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Febrile seizures affect 2%-5% of U.S. children and are considered benign although associated with an increased risk of epilepsy and, rarely, with sudden unexplained death. We compared rates of mortality, neurodevelopmental disorders, and neuropathology in young children with simple and complex febrile seizures to healthy controls. We systematically reviewed studies of 3- to 72-month-old children with simple or complex febrile seizures ≤30 min. We searched studies with outcome measures on mortality, neurodevelopment, or neuropathology through July 18, 2022. Bias risk was assessed per study design. Each outcome measure was stratified by study design. PROSPERO registration is CRD42022361645. Twenty-six studies met criteria reporting mortality (11), neurodevelopment (11), and neuropathology (13), including 2665 children with febrile seizures and 1206 seizure-free controls. Study designs varied: 15 cohort, 2 cross-sectional, 3 case-control, 5 series, and 1 case report. Mortality outcomes showed stark contrasts. Six cohort studies following children after febrile seizure (n = 1348) reported no deaths, whereas four child death series and 1 case report identified 24.1% (108/449) deaths associated with simple (n = 104) and complex (n = 3) febrile seizures ≤30 min. Minor hippocampal histopathological anomalies were common in sudden deaths with or without febrile seizure history. Most electroencephalography (EEG) studies were normal. Neuroimaging studies suggested increased right hippocampal volumes. When present, neurodevelopmental problems usually preexisted febrile-seizure onset. Risk bias was medium or high in 95% (18/19) of cohort and case-control studies vs medium to low across remaining study designs. Research on outcomes after simple or brief complex febrile seizures is limited. Cohort studies suffered from inadequate sample size, bias risk, and limited follow-up durations to make valid conclusions on mortality, neurodevelopment, and neuropathology. Sudden death registries, focused on a very small percentage of all cases, strongly suggest that simple febrile seizures are associated with increased mortality. Although most children with febrile seizures have favorable outcomes, longer-term prospective studies are needed., (© 2023 The Authors. Epilepsia published by Wiley Periodicals LLC on behalf of International League Against Epilepsy.)

Objective: Family members carrying the same SCN1A variant often exhibit differences in the clinical severity of epilepsy. This variable expressivity suggests that other factors aside from the primary sodium channel variant influence the clinical manifestation. However, identifying such factors has proven challenging in humans., Methods: We perform whole exome sequencing (WES) in a large family in which an SCN1A variant (p.K1372E) is segregating that is associated with a broad spectrum of phenotypes ranging from lack of epilepsy, to febrile seizures and absence seizures, to Dravet syndrome. We assessed the hypothesis that the severity of the SCN1A-related phenotype was affected by alternate alleles at a modifier locus (or loci)., Results: One of our top candidates identified by WES was a second variant in the SCN1A gene (p.L375S) that was shared exclusively by unaffected carriers of the K1372E allele. To test the hypothesized that L375S variant nullifies the loss-of-function effect of K1372E, we transiently expressed Nav1.1 carrying the two variants in HEK293T cells and compared their biophysical properties with the wild-type (WT) variant, and then co-expressed WT with K1372E or L375S with K1372E in equal quantity and tested the functional consequence. The data demonstrated that co-expression of the L375S and K1372E alleles reversed the loss-of-function property brought by the K1372E variant, whereas WT-K1372E co-expression remained partial loss-of-function., Significance: These results support the hypothesis that L375S counteracts the loss-of-function effect of K1372E such that individuals carrying both alleles in trans do not present epilepsy-related symptoms. We demonstrate that monogenic epilepsies with wide expressivity can be modified by additional variants in the disease gene, providing a novel framework for the gene-phenotype relationship in genetic epilepsies., (© 2022 International League Against Epilepsy.)

Objective: Febrile seizures (FSs) are the most common form of seizures in children. Single short FSs are benign, but FSs lasting longer than 30 min, termed febrile status epilepticus, may result in neurological sequelae. However, there is little information about an intermediary condition, brief recurrent FSs (RFSs). The goal of this study was to determine the role of RFSs on spatial learning and memory and the properties of spontaneous hippocampal signals., Methods: A hippocampus-dependent active avoidance task was used to assess spatial learning and memory in adult rats that underwent experimental RFSs (eRFSs) in early life compared with their littermate controls. Following completion of the task, we utilized high-density laminar probes to measure spontaneous hippocampal CA1 circuit activity under urethane anesthesia, which allowed for the simultaneous recording of input regions in CA1 associated with both CA3 and entorhinal cortex., Results: RFSs did not result in deficits in the active avoidance spatial test, a hippocampus-dependent test of spatial learning and memory. However, in vivo high-density laminar electrode recordings from eRFS rats had significantly altered power and frequency expression of theta and gamma bandwidths as well as signaling efficacy along the CA1 somatodendritic axis. Thus, although eRFS modified CA1 neuronal input/output dynamics, these alterations were not sufficient to impair active avoidance spatial behavior., Significance: These findings indicate that although eRFSs do not result in spatial cognitive deficits in the active avoidance task, recurrent seizures do alter the brain and result in longstanding changes in the temporal organization of the hippocampus., (© 2021 International League Against Epilepsy.)

Objectives: Although secondary hemophagocytic lymphohistiocytosis (HLH) has been reported in children with critical illness of various etiologies, it has not been reported in patients with febrile infection-related epilepsy syndrome (FIRES). We describe a series of patients with concurrent HLH and FIRES in an effort to establish common pathophysiologic abnormalities., Methods: Five patients with FIRES who were assessed for HLH were identified from a neurocritical care database. All were previously healthy and had extensive diagnostic testing. All had clinical deterioration with multiorgan dysfunction prompting HLH screening 20-29 days after hospitalization. Markers for inflammatory dysregulation were assessed in cerebrospinal fluid (CSF) and serum at various time points. Outcomes were assessed 6 months after presentation., Results: Three patients met clinical criteria for secondary HLH. Elevation of specific cytokines/chemokines was variable. CSF neopterin, high mobility group box 1 (HMGB1), and C-X-C motif chemokine ligand 8 (CXCL8) were significantly elevated in all. Interleukin-1β (IL-1β) and IL-18 were not elevated in any of the samples. Treatment and outcomes were variable., Significance: We describe 3 patients with HLH and FIRES. The co-occurrence of these 2 rare disorders suggests the possibility of a common immune dysregulation phenotype prolonging epileptogenesis. HLH screening in critically ill patients with FIRES may yield a broader understanding of shared inflammatory processes., (Wiley Periodicals, Inc. © 2018 International League Against Epilepsy.)

Purpose: Childhood convulsive status epilepticus (CSE), in particular prolonged febrile seizures (PFS), has been linked with mesial temporal sclerosis (MTS). Previous studies have shown that hippocampal injury occurs in the acute phase immediately following CSE, but little is known about the longer term evolution of such injury. This study aimed to investigate the longer term outcome of childhood CSE with sequential magnetic resonance imaging (MRI) looking for progressive hippocampal injury during the first year post-CSE., Methods: Eighty children (0.18-15.5 years) underwent brain MRI 1 month post-CSE, 50 with a repeat MRI at 6 months and 46 with repeat MRI at 12 months post-CSE. Thirty-one control subjects without neurologic problems had a single brain MRI for comparison. Hippocampal volumes were measured from each MRI scan by two independent observers, and hippocampal growth rates were estimated in each patient with suitable imaging., Key Findings: Hippocampal volume loss was found in 20-30% of patients and was not associated with the etiology or clinical features of CSE, including seizure duration or focality. A borderline association was found between a history of previous seizures (p = 0.063) and the number of previous febrile seizures (p = 0.051), suggesting that multiple insults may be important in the pathogenesis of progressive hippocampal injury., Significance: It is apparent that progressive hippocampal damage can occur after CSE of any etiology and is not limited to PFS. Repeated seizures may play an important role, but further follow-up is needed to determine any other risk factors and proportion of children showing initial volume loss progress to clinical MTS and temporal lobe epilepsy., (Wiley Periodicals, Inc. © 2013 International League Against Epilepsy.)

Objective: Epilepsy following febrile seizures (FS) has been estimated between 2% and 7%. It concerns a prospective study in a large sample of children with a long-term follow-up. The aim of this study is to identify the prognostic factors that can lead children with FS to epilepsy., Methods: Children with a first episode of FS were included. We gathered information about prenatal and perinatal history, family history of FS and epilepsy in first- and second degree relatives, age at the time of the initial FS, dates of FS recurrences, focality, duration of the FS and recurrent episodes within the same febrile illness, height and duration of fever prior to the seizure, cause of the fever, and frequency of febrile illnesses. Patients were seen every 4-6 months and also at each recurrence., Key Findings: A group of 560 children with a first FS met all entry criteria. Epilepsy was recorded at 5.4%. Statistical analysis was performed between children with epilepsy and those with no afebrile seizure. We analyzed FS recurrences in accordance with the occurrence of epilepsy. From the third FS recurrence and beyond, only focality continued to have prognostic value., Significance: Main prognostic factors for the development of epilepsy after FS are: (1) complex FS that increased the risk for epilepsy 3.6 times, (2) age at onset of FS beyond the third year of life that raised the risk 3.8 times, (3) positive family history of epilepsy 7.3 times, and (4) multiple episodes of FS about 10 times. Focality at the first and the second FS recurrence increased the risk of epilepsy about 9.7 and 11.7 times, respectively. Focality was the only factor that continued to be significant in further FS recurrences. A prognostic profile of each child with FS would be very useful for the follow-up of these children., (Wiley Periodicals, Inc. © 2013 International League Against Epilepsy.)

Purpose: Prolonged febrile seizures (PFS) lasting ≥15 min have been associated with increased risk for epilepsy in later life. Initial treatment, mostly prehospital, aims to prevent its evolution to febrile status epilepticus (FSE) and reduce adverse outcome. Paucity of information is available on the immediate treatment before reaching a hospital facility., Methods: We obtained data, prospectively, on all children who presented from January 2008 to March 2010 with PFS to the emergency rooms of four Israeli medical centers. Information related to seizure semiology, treatment, and medical history was collected into a predefined pro forma form and reviewed centrally., Key Findings: Sixty children, median age 18.3 months (interquartile range [IQR] 12-28) were included with a median seizure duration of 35 min (IQR 26-60), 43 (71.7%) lasting ≥30 min. Seizures had focal onset in 34 infants (57%). Fifty-four families (90%) activated the ambulance service; median ambulance arrival time was 8 min (IQR 5-10), 33 (61%) were medically treated by the ambulance paramedic, of whom 15 (45%) responded to treatment. Twelve children with active seizures did not receive medications. Initial treatment with rectal diazepam was more common in those with seizure duration >30 min., Significance: Most children with PFS are treated with antiepileptic drugs early by the ambulance service. However, even timely treatment does not prevent status epilepticus in the majority of cases. These data highlight the need for effective early treatment of this common pediatric emergency., (Wiley Periodicals, Inc. © 2013 International League Against Epilepsy.)

Purpose: 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., Methods: 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., Key Findings: 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., Significance: 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., (Wiley Periodicals, Inc. © 2013 International League Against Epilepsy.)

Purpose: Eyelid myoclonia with absences (EM) is an uncommon type of absence seizure associated with a variety of epilepsy syndromes. The syndrome of epilepsy with EM (EMA) has been proposed to denote the onset of frequent EM induced by eye closure and photic stimulation beginning in childhood. The clinical genetics of EMA has not been well characterized, although a family history of seizures is not infrequent., Methods: Individuals with EMA were ascertained by referral and through the investigators' clinical practices. All available family members were assessed for seizures using a validated seizure questionnaire. Electroclinical data were obtained on each proband and all affected family members; pedigrees were constructed. Families were analyzed for phenotypic patterns., Key Findings: Eighteen individuals with EMA were recruited. A history of seizures was found in 34 relatives in 15 (83%) of 18 families. In terms of epilepsy syndromes, 9 relatives from 7 of 15 families had febrile seizures. Two relatives had EMA. Classical genetic generalized epilepsy (GGE) syndromes were seen in five relatives: two generalized tonic-clonic seizures alone, two childhood absence epilepsy (CAE), and one juvenile myoclonic epilepsy (JME). Genetic epilepsy with febrile seizures plus (GEFS+) phenotypes occurred in 16 relatives. On review of the epilepsy syndromes within each family, seven families had a pattern consistent with GEFS+, whereas three families had classical GGE., Significance: The clinical genetics of EMA is suggestive of complex inheritance with shared genetic determinants overlapping with both classical GGE and GEFS+. The epilepsy syndromes in relatives of probands with EMA differ from those found in families of probands with CAE, supporting the concept that patients with EMA have a syndrome that is distinct from CAE. This presumably reflects different genetic components contributing to their genetic architecture., (Wiley Periodicals, Inc. © 2012 International League Against Epilepsy.)

The factors that contribute to hippocampal damage as a sequela, and its frequency, in patients experiencing febrile status epilepticus, remain unknown. Of the 472 patients with febrile seizures admitted to our hospital between February 2004 and August 2008, 77 had prolonged seizures. Among them, 59 underwent magnetic resonance imaging (MRI). A 21-month-old girl showed hippocampal changes after her first episode of febrile status epilepticus. The seizure lasted about 35 min, with eye deviation to the right and ictal rhythmic discharges in the left hemisphere. MRI at 72 h after the seizure revealed high-signal intensities in T(2) and fluid-attenuated inversion recovery (FLAIR) images of the left hippocampus. Left hippocampal volume diminished over the next several months suggesting the occurrence of neuronal cell death. In no other cases, not even those with longer seizure durations, did significant hippocampal changes develop. The frequency of hippocampal damage was 1.7% in this case series. The involvement of factors other than seizure duration merits further study., (Wiley Periodicals, Inc. © 2011 International League Against Epilepsy.)

Purpose: Neuroimaging studies suggest a history of febrile seizures, and depression, are associated with hippocampal volume reductions in patients with temporal lobe epilepsy (TLE)., Methods: We used radial atrophy mapping (RAM), a three-dimensional (3D) surface modeling tool, to measure hippocampal atrophy in 40 patients with unilateral TLE, with or without a history of febrile seizures and symptoms of depression. Multiple linear regression was used to single out the effects of covariates on local atrophy., Key Findings: Subjects with a history of febrile seizures (n =15) had atrophy in regions corresponding to the CA1 and CA3 subfields of the hippocampus contralateral to seizure focus (CHC) compared to those without a history of febrile seizures (n = 25). Subjects with Beck Depression Inventory II (BDI-II) score ≥ 14 (n = 11) had atrophy in the superoanterior portion of the CHC compared to subjects with BDI-II <14 (n = 29)., Significance: Contralateral hippocampal atrophy in TLE may be related to febrile seizures or depression., (Wiley Periodicals, Inc. © 2011 International League Against Epilepsy.)

SCN1A is the most clinically relevant epilepsy gene and is associated with generalized epilepsy and febrile seizure plus (GEFS+) and Dravet syndrome. We postulated that earlier onset of febrile seizures in the febrile seizure (FS) and febrile seizure plus (FS+) phenotypes may occur in the presence of a SCN1A mutation. This was because of the age-related onset of Dravet syndrome, which typically begins in the first year of life. We found that patients with FS and FS+ with SCN1A mutations had earlier median onset of febrile seizures compared to the population median. Patients with GABRG2 mutations had a similar early onset in contrast to patients with SCN1B mutations where onset was later. This study is the first to demonstrate that a specific genetic abnormality directly influences the FS and FS+ phenotype in terms of age of onset.

Familial febrile seizures occur in both generalized epilepsy with febrile seizures plus (GEFS+) and autosomal dominant febrile seizures (ADFS). The literature largely separates families with GEFS+ from those with ADFS. However, there is clinical overlap, and families with ADFS also include individuals with afebrile seizures. The phenotypic spectrum of GEFS+ is broader now than when first described, resulting in unclear boundaries between these two familial syndromes. The purpose of this report is to highlight the phenotypic similarities of GEFS+ and ADFS. A multigenerational family with febrile and afebrile seizures is described and the clinical features are compared to those of previously reported GEFS+ and ADFS families. This family meets the requirements for both ADFS and the broader definition of GEFS+. Linkage analysis has shown no clear linkage to known febrile seizure or GEFS+ loci. Despite locus heterogeneity, identified mutations in reported GEFS+ have so far all been in sodium channel or gamma-aminobutyric acid (GABA)-receptor genes, with other modifier genes postulated to affect individual phenotypes. The two mutations identified in families with ADFS are in genes implicated in GEFS+, SCN1A, and GABRG2. We conclude that it is inappropriate to separate GEFS+ and ADFS at present given the clinical and genotypic overlap.

Febrile seizures are the most common seizure disorder in childhood, affecting 2-5% of children. Simple febrile seizure is defined as a short (<15 min) generalized seizure, not recurring within 24 h, that occurs during a febrile illness not resulting from an acute disease of the nervous system in a child aged between 6 months and 5 years, with no neurologic deficits and no previous afebrile seizures. These recommendations address the instructions for management of the first febrile seizures, giving criteria for hospital admission, diagnosis, differential diagnosis, and treatment of a prolonged seizure. The authors stressed the benign prognosis of the majority of cases and the risk factors for recurrence of febrile seizures and appearance of epilepsy later on. Both continuous and intermittent anticonvulsant therapy are efficacious in preventing single febrile seizures, but side effects may be so important to overcome the benefits. These treatments are indicated in very selected patients.

Benign familial infantile seizures (BFIS) is a dominant idiopathic epilepsy with partial and secondarily generalized seizures with age of onsetr between 3 and 12 months. Here we describe a four-generation family with some characteristic features of BFIS but with unusual clinical signs, in eight affected members with an unusual clinical phenotype. Onset was consistently between 14 and 20 months of age with clusters of complex-partial or generalized tonic-clonic seizures and a high rate of febrile seizures, which have not been described for BFIS previously. All affected members showed multifocal interictal epileptiform discharges in the EEG. The known loci for benign familial neonatal/infantile seizures (BFNS/BFNIS), generalized epilepsy with febrile seizures plus (GEFS+) and the BFIS locus on chromosome 19q were excluded. Further genetic analysis showed suggestive linkage to the major BFIS locus on chromosome 16 between markers D16S690 and D16S3136. This ;;BFIS-like'' syndrome may enlarge the phenotypic spectrum of diseases linked to the chromosome 16 region.

Purpose: A prospective population-based case-control study was performed to ascertain whether febrile convulsion (FC) in early childhood is associated with neurocognitive attention deficits in school age., Methods: A total of 103 children, confirmed to have FC by age 3 years from a population survey of 4,340 live-birth newborns in Tainan City, Taiwan, was followed up until at least age 6 years. An achievement test, behavioral ratings, and computerized neurocognitive battery assessing various subcomponents of attention were given to 87 FC children (FC group) and 87 randomly selected population-matched control (CC group)., Results: Compared with the CC group, the FC group did not have scholastic performance or behavioral outcome disadvantage. Overall FC group performance was distinguished by significantly higher scores in the achievement test and fewer missing errors (p < 0.005) and commission errors (p < 0. 05), less variability in reaction time (p < 0.005), and a nonsignificant trend of impulsivity. Attention performance of the FC and CC groups were comparable. Within the FC group, age at onset, complex FC, recurrence of FC, development of unprovoked seizures, or prior use of phenobarbital had no adverse effects on neurocognitive attention outcome., Conclusions: This population study suggests that FC in early childhood does not have adverse effects on behavior, scholastic performance, and neurocognitive attention. On the contrary, the FC group demonstrated significantly better control of distractibility and attention at school age.

Recent epidemiologic data indicate that the vast majority of children with febrile seizures have a normal longterm outcome. A precise knowledge of the short- and long-term outcome with or without treatment, and short- and long-term side effects is an important prerequisite for assessing the various treatment strategies. We focus on the impact of short-term or prophylactic treatment on the short- and long-term outcome of various types of febrile seizures. There is universal agreement that daily prophylaxis with antiepileptic agents should never be used routinely in simple febrile seizures, but only in highly selected cases, if at all. Intermittent diazepam (DZP) prophylaxis at times of fever may or may not reduce the recurrence rate, but it does not appear to improve the long-term outcome as compared with short-term seizure control. The treatment may be used to reduce the recurrence rate for a small arbitrarily defined group with multiple simple febrile seizures, complex febrile seizures, especially focal, prolonged or both, febrile status, and when parental anxiety is severe. However, there is no evidence that treatment of simple febrile seizures can prevent the rare cases of later epilepsy, and many children with complex febrile seizures have a benign long-term outcome, even without treatment. Many prefer a "wait and see" policy. An attractive alternative is to treat new febrile seizures with rectal DZP in solution at seizure onset, given by the parents at home to prevent febrile status. Newer, less well documented short-term strategies include nasal, oral, or rectal administration of other benzodiazepines. Short-term seizure control of febrile status and careful parental counseling are the two most important targets of treatment.

We studied 47 consecutive patients who underwent temporal resection for seizure control. Nineteen (40%) had febrile convulsions preceding onset of their habitual seizures. In 17 of 18 patients whose disease duration was known, the febrile convulsions were prolonged (mean 4 h). As compared with patients without preceding febrile convulsions, patients with antecedent febrile convulsions had a significantly higher prevalence of positive family history of febrile convulsions, an increased incidence of retrospectively identified gestational or perinatal complications, and no foreign tissue lesions. Pathologic studies showed gliosis and cell loss in mesiotemporal structures, usually moderate, in addition to usually mild gliosis in lateral temporal cortex. These patients had an excellent outcome after temporal resection: 84% were seizure-free, had residual auras only, or occasional convulsions with medication discontinuation. One patient (5%) had > 90% improvement. Two patients (11%) in whom the hippocampus was totally spared continued to have complex partial seizures: in both, seizures stopped after reoperation and hippocampal resection. Thus, 95% of these patients had an excellent result. Only 16% required invasive preoperative studies to confirm lateralization. These results were significantly better than those of the group without preceding febrile convulsions (p = 0.0013).

Etiologic, pathologic, and clinical features of possible prognostic significance were studied in 118 children who underwent frontal or temporal lobe epilepsy surgery at the Montreal Neurological Institute (MNI) between 1940 and 1980 (excluding tumor cases). Mean age of seizure onset was 5.1 years (range 0-12 years), mean age at operation was 11.7 years (range 0.6-15 years), and median duration of follow-up was 15 years (range 2-31 years). Thirteen of 45 frontal surgery patients and 43 of 73 temporal surgery patients had an excellent outcome. In the frontal surgery group, this was restricted almost entirely to patients with an established etiology, particularly if this included a circumscribed resectable structural abnormality. In the temporal surgery group, a history of early, prolonged febrile seizures was usually associated with good outcome, unless there was additional evidence of extratemporal epilepsy. In the entire series, the presence of a nonresectable structural lesion or secondarily generalized seizures was associated with a poor outcome.

The cumulative risk of seizures and epilepsy was investigated in a prospectively identified cohort of 221 children with mental retardation (MR) born between 1951 and 1955 in Aberdeen, Scotland. By age 22 years, 33 (15%) had epilepsy. An additional 16 (7%) had had at least one seizure, but did not meet the criteria for epilepsy. The cumulative risk of epilepsy was 9, 11, 13, and 15% at 5, 10, 15, and 22 years, respectively. In children with MR and no associated disabilities, the cumulative risk of epilepsy was only 2.6, 3.2, 3.9, and 5.2% at 5, 10, 15, and 22 years. In children with MR and cerebral palsy (CP), the cumulative risk was 28, 31, and 38% at 5, 10, and 22 years. Children with a postnatal injury associated with MR had a cumulative risk of epilepsy of 53, 66, and 66% at 5, 10, and 15 years after the injury. By age 22 years, 39% had achieved 5-year seizure-free remission, including 56% of children with MR without associated disability, 47% of children with MR and CP, and 11% of children with a postnatal injury. We conclude that, in the absence of associated disability or postnatal injury, the risk of epilepsy in the retarded population is low. Epilepsy in this population also will frequently enter remission in later life.

Forty patients with partial epilepsy that began before they were aged 3 years were recorded at the Centre Saint-Paul between 1981 and 1986 with a follow-up ranging from 1 year 9 months to 20 years. We analyzed the following data: age at onset, clinical features of seizures at onset and during the follow-up period, ictal and interictal EEG features, etiologic circumstances, evolution of the epilepsy, and psychomotor development. The age of onset was mostly between 2 months and 2 years (more than two thirds of cases). Most had partial symptomatic epilepsy. In nine cases, epilepsy was preceded by febrile convulsions. Seizures at onset were of the following type (in order of decreasing occurrence): unilateral seizures, complex partial seizures, elementary partial seizures, and other seizures, often difficult to classify. A few patients with infantile spasms associated with focal or multifocal EEG abnormalities, differing from West's syndrome, were included in this study. We discuss the problem arising from the classification of infantile seizures and epilepsies.

Clinical features of all our 46 patients whose recurrent partial seizures began at age less than or equal to 47 months were studied. Thirty-seven (80%) had motor seizures which were exclusively unilateral in 14 patients, bilaterally synchronous in 8, and both unilateral and bilaterally synchronous in 15. Thirty-three patients (72%) had complex partial seizures (CPS), and 11 patients (24%) had simple partial somatosensory seizures. Clinical seizures were recorded by EEG in 18 patients (39%), and a subclinical (larval) seizure was recorded in 1; those in 10 patients arose from a temporal lobe, 4 were from a frontal lobe, and in 5 they involved two adjacent lobes from onset. Several extra-ictal features reliably indicated lobe or hemisphere of seizure onset. The lobe of principal interictal EEG spikes corresponded to seizure origin in 33 of 34 patients (97%), and regional delta activity corresponded to lobe of seizure origin in 22 of 24 patients (92%). The seizures of all 13 patients with unilateral neurologic signs corresponded to the hemisphere implicated by the deficit. Computed tomography (CT) scan showed lesions in 30 patients (65%) and corresponded to the lobe of seizure onset in 27 of the 30 (90%). Seizures arose primarily from a frontal lobe in 22 of 46 patients (48%), from a temporal lobe in 14 patients (30%), and from an occipital-parietal-posterotemporal region in 6 patients (13%); only the hemisphere of origin could be determined in 4 patients. Cortical developmental abnormalities were the most common known cause (12 patients, 26%); febrile seizures and primary brain tumors occurred in 10 and 8 patients, respectively.(ABSTRACT TRUNCATED AT 250 WORDS)

Isolated infantile convulsions were noted in 206 patients (13%) of 1,572 patients who underwent cortical resection for medically refractory focal epilepsy at the Montreal Neurological Institute over the period 1928 through 1977. In 59 patients (29% of the 206), the isolated infantile convulsion was associated with an identifiable febrile systemic or neural illness. In 54 patients (26%), there was a definite history of cerebral birth injury in addition to the isolated infantile convulsions. Twenty patients (10%) had some other potential etiological factor for the later development of seizures, such as postnatal head injury, Sturge-Weber syndrome, etc., in addition to the isolated infantile convulsions. In 95 patients, 6% of the total 1,572 patient series and 41% of those with isolated infantile convulsions, the latter was the sole apparent cause for the later development of recurring seizures. Although this was the case in a small but significant percentage in this series of patients, more frequently, the isolated infantile convulsion represents a manifestation of invasion of the brain by an infectious agent of a systemic or brain disease, or is a harbinger of the later development of a seizure tendency due to some other preexisting etiological factor.

Benign idiopathic neonatal convulsions and partial benign childhood epilepsy, the most frequent types of childhood benign epilepsy, are both characterized by partial seizures. This study was performed to determine whether any kind of benign epilepsy with partial seizures occurs in the age range between these two syndromes. We identified 17 cases of benign epilepsy with partial seizures from a review of 442 records collected in a 5-year period in which seizures occurred between 8 days and 3 years of age. Six patients with onset before 6 weeks of life had clinical features characteristic of benign idiopathic neonatal convulsions, and appeared to be a delayed type of this syndrome. Eleven patients had clinical features characteristic of partial benign childhood epilepsy. In the age range between occurrence of these two recognized epileptic syndromes, we identified no case of partial epilepsy with favorable outcome.