Positive Family History Of Seizures Research Articles

Electroencephalography, Magnetic Resonance Imaging and Response to Treatment in Children with Focal Seizures A Prospective Cohort Study.

This study aimed to evaluate patients with focal seizures, their clinical manifestations, and their response to treatment, then compared the accuracy of electroencephalography and Magnetic Resonance Imaging (MRI) to assess the cost-effectiveness of the latter. After selecting the appropriate individuals, the authors obtained the data by clinically evaluating the cases and interviewing them or their caretaker(s) on admission and the six-month follow-up visit. The researchers then analyzed the obtained data. Most cases (88.4%) had idiopathic seizures. A positive family history of seizures was observed in eight cases (5.4%). Respectively, the occipital, frontal, and temporal lobes showed the highest frequency of abnormalities on electroencephalography, while periventricular leukomalacia was the highest abnormal MRI finding (4.1%). However, in 87.8% of cases, this modality's results were normal. No recurrence of seizures was observed in 116 cases (78.9%) on the six-month follow-up visit, pointing towards an appropriate response to treatment. While this study revealed that most had normal MRI, reporting an abnormality in electroencephalography was a more prevalent occurrence. This finding undermines the cost-effectiveness of the former modality, even though its importance in diagnosing the underlying abnormalities necessitates further studies regarding the subject at hand. Furthermore, the study of age groups showed that treatment response is less desirable in children under two years of age, suggesting more intense workups.

Psychogenic nonepileptic (functional) seizures: Significance of family history and model learning

PurposeIf a positive family history of seizures plays a significant role that contributes to the risk for developing psychogenic nonepileptic seizures (PNES) by means of model learning, one would expect that patients with PNES with a family history of seizures show a different semiology than those without such a history. We investigated whether the above hypothesis is valid. MethodsIn this retrospective study, all patients with PNES, who were diagnosed at Shiraz Comprehensive Epilepsy Center at Shiraz University of Medical Sciences, Iran, from 2008 until 2019, were investigated. Demographic and clinical characteristics were compared between patients with a positive family history of seizures and those without such a history ResultsDuring the study period, 274 patients with PNES-only had the inclusion criteria. Seventy-seven (28%) patients had a positive family history of seizures and 197 (72%) patients did not have such a history. There were no significant demographic or clinical differences between the two groups. ConclusionIt seems that a positive family history of seizures and model learning does not play a significant role in the development of PNES. Investigators should explore other potentially significant contributors and risk factors for developing PNES in future studies.

Socio-demographic risk factors associated with febrile seizures at Puducherry: a retrospective study

Background: Febrile seizures occur commonly in the under 5 age group and is associated with few risk factors causing its recurrence like very high fever, family history of seizures, low sodium levels and younger age of onset which are subject to seasonal and wide geographical variations. This study aimed at detecting the major risk factors associated with recurrent febrile seizures in an Indian population.Methods: A retrospective hospital-based study was conducted among a total of 300 cases aged 6 months to 5 years attending to the paediatric OPD with history of fever followed by febrile seizures. Information regarding socio-demographic and clinical variables associated with febrile seizure was collected and analyzed.Results: The mean age of the study participants was 25.6±2.2 months and majority (60%) were males. Family history of seizures was present in 25.3% (n=76) of the children with febrile seizures. Respiratory infections (73.3%) and gastroenteritis (17%) were the major infective reasons associated with the occurrence of febrile seizures followed by pneumonia (6.3%) and urinary tract infections (5%). Recurrence of FS was significantly higher among the children with family history of FS (p=0.009), age at onset lesser (p<0.001) and simple FS seizures.Conclusions: Younger age at onset and positive family history of seizures were important socio-demographic risk factors associated with recurrent febrile seizures.

Predictors of outcome after surgery in 134 children with drug-resistant TLE

PurposeTo determine the factors predicting seizure outcome in children who underwent anterior temporal lobectomy (ATL) for drug-resistant temporal lobe epilepsy (TLE). MethodsAmong the 664 patients who underwent ATL for TLE from 1995 to 2008, there were 134 children. “Excellent” outcome in them was defined as seizure freedom during the entire follow-up (Engel class I a); “good outcome”, if in remission last 2 years, and the rest as “unfavorable outcome”. To identify the potential predictors of seizure recurrence, the attributes of recurred and non-recurred groups was compared by univariate and multivariate analysis. ResultsOf the 134 children, at a mean post-operative follow-up of 8.1 years, there were 82 (61.1%) with excellent outcome and 26 (19.4%) with good outcome (category-1; seizure-free, 80.6%). Drugs could be successfully withdrawn in 69 (63.9%). 26 patients (22.4%) had seizure recurrence, (category-2, treatment/surgical failure).Whereas, of the 530 adults who underwent ATL during this time period, only 46.8% never had seizures (category-1) and 53.2% had seizure recurrence in the form of acute post-operative seizures or auras or habitual or non-habitual seizures (category-2).The hazard of seizure recurrence in children increased with positive family history of seizures and /or epilepsy, normal neuroimaging, spikes in post-operative EEG at 3-months, normal histopathology and duration of epilepsy ≥5 years. ConclusionsThe information on the predictive factors causing seizure recurrence/freedom in children with drug-resistant TLE is important in surgical selection. Surgery for pediatric TLE results in favorable outcome, hence one should subject children for resective surgery at the earliest especially in lesions known to cause refractoriness.

Adaptive functioning in pediatric epilepsy: Contributions of seizure-related variables and parental anxiety

Young people with epilepsy are less likely to achieve the level of independence attained by their peers. We examined the seizure-related variables that placed a group of 97 pediatric patients with intractable seizures at risk for poor adaptive functioning. Analyses evaluated both the direct effects of the medical variables and indirect effects that were mediated through increased parental anxiety about their child's epilepsy. Higher numbers of anticonvulsants, presence of seizures that secondarily generalize, longer duration of seizure disorder, and younger age at onset were all identified as risk factors for poor adaptive functioning. Depending on the specific behavioral domain of adaptive functioning, the effects were sometimes direct and sometimes indirect. Lower levels of parental education and positive family history of seizures were associated with higher levels of parental anxiety. Interventions that target parental anxiety about seizures may mitigate the deleterious effects of epilepsy on social development.

Clinical genetic study of the epilepsy‐aphasia spectrum

To characterize the frequency and nature of the family history of seizures in probands with epilepsy falling within the epilepsy-aphasia spectrum (EAS) in order to understand the genetic architecture of this group of disorders. Patients with epileptic encephalopathy with continuous spike-and-wave during sleep (ECSWS), Landau-Kleffner syndrome (LKS), atypical benign partial epilepsy (ABPE), and intermediate epilepsy-aphasia disorders (IEAD) were recruited. All affected and available unaffected relatives up to three degrees of relatedness underwent phenotyping using a validated seizure questionnaire. Pedigrees were constructed for all families. The proportion of affected relatives according to each degree of relatedness was calculated. The epilepsy phenotypes in close relatives were analyzed. The data were compared to the families of probands with benign childhood epilepsy with centrotemporal spikes (BECTS) using the same methodology. Thirty-one probands, including five ECSWS, three LKS, one ABPE, and 22 IEAD were recruited. The mean age of seizure onset was 3.9 (range 0.5-7) years. A male predominance was seen (68%, 21/31) . Sixteen (51.6%) of 31 had a positive family history of seizures. Among 1,254 relatives, 30 (2.4%) had a history of seizures: 13 (10.2%) of 128 first-degree relatives, 5 (1.7%) of 291 second-degree relatives, and 12 (1.4%) of 835 third-degree relatives. Thirteen had febrile seizures, including two who had both febrile seizures and epilepsy. Of the 19 relatives with epilepsy, 4 had BECTS, 4 epilepsies with focal seizures of unknown cause, 3 IEAD, and 7 unclassified. One had genetic generalized epilepsy. In the families of the BECTS probands, 9.8% of first-degree, 3% of second-degree, and 1.5% of third-degree relatives had seizures, which was not significantly different from the EAS cohort families. The frequencies of seizures in relatives of probands with EAS suggest that the underlying genetic influence of EAS is consistent with complex inheritance and similar to BECTS. The phenotypic pattern observed in the affected relatives comprised predominantly febrile seizures and focal seizures. These findings suggest that a shared genetic predisposition to focal epilepsies underpins the epilepsy-aphasia spectrum.

Genetic seizure susceptibility underlying acute encephalopathies in childhood

We herein investigated risk factors of pediatric acute encephalopathy (AE) regarding the hitherto uncharacterized genetic background of seizure susceptibility underlying the pathogenesis of AE. The study included 15 patients with a history of various types of AE in childhood. We undertook the mutational analysis of the neuronal sodium channel alpha 1 subunit (SCN1A) gene which is the most representative gene for hyperthermia-induced seizure susceptibility. Six patients (40%) had a positive family history of seizures or AE, especially febrile seizures, in first- or second-degree relatives. The SCN1A-R1575C mutation was detected in a patient with a history of acute encephalitis with refractory, repetitive partial seizures (AERRPS) and also in the patient's apparently healthy father. In the present study, dense familial seizure predisposition was present in the patients with AE. Although the presence of seizure susceptibility alone is insufficient to cause AE, it can exacerbate seizures and the subsequent development of inflammatory reactions in the brain when environmental factors are included. Genetic seizure susceptibility may contribute to some types of AE in childhood.

Juvenile myoclonic epilepsy: disease expression among Indian families.

Juvenile myoclonic epilepsy (JME) is a common, age related, idiopathic generalized epileptic syndrome. We aimed to define the expression of JME in Indian probands and study the occurrence of seizures/epileptic syndromes in their family members. We studied 225 JME probands with a uniform protocol. recording the type and frequency of seizures, precipitating factors, EEG data, and family history. Detailed family pedigrees were drawn to include all the 1st- and 2nd-degree relatives of probands. The seizures/epileptic syndromes in relatives examined were classified in a uniform way. The clinical and EEG characteristics of 225 JME probands from India were similar to those reported in probands from different ethnic backgrounds. The incidence of febrile convulsions in probands with JME was similar to that of the general population but was much lower (0.2%) among their relatives. A positive family history of seizures among 1st- or 2nd-degree relatives was noted in 79 of 225 (35%) probands. The risk of relatives being affected as well as their risk of expressing a type of idiopathic generalized epilepsy (IGE) varied as a function of the degree of relation with the probands. The clinical expression of JME among probands from India is fairly similar to that reported in probands of different ethnic backgrounds. The risk of relatives being affected as well as their risk of expressing a type of IGE (including JME) varies as a function of the degree of relation with the probands. The reduced occurrence of febrile convulsions among the relatives of JME probands probably represents an ascertainment bias. A much larger database of this type should be helpful in understanding the interactions of different genes that are believed to be responsible for some of the inherited human epileptic syndromes like JME and other IGEs.

Increased number of febrile seizures in children born very preterm: Relation of neonatal, febrile and epileptic seizures and neurological dysfunction to seizure outcome at 16 years of age

In prematurely born population, a cascade of events from initial injury in the developing brain to morbidity may be followed. The aim of our study was to assess seizures in prematurely born children from birth up to 16 years and to evaluate the contribution of different seizures, and of neurological dysfunction to the seizure outcome. Pre- and neonatal data and data from neurodevelopmental examination at 5 years of 60 prospectively followed children born at or before 32 weeks of gestation, and of 60 matched term controls from the 2 year birth cohort were available from earlier phases of the study. Later seizure data were obtained from questionnaires at 5, 9, and 16 years, and from hospital records and parent interviews. In the preterm group, 16 children (27%) exhibited neonatal seizures, 10 children (17%) had seizures during febrile illness and 5 children had epilepsy. Eight children had only febrile seizures, and 3 of these had both multiple simple and complex febrile seizures and neurodevelopmental dysfunction. None of the 8 children had experienced neonatal seizures, 6 had a positive family history of seizures, but none developed epilepsy. The children with epilepsy had CP and neurocognitive problems, and all but one had experienced neonatal seizures; two of them had also had fever-induced epileptic seizures. In controls 3 children (5%) had simple febrile seizures. Children born very preterm have increased rate of febrile seizures compared to the controls. However, no cascade from initial injury via febrile seizures to epilepsy could be shown during the follow-up of 16 years. Symptomatic epilepsy in prematurely born children is characterised by neonatal seizures, major neurological disabilities and early onset of epilepsy.

Familial Temporal Lobe Epilepsy with Auditory Features

tial seizures with auditory features, which were described as a radio or a motorcycle running noise or “as if there were people talking on TV something she could not understand.” These symptoms could be followed or accompanied by distorted vision: “people’s face became disfigured.” She has good seizure control with carbamazepine. Her routine electroencephalogram (EEG) was normal. Magnetic resonance imaging (MRI) showed enlargement of left lateral temporal lobe and absence of first and second temporal sulci, and abnormal shape and axis of hippocampus (Fig. 2).

Padrão eletrográfico ictal subclínico em um caso de epilepsia parcial benigna da infância com pontas centro-temporais

There are few reports of the ictal pattern of benign partial epilepsy with centrotemporal Spikes (BECTS) in the literature. This paper describes the case of a 7-year-old boy without significant perinatal history or psychomotor development delay. There was positive family history of seizures. Magnetic resonance imaging scans of the head was normal. The patient had a nocturnal isolated seizure, followed by a postictal motor deficit at the left arm. The EEG two days after the seizure showed a normal background activity with spikes in the centrotemporal bilateral area, and two subclinicals rhythmic discharges of spike and wave, with a duration of more than 50 seconds in the right centrotemporal area. This pattern of rhythmic discharge wasn not described in BECTS before.

Febrile convulsions with focal sharp waves: A subgroup of benign partial epilepsies of childhood with multifactorial etiology

Studies on the prognosis of febrile convulsions (FC) have scarcely considered the heterogeneity of FC and its relationship to the risk and the variable nature of subsequent afebrile seizures. Meaningful study of this issue requires a sample of FC children that is as homogeneous as possible. One such FC subgroup is children with focal sharp waves (shw) in the electroencephalogram (EEG) of a type characteristic of benign partial epilepsies (5%–10% of all FC children). Because the incidence of FC is elevated in children with benign partial epilepsy, the question arises whether these entities have certain pathogenetic mechanisms in common. Because only children with long-term follow-up are suitable for a study of these problems, the results obtained here allow no quantitative inferences regarding prognosis. Inclusion criteria were: 1) FC as the first seizure symptom; 2) focal shw characteristic of benign partial epilepsy; 3) first EEG before the fifth year of life; 4) at least four EEGs between the second and the eighth year of life. One-hundred and twenty-eight children were ascertained. Duration of follow-up ranged from 1–33 years. A positive family history of seizures was found in 34% of probands. The incidence of neonatal seizures was remarkably elevated (6%). Prolonged FC and FC with focal symptoms were significantly overrepresented. Long lasting FC (>60 minutes) were associated with an occipital location of the shw. Fifty-nine children suffered subsequent afebrile seizures or epilepsy (selected material): idiophathic partial epilepsy (58%); idiopathic generalized epilepsy (12%); severe epilepsies (22%), usually with intractable complex partial seizures. The latter were clearly associated with FC lasting longer than 1 hour. In 91% of the patients generalized genetic EEG patterns like generalized spikes and waves, photoparoxysmal response, and theta rhythms were eventually detected. Among the shw findings, precentral (70%) and occipital (50%) foci predominated. Of 69 investigated siblings focal shw were detected in 14%. Febrile convulsions with focal shw represent a multifactorial disorder. The combined action of five factors seems relevant: 1) a hereditary impairment of brain maturation (represented by the shw-EEG trait); 2) a genetic anomaly expressed by photoparoxysmal response; 3) a genetic cortical hypersynchronization (theta rhythms); 4) the generalized spike wave abnormality; and 5) a genetic predisposition to FC that is represented by a high incidence of FC in relatives but is not expressed specifically in the EEG. Subsequent epilepsies are idiopathic (mostly partial) or the consequence of FC lasting more than 1 hour.

Index of Suspicion

Index of Suspicion

Human leukocyte antigen studies in Indian probands with seizures associated with single small enhancing computed tomography lesions and seizure types in their family members

We report the preliminary results of human leukocyte antigen (HLA) class I typing in 63 Indian probands with the syndrome of seizures associated with single, small, enhancing computed tomography (CT) lesions. (SSEL) and the occurrence of seizure types among their family members. Sixteen of the 63 (25%) probands had a positive family history of seizures among the first- and second-degree relatives. There were 9 sibs, 3 parents, and 12 second-degree relatives among the 24 affected family members. The majority of affected relatives (21 of 24) were examined; epilepsy with generalized tonic-clonk seizures (EGTCs) was the commonest seizure type in 10 (42%), and febrile convulsions (FC), SSEL, epilepsy with secondarily generalized seizures (SGE), and single seizures were noted in 2 (8%), 4 (17%), 3 (13%), and 2 (8%) relatives, respectively. The frequency of HLA-A11 was decreased ( p p p p -value was applied. Our study suggests that many cases with the syndrome of SSEL exist in a subset of an Indian population that may be genetically predisposed to seizures. It is likely that some of these Indian patients with SSEL have an inherited predilection for seizures and are also genetically susceptible to some infection or infestation.

Disease expression among probands with juvenile myoclonic epilepsy and their family members in two population groups

We report clinical and EEG features of 65 probands with juvenile myoclonic epilepsy (JME) and the occurrence and character of epilepsy among 664 blood relatives of 26 of the 65 probands with a positive family history of seizures. Forty-six families from India and 19 families from the United States were investigated. Among the probands, sex distribution was equal; the mean age at onset of generalized tonic-clonic seizures (GTCS) and myoclonic jerks was 14.2 and 14.3 years, respectively; myoclonic jerks alone occurred in 2 (3%); 5 (8%) had absence seizures; and bilaterally symmetrical polyspike and wave complexes were evident during interictal EEG in 50 (77%). Twenty-six probands (40%) had blood relatives with seizures. Clinical and EEG features were similar in Indian and American probands and in probands with and without a family history of seizures. Sixty-two blood relatives had seizures: 38 (61%) had idiopathic generalized epilepsy (IGE), including 11 (18%) with JME; 9 (15%) had single, spontaneous generalized seizures before age 30 years; and the remainder had febrile convulsions (4, 6%) or symptomatic, partial or unclassified epilepsy (11, 18%). Both the proportion of family members observed to have epilepsy and the epilepsy syndrome expressed in family members varied as a function of the degree of relation. First-degree relatives had greater occurrence of epilepsy, IGE, and JME than did more distant blood relatives. These data suggest that JME is a complex genetic disorder and support a multigenic pattern of inheritance.

Prevalence and pattern of epilepsy (Lath/Mirgi/Laran) in rural Kashmir, India.

The rural population of 63,645 living in the mountainous Kuthar Valley of South Kashmir, Northwest India was surveyed to determine the prevalence of major neurologic disorders, including epilepsy (called Lath/Mirgi/Laran in the local language). The survey was done according to a World Health Organization protocol (1981). House-to-house screening was done by Anganwadi workers to identify people with possible epilepsy. The screening questionnaire was translated into local vernacular. Persons who had some indication of a history of seizures or other neurologic disease were subsequently examined by a neurologic team. The diagnostic criteria of Hauser and Kurland (1975) were used to define cases of active epilepsy and seizure classification (ILAE, 1981) was done only with clinical data. One hundred fifty-seven cases of active epilepsy were detected, giving a crude prevalence rate of 2.47/1,000 general population. In those aged less than 14 years, prevalence was 3.18/1,000. Ninety-five (60.5%) of all cases were male; 91% of active epilepsy cases had onset of seizures before age 30 years. Mean age of onset in males was 5.3 years, and in females it was 7.1 years. Mean duration of seizures was 6 years; 78.9% cases had generalized seizures, 74.5% cases were receiving no specific treatment, 99.4% cases were born of home delivery, and 8.9% cases had a positive family history of seizures. Mental retardation was the most common associated abnormality in 22.9% of cases.

EEG Abnormalities in Nonepileptic Patients

A total of 202 nonepileptic patients (120 males and 82 females) who exhibited spike abnormalities at least twice in their EEG examinations were studied. The incidence of spike abnormalities among nonepileptic patients was 8.1% (847/10,473). The majority (90%) were under age 19. Headache, dizziness and vomiting, and abdominal pain were more frequently observed compared with controls. Mild paroxysmal EEG abnormalities such as diffuse irregular slow wave bursts with spike (27%), positive spikes (25%) or small spike (8.4%) were commonly detected. In three patients who developed epileptic seizures during the follow-up period, more specific EEG abnormalities were often exhibited. Other factors like the age at onset before 9, characteristic clinical symptoms or a positive family history of seizures were confirmed to be necessary for the manifestation of clinical seizures.