热性惊厥持续状态复发的危险因素分析

目的 探讨儿童热性惊厥持续状态(FSE)复发的危险因素.方法 收集138例FSE患儿的临床资料,并于出院后进行2个月至8.3年的随访.根据随访结果,将患儿分为热性惊厥复发组、癫痫进展组及无惊厥复发组,分析FSE复发的相关因素.结果 根据随访结果,热性惊厥复发30例(21.7％)(热性惊厥复发组),8例(5.8％)进展为...     

Clinical evaluation of the patients with epilepsy following febrile convulsions

We studied clinical, EEG and developmental features of 46 epileptic children following febrile convulsions. Incidence of developing epilepsy was 9.9 percent. Eleven patients (group G) out of 46 had generalized epileptic seizures, and 34 patients (group P) had partial seizures. Febrile convulsions of early onset, partial seizures and postictal neurological symptoms were more striking in group P (p less than 0.05), whereas febrile convulsions of late onset and prolonged seizures were slightly dominant in group G. And EEG abnormalities were more frequent in group P (p less than 0.05). Group P patients had significant number of risk factors (complex features of febrile convulsions) than group G patients (p less than 0.01). The interval between the last febrile convulsion and subsequent epileptic seizures was shorter in group G (p less than 0.01). Although subsequent epileptic seizures were well controlled in the both groups (91% in group G and 82% in group P), intractable seizures were recognized in 9% of group P patients. The patients who had risk factors of prolonged seizures, postictal neurological symptoms and early onset manifested poor controlled epileptic seizures (p less than 0.01). Motor or mental deficits were more frequently associated with group P: in some patients they had been observed before the onset of febrile convulsions. These results suggest that pathogenesis of epilepsy following febrile convulsions may be different among various seizure types of subsequent epilepsy. And the risk factors during febrile convulsions may be related to the prognosis of subsequent epileptic seizures as well as the incidence of developing epilepsy.     

The risk of epilepsy following febrile convulsions

A cohort of 666 children who had convulsions with fever were followed to determine the risks of subsequent epilepsy. High risks were found in children with preexisting cerebral palsy or mental retardation. Other major risk factors were atypical features of the febrile convulsions (such as focal seizures) and duration of febrile seizures for 10 minuts or more. The risk of developing epilepsy by age 20 was about 6 percent for all children who had experienced febrile convulsions. However, this risk figure consisted of a combination of 2.5 percent of children without prior neurologic disorder or atypical or prolonged seizures, and 17 percent of those with such complications.     

Unprovoked seizures after complex febrile convulsions

Children with complex febrile convulsions bear a higher risk of developing epilepsy than children with simple febrile convulsions. Complex febrile convulsions are defined by the presence of prolonged seizures, partial seizures and multiple seizures occurring during the same day. The aim of this study is to delineate the relative significance of each of the three criteria defining complex febrile convulsions. Fifty-seven out of 477 children (12%) admitted for febrile convulsions had complex febrile convulsions and normal neurological examination. Follow-up was available for 48 (84%) of them. Thirteen of these 48 (27%) had epilepsy at follow-up. The mean age of seizure onset among the patients with subsequent afebrile seizures was significantly lower than the rest (10.8 months versus 16.8 months). The patients with partial febrile convulsions showed a trend toward a higher risk (45%) of developing epilepsy than the patients with multiple febrile convulsions (21%).     

Is the long-term outcome of children following febrile convulsions favorable?

The study comprised 80 children aged 6 to 9 years with a history of febrile convulsions. A neurological examination, an interview to assess psychiatric anomalies, and a series of neuropsychological tests were performed on patients with previous febrile convulsions and on matched healthy controls. Children with non-febrile seizures or CNS infections were excluded. Recurrence of febrile seizures in the study group was 41% ( N =33), 18 children (22%) had prolonged febrile convulsions, six (7.5%) patients and two controls showed discrete neurological abnormalities. Behavioral anomalies were exhibited by 22% of the patients and 6% of the healthy children. The neuropsychological test results did not demonstrate significant differences between the children with febrile convulsions and the healthy controls. However, in children with prolonged febrile convulsions, non-verbal intelligence was found to be significantly lower as compared with children with simple febrile seizures and with controls. None of the other parameters tested yielded any differences between patients and controls. Children with multiple recurrences of febrile convulsions performed poorer in all tests when compared with children with only one febrile seizure or with controls. Other factors such as a positive family history of epilepsy, age at onset of febrile convulsions, or duration of the seizure were not found to be of prognostic significance.     

Age at onset of seizures in young children

Age at onset of seizures in young children and its relationship to factors such as prior neurological status and neurological outcome were examined. Of 52,360 children, 39,270 of whom were followed for the full 7 years, a total of 2,635 experienced one or more seizures between birth and 7 years of age. The incidence of nonfebrile convulsions was highest in the first year of life, especially in the first month. Children with neonatal seizures who later developed nonfebrile seizures did so early, two-thirds by 6 months and three-quarters by 1 year of age. Children with neurological or developmental abnormality assessed in the first year of life did not have their first seizure earlier than children without abnormality. Neurological abnormality in the first year of life before any seizure, and the presence of minor motor seizures, were associated with an increased rate of mental retardation and cerebral palsy at age 7, but early age at onset appeared to have little prognostic value regarding intellectual function, cerebral palsy, and epilepsy.     

Childhood-onset epilepsy with and without preceding febrile seizures

OBJECTIVE: To identify characteristics in children with epilepsy that differ between those who did versus did not have a history of preceding febrile seizures. BACKGROUND: Febrile seizures precede epilepsy in 10 to 15% of children. Little is known about the specific types of epilepsy associated with febrile seizures. METHODS: In a community-based, prospectively identified cohort of children, the association between prior febrile seizures and characteristics of the children's epilepsy (seizure type, epilepsy syndrome, age at onset, underlying etiology, family history) were examined for 524 of the children who were aged > or =1 year at onset of epilepsy. RESULTS: Seventy-three (13.9%) had febrile seizures. Children with febrile seizures were more likely to have a first-degree or a second-higher-degree relative with febrile seizures and less likely to have childhood absence epilepsy and absence seizures compared with children without febrile seizures. This was especially true for simple febrile seizures. There was no specific association with localization-related forms of epilepsy. Complex, but not simple, febrile seizures were associated with younger age at onset of epilepsy. There was no evidence that focal or prolonged febrile seizures were associated with localization-related epilepsy or temporal lobe epilepsy per se. Of the three children whose initial MRIs demonstrated hippocampal atrophy, none had a history of febrile seizures. CONCLUSIONS: At the time of diagnosis, febrile seizures are not specifically related to temporal lobe epilepsy or localization-related epilepsy in general. A genetic component for febrile seizures is suggested by its positive associations with family history, especially for simple febrile seizures. Complex febrile seizures represent an underlying age-dependent susceptibility.     

Design and phenomenology of the FEBSTAT study

Purpose: Febrile status epilepticus (FSE) has been associated with hippocampal injury and subsequent hippocampal sclerosis (HS) and temporal lobe epilepsy. The FEBSTAT study was designed to prospectively examine the association between prolonged febrile seizures and development of HS and associated temporal lobe epilepsy, one of the most controversial issues in epilepsy. We report on the baseline phenomenology of the final cohorts as well as detailed aims and methodology. Methods: The “Consequences of Prolonged Febrile Seizures in Childhood” (FEBSTAT) study is a prospective, multicenter study. Enrolled are children, aged 1 month to 6 years of age, presenting with a febrile seizure lasting 30 min or longer based on ambulance, emergency department, and hospital records, and parental interview. At baseline, procedures included a magnetic resonance imaging (MRI) study and electroencephalography (EEG) recording done within 72 h of FSE, and a detailed history and neurologic examination. Baseline development and behavior are assessed at 1 month. The baseline assessment is repeated, with age‐appropriate developmental testing at 1 and 5 years after enrollment as well as at the development of epilepsy and 1 year after that. Telephone calls every 3 months document additional seizures. Two other groups of children are included: a “control” group consisting of children with a first febrile seizure ascertained at Columbia University and with almost identical baseline and 1‐year follow‐up examinations and a pilot cohort of FSE from Duke University. Key Findings: The FEBSTAT cohort consists of 199 children with a median age at baseline of 16.0 months (interquartile range [IQR] 12.0–24.0) and a median duration of FSE of 70.0 min (IQR 47.0–110.0). Seizures were continuous in 57.3% and behaviorally intermittent (without recovery in between) in 31.2%; most were partial (2.0%) or secondary generalized (65.8%), and almost all (98.0%) culminated in a generalized tonic–clonic seizure. Of the 199 children, 86.4% had normal development and 20% had prior febrile seizures. In one third of cases, FSE was unrecognized in the emergency department. The Duke existing cohort consists of 23 children with a median age of FSE onset of 18.0 months (IQR 14.0–28.0) and median duration of FSE of 90.0 min (IQR 50.0–170.0). The Columbia control cohort consists of 159 children with a first febrile seizure who received almost the same workup as the FEBSTAT cohort at baseline and at 1 year. They were followed by telephone every 4 months for a median of 42 months. Among the control cohort, 64.2% had a first simple FS, 26.4% had a first complex FS that was not FSE, and 9.4% had FSE. Among the 15 with FSE, the median age at onset was 14.0 months (IQR 12.0–20.0) and the median duration of FSE was 43.0 min (IQR 35.0–75.0). Significance: The FEBSTAT study presents an opportunity to prospectively study the relationship between FSE and acute hippocampal damage, the development of mesial temporal sclerosis, epilepsy (particularly temporal lobe epilepsy), and impaired hippocampal function in a large cohort. It is hoped that this study may illuminate a major mystery in clinical epilepsy today, and permit the development of interventions designed to prevent the sequelae of FSE.     

FOLLOW-UP STUDY OF 482 CASES WITH CONVULSIVE DISORDERS IN THE FIRST YEAR OF LIFE

A total of 482 patients who had had one or more seizures in the first year of life were followed for at least five years (most for more than 10 years). The patients were divided into four groups: febrile convulsions, infantile spasms, status epilepticus and 'other'. Of those with febrile convulsions, 62 per cent developed normally, compared with 14 per cent in the group with infantile spasms, 15 per cent with status epilepticus, and 24 per cent in the 'other' group. Findings on recurrent seizures, epilepsy and mental retardation and/or neurological abnormalities are also reported. Epilepsy developed equally frequently among those with partial and with generalised seizures, but the former more frequently became mentally retarded. The effects of severity of seizures and other factors are discussed. In general, this research confirmed the grave prognosis after seizures during the first year of life, and not only for West syndrome and status epilepticus. The outcome was more favourable when the seizures were cryptogenic or febrile, isolated, with onset in the second six months, generalised, and when the EEG was normal between seizures.     

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.     

Temporal lobe epilepsy in childhood: reappraisal of etiology and outcome

This article reports the neurologic and psychologic findings, seizure characteristics, family histories, and etiology of clinically and electroencephalographically defined temporal lobe epilepsy in 63 children who were studied retrospectively. Subsequent data were available for 53 patients (84%), 15 of whom had undergone temporal lobectomies; 38 patients had been managed conservatively for at least 2 years. Previous, complicated febrile convulsions were the most common predisposing factor, occurring in 13 patients (21%), while 6 patients had tumors (10%). Of the 10 children whose onset of temporal lobe seizures occurred before 2 years of age, 5 had tumors. The presence of emotional or behavioral problems was related significantly to the presence of borderline or low intelligence, but not to the frequency of seizures. Although there was a tendency for a reduction in seizure frequency over time, only 10% of those managed by medical therapy alone were seizure-free at a mean subsequent examination interval of 6.6 years.     

Komplizierter Fieberkrampf bei eineiigem Zwilling

The case of an identical twin who had a prolonged febrile convulsion at the age of 8 months and developed left mesial temporal lobe epilepsy at the age of 2 years, with left mesial temporal lobe sclerosis in the MRI, is presented. The unaffected twin had frequent short febrile convulsions, and, at the age of 4 years, several hours of unresponsiveness after short febrile convulsions in the course of meningitis; his MRI was normal including T₂ relaxometry. We conclude, analogous to other studies, that prolonged febrile convulsions in children younger than 4 years increase the risk of subsequent mesial temporal lobe epilepsy. Our case also illustrates a genetic component in the occurrence of febrile convulsions.     

Clinical and electroencephalographic findings prior to the onset of juvenile myoclonic epilepsy: A case series

The purpose of this study was to investigate the timing of generalized electroencephalographic abnormalities in patients with juvenile myoclonic epilepsy who were followed up long term before the onset of juvenile myoclonic epilepsy. We enrolled juvenile myoclonic epilepsy patients whose course of epilepsy had been observed for >5 years before the onset of juvenile myoclonic epilepsy, those who had undergone electroencephalogram recording more than twice before the onset of juvenile myoclonic epilepsy, and those who had terminated antiseizure medications for at least 2 years before the onset of juvenile myoclonic epilepsy. Patients who had transitioned from childhood absence epilepsy to juvenile myoclonic epilepsy were excluded. We retrospectively reviewed the medical records and neurophysiological data of the patients. Four patients met the inclusion criteria. One patient was diagnosed with febrile seizures during childhood, and the remaining three had transitioned to juvenile myoclonic epilepsy from other epileptic disorders, such as self-limited epilepsy with autonomic seizures, genetic epilepsy with febrile seizure plus, or nonspecific genetic generalized epilepsy. All patients exhibited generalized spike–wave discharges or photoparoxysmal responses for >2 years before the onset of juvenile myoclonic epilepsy. The four patients had transitioned to juvenile myoclonic epilepsy from other epileptological preconditions. Patients with juvenile myoclonic epilepsy may show generalized electroencephalographic abnormality many years prior to the onset of symptoms. Generalized spike–waves on the electroencephalogram during the course of any type of epilepsy or febrile seizure may be a risk factor for developing juvenile myoclonic epilepsy.     

Febrile convulsions in a Serbian region: a 10-year epidemiological study

The first population-based study in the central region of the Republic of Serbia (total population 283,103) was carried out to assess some epidemiological features of febrile convulsions among children of between 6 months and 5 years of age. During the 10-year period, 1986 to 1995, there were 570 cases of the first febrile convulsions (287 males and 283 females). The average annual incidence rate was 3/1000 (2.9/1000 in males and 3.0/1000 in females), with the highest in 1995. During the study period, a significantly increased linear regression trend was observed. During the follow-up period of 5 years for children who had their first febrile convulsions in 1989 and 1990 (total 154 cases), 27 (17.5%) had a recurrence of the disorder, and ten (6.5%) had one or more afebrile seizures, of whom seven children (4.5% of total sample) developed epilepsy (recurrent afebrile seizures).     

The Idiopathic Form of West Syndrome

Summary: The clinical and electroencephalographic data of 31 children with cryptogenic West syndrome (WS), selected from a series of 103 WS patients, with a follow-up between 4 and 12 years, were studied retrospectively to verify whether this group included patients who fulfilled the criteria for an idiopathic etiology. The results identified a possible idiopathic etiology in 17 patients (55%), who had a family history of other forms of idiopathic epilepsy or febrile convulsions, or who developed, during the follow-up, an EEG genetic trait such as a photoconvulsive response or spike-and-wave discharges, or rolandic spikes. All 17 children had a favorable outcome and all had normal neuropsychological development. Four children (13%) fulfilled the criteria for a true cryptogenic etiology, a causative lesion being suspected, but never proved. At the end of the follow-up all four had seizures, or developmental delay or both, all signs that suggest an underlying cerebral lesion. The other 10 children, representing 32% of the cryptogenic cases, had a good prognosis, with early disappearance of spasms and hypsarrhythmia, and normal neurological development, but none had an EEG epileptic trait or family history of epilepsy or febrile convulsions; although they could have had an idiopathic WS, this was not proved. We conclude that among the children classified as having a cryptogenic WS, many—in our series at least 55%—fulfill the criteria for an idiopathic etiology.     

Intermittent prophylaxis in febrile convulsions with oral diazepam]

Intermittent prophylaxis with oral diazepam is presented as an optional treatment for febrile seizures. This proposition is justified by the severe side effects of the currently used chronic anticonvulsant drug therapy in febrile seizures (phenobarbital and valproate). Nineteen patients aged between 3 months and 5 years were treated. They had either simple or complex febrile seizures. Sixteen patients had at least one prognostic factor for recurrence of febrile seizures: first febrile seizure before 15 months of age, positive family history for epilepsy or febrile seizures, occurrence of a complex febrile seizure or abnormal neurological examination. Three patients had none (cases 8, 12 and 13). We recommended 2.5mg b.i.d. for children younger than 12 months, 5mg b.i.d. for children older than 12 months and younger than 3 years, and 7.5 b.i.d. for children older than 3 years. The results showed that only one patient had febrile convulsions while taking adequate diazepam dosage. Transient side effects occurred in 36.8% of the cases.     

Psychogenic status epilepticus in children

Epilepsy features, psychiatric profile, psychosocial factors, and outcome are described for six children (three males) aged 5-15 years (mean 12.1) with psychogenic status epilepticus (PSE), i.e., prolonged or repetitive psychogenic seizures (PSs), >30 minutes, simulating status epilepticus. They had epilepsy, they were on chronic anticonvulsants (ACVs), and some had other neurological deficits. All received intravenous and/or rectal ACVs prior to suspicion of PSE. PSE was confirmed via video/EEG, demonstrating no epileptogenic activity during alleged seizures. Provocation and placebo therapy techniques were used in two. Psychiatric assessment identified comorbid disorders such as depression, anxiety disorder, obsessive-compulsive disorder, obsessive-compulsive symptoms, and posttraumatic stress disorder. Psychosocial stressors were almost ubiquitous. Psychiatric intervention included psychotherapy, family therapy, and medical treatment in one patient. Outcome was monitored for an average of 3.6 years (3-5 years). PSE did not recur. PSs recurred in three. Psychiatric comorbidity improved in four, who accepted psychiatric intervention and whose epilepsy also improved. In conclusion, the occurrence of PSE in children and adolescents with epilepsy is stressed. Prompt diagnosis was often missed in the acute care setting, and this carries important implications for iatrogenic complications. PSE diagnosis resulted in identification and management of comorbid psychiatric disorders. This was probably important in reducing the predominating anxiety and affective disorders in most patients as well as PSE recurrence. Epilepsy severity and associated deficits were most likely important factors in determining outcome.     

Long term outcome in children affected by absence epilepsy with onset before the age of three years

ObjectiveThe goal of this study was to define the long-term outcome of absence epilepsy presenting before the age of 3 years.MethodsWe retrospectively studied the medical records of 40 children from eight neuropediatric centers in Italy with respect to the personal and family histories of epilepsy or febrile seizures, time of follow-up, cognitive functions, treatment, and outcome.ResultsForty patients were enrolled in this study. They all fulfilled the criteria for absence epilepsy with 3-Hz spike–wave complexes on the EEG, normal neurological examination, and no other seizures types. Seizure onset occurred between 24.1 and 36.0 months. There was a family history of epilepsy in 28%, and of febrile seizures in 13%. Thirty-three patients were treated with valproic acid (VPA), mostly used in monotherapy (26 patients) or in association with ethosuximide. At final follow-up, 33 patients were seizure free and 29 had normal EEGs. Thirty-four patients had a normal intelligence quotient (IQ), whereas 6 had a decreased IQ, mainly associated with poor control of seizures.ConclusionIn our series, absence seizures presenting before the age of 3 appeared to have quite a good long-term clinical prognosis; the neuropsychological outcome was comparable to that of childhood epilepsy presenting after 3 years of age.     

Long-Term Prognosis of Convulsive Disorders in the First Year of Life: Mental and Physical Development and Seizure Persistence

Summary: A follow-up study was made on 304 children (164 boys, 140 girls) with convulsive disorders, excluding occasional convulsions, in the first year of life. All patients except 45 who died were followed until 6 years of age or older. At the final follow-up, the subjects were divided into six groups according to the degree of mental and physical development (groups I-VI). Seizures were regarded as absent if the patient had been seizure free for more than 3 years. At the final follow-up, seizures had ceased in 57.7%, and 43.4% had normal mental and physical development (group I). As to the initial diagnosis, the percentage of group I at the final follow-up was 81.8% with febrile convulsions and 37.6% with epilepsy. In patients without seizures it was 69.7% with febrile convulsions and 55.8% with epilepsy. Some 80.6% of patients with unclassified generalized motor seizures, 11.5% of those with infantile spasms, 2.9% of those with secondary generalized epilepsy other than infantile spasms, 46.4% of those with partial seizures, and 25.0% of those with hemiconvulsive seizures were finally placed in group I. The percentage of patients without seizures was 81.4, 33.0, 34.4, 57.7, and 100%, respectively. As has been suggested, among the first-year epilepsies, a subgroup with a more favorable prognosis may exist. Further studies regarding the etiology, ictal EEGs, and effectiveness of treatment and long-term prognosis of these cryptogenic benign infantile convulsions are needed to provide a firm basis for understanding convulsive disorders in the first year of life.     

Febrile seizures in a South Indian district: incidence and associations

One thousand four hundred and three children participated in a home-based survey of psychiatric disorders in 8- to 12- year-old children in Calicut District, Kerala, India. One thousand one hundred and ninety-two consecutive children underwent neurological and psychometric assessments. The projected number of children with a history of febrile seizures was 120 giving a lifetime incidence of 10.1%. Recurrent febrile seizures predominated and these were strongly associated with a history of perinatal adversity. Febrile seizures were independently associated with indices of infective illness and mothers' education. Epilepsy developed in 2.7% of children with febrile seizures, but no evidence was found that febrile seizures had adverse intellectual or behavioural sequelae.