy or in combination with clonazepam (CZP) was superior to other AEDs or combinations, but VPA also seemed effective. Adverse effects leading to the discontinuation of an AED were observed in 25% of the patients, most frequently in patients receiving phenobarbital (PB). No patient receiving LTG had to discontinue the drug due to adverse effects. CONCLUSION: Epilepsy in JNCL can usually be successfully treated with the current AEDs. In Finnish patients with JNCL, treatment is based on LTG, or, secondarily, VPA. In combination therapy, CZP seems a valuable add-on AED.     

Epilepsy in fragile X syndrome

Epilepsy is reported to occur in 10 to 20% of individuals with fragile X syndrome (FXS). A frequent seizure/EEG pattern in FXS appears to resemble that of benign focal epilepsy of childhood (BFEC, benign rolandic epilepsy). To evaluate seizure frequency and type in a Chicago FXS cohort, data regarding potential seizure history were reviewed for 136 individuals with FXS (age range 2 to 51 years: 113 males and 23 females). Seizures occurred in 15 males (13.3%) and one female (4.8%): of these, 12 had partial seizures. EEG findings were available for 35 individuals (13 of 16 with seizures and 22 of 120 without seizures) and showed an epileptiform abnormality in 10 (77%) individuals with seizures and five (23%) individuals without seizures--the most common epileptiform pattern being centrotemporal spikes. Seizures were easily controlled in 14 of the 16 individuals with seizures. Many individuals, including all with centrotemporal spikes, had remission of seizures in childhood. The most common seizure syndrome resembled BFEC and this pattern had the best prognosis for epilepsy remission. Deficiency of FMRP (fragile X mental retardation protein) appears to lead to increased neuronal excitability and susceptibility to epilepsy, but particularly seems to facilitate mechanisms leading to the BFEC pattern.     

Clinical genetic studies in benign childhood epilepsy with centrotemporal spikes

Purpose: To accurately determine the frequency and nature of the family history of seizures in patients with benign childhood epilepsy with centrotemporal spikes (BECTS). Method: Participants with BECTS were recruited from the electroencephalography (EEG) laboratories of three pediatric centers and by referral. Pedigrees were constructed for up to three degrees of relatedness for each proband. All available affected and unaffected individuals underwent phenotyping using a validated seizure questionnaire. The proportion of affected relatives according to degree of relatedness was calculated and phenotypic patterns were analyzed. Key Findings: Fifty‐three probands with BECTS had a mean age of seizure onset at 7.8 years (range 2–12 years). Thirty‐four (64%) of 53 patients were male. For 51 participants, pedigrees were available for three degrees of relatedness. Fifty‐seven (2.7%) of 2,085 relatives had a history of seizures: Twenty‐one (9.8%) of 214 first‐degree, 15 (3%) of 494 second‐degree, and 21 (1.5%) of 1,377 third‐degree relatives. Febrile seizures were the most frequent phenotype, occurring in 26 of 57 affected relatives. There were 34 relatives with epilepsy: 6.5% (14 of 214) first‐degree, 1.8% (9 of 494) second‐degree, and 0.8% (11 of 1,377) third‐degree relatives. Of 21 affected first‐degree relatives: 8 of 21 had febrile seizures (FS), 4 had BECTS, 2 had epilepsy‐aphasia spectrum disorder, one had temporal lobe epilepsy with hippocampal sclerosis, 2 had focal epilepsy of unknown cause, 2 had genetic generalized epilepsies, and 3 had miscellaneous. Significance: The frequency of epilepsies in relatives and the heterogeneous syndromes observed suggest that BECTS has a genetic component consistent with complex inheritance. Focal epilepsies are the most common seizure disorder observed in relatives, especially BECTS and epilepsy‐aphasia spectrum disorder. Additional acquired or environmental factors are likely to be necessary for expression of the seizure disorder.     

Seizure recurrence after reduction of an antiepileptic drug in patients with unprovoked seizures and severe neurological abnormalities

A prospective study of antiepileptic drug (AED) reduction in patients with unprovoked seizures and severe neurological abnormalities after a seizure-free period of more than 5 years was performed. From a hospital for severely handicapped children (150 patients) and an institution for mentally handicapped people (89 persons), 13 patients were enrolled to this study after informed consent was obtained. All patients had experienced a seizure-free period of more than 7 years (median, 10 years). The patients had IQs of less than 50 and were almost dependent in their life. Five patients had additional motor deficits. The patients had been taking one to three AED (mean, 1.9) before reduction and only one AED was withdrawn. During the following 2years, four of the 13 patients (31%) showed a recurrence of seizures. The age at the time of the last seizure was lower in the seizure-free patients. As to the 10 patients with onset ages of 10 or less, a significant factor as to seizure recurrence was whether or not seizures were controlled before the age of 11 years (P < 0.05, Fisher's exact probability test). It is suggested that a patient with severe neurological abnormalities, in whom epilepsy or unprovoked seizures are controlled before the age of 11 years (i.e. before adolescence) could be a candidate for the reduction of AED.     

Recurrent seizures and behavior problems in children with first recognized seizures: a prospective study

PURPOSE: Children with epilepsy have high rates of behavior problems. The purpose was to describe prospectively the association of seizures and behavior problems in children with new-onset seizures. METHODS: Subjects were 224 children with new-onset seizures (aged 4-14 years) and 159 siblings (4-18 years). Caregiver's ratings of the behavior were collected 4 times: at baseline, and at 6, 12, and 24 months. During the 2-year period, 163 (73%) children had at least one additional seizure, and 61 (27%) had none. Data were analyzed by using repeated measures analysis of variance both with and without covariates [site, age, gender, race, caregiver education (years), and seizure medications]. RESULTS: On average, children had higher CBCL Total and Internalizing Behavior Problems scores across all times when experiencing recurrent seizures than when not experiencing recurrent seizures (Total Problems, p = 0.041, controlling for demographics and seizure medications). Siblings had significantly lower Total and Internalizing Problems scores than both children experiencing (Total Problems adjusting for covariates, p = 0.0001) and not experiencing recurrent seizures (p = 0.0004). Externalizing Problems scores were not significantly different among children with recurring seizures, children without recurring seizures, and siblings. CONCLUSIONS: Recurrent seizures significantly predicted behavior problems very early in the course of a seizure condition, even when key child, demographic, and seizure variables were controlled. Explanations for these findings include the possibilities that both seizures and behavior problems are caused by an underlying neurological disorder, that seizures per se disrupt behavior, or that children have negative psychological responses to seizure activity.     

Fieberkrämpfe

Febrile seizures are the most common seizure type in man and may be classified as simple or complex. The overall recurrence rate is 30–35%. Predictors of recurrence include a family history of febrile seizures, age at onset of less than 18 months, and a relatively low body temperature at the time of the seizure. The risk of subsequent epilepsy is only slightly elevated in children with simple febrile seizures. Factors that increase epilepsy risk are complex febrile seizures, abnormal neurological development, and a family history of epilepsy. While older studies did not report increased mortality in children with frebrile seizures, a recent large population-based study found increased mortality during the first 2 years after a febrile seizure in children with complex febrile seizures, seizure onset in the first year of life as well as seizures triggered by a body temperature of less than 39°C. Semiology and prognosis of febrile seizures are heterogeneous. Simple febrile seizures have a good prognosis with diagnostic and therapeutic procedures being uniform at the international level. Children with complex febrile seizures have a poorer prognosis, concerning both risk of epilepsy and mortality within the first 2 years after the first febrile seizure. In these children, decisions concerning diagnostic work-up and medical management are based on the risk profile of the individual patient.