伴高热惊厥史的儿童癫痌病例分析

分析伴高热惊厥史的癫痌患儿的临床特点,探讨高热惊厥脑损伤及其与颞叶癫痌的关系。 方法对1996～1999年本院儿科神经病房480例住院癫痌患儿进行回顾性分析,包括首发年龄、家族史、持续时 间、癫痌发作类型、神经影像学及脑电图改变等。结果115例(23.9％)患儿有前期高热惊厥史。伴高热惊厥史 的患儿癫痌发作早且易于出现癫痌持续状态。与无高热惊厥史的患儿相比,伴高热惊厥史的患儿强直-阵挛发作 较多,复杂部分性发作较少。408例患儿曾行影像学检查,4例提示有海马硬化者均无高热惊厥史。在伴高热惊厥史 的癫痌患儿中脑电图局灶起源的异常放电显著低于无高热惊厥史的癫痌患儿。有6.08％(7/115)伴高热惊厥史的癫 痌惠儿和6.84％(25/365)无高热惊厥史的癫痌患儿脑电图表现为单纯颞叶异常放电,二组相比无明显差异。结论 在癫痌患儿中,高热惊厥可能伴有脑损伤,且可能与后期的癫痌发生有关,伴高热惊厥史者不一定发展为颞叶癫痌。     

Low morbidity and mortality of status epilepticus in children

In an ongoing study of status epilepticus, 193 children with status epilepticus of varying causes have been followed up for a mean period of 13.2 months. Of these, 97 patients were recruited prospectively. The patients' ages ranged from 1 month to 18 years (mean, 5.0 years). The cause of the status epilepticus was classified as idiopathic in 46 cases, remote symptomatic in 45, febrile in 46, acute symptomatic in 45, and progressive neurologic in 11. The mortality and incidence of sequelae following status epilepticus was low and primarily a function of etiology. Seven children died within 3 months of having the seizure. New neurologic deficits were found in 17 (9.1%) of the 186 survivors. All of the deaths and 15 of the 17 sequelae occurred in the 56 children with acute or progressive neurologic insults. Only two of the 137 children with other causes sustained any new deficits (P less than .001). Duration of the status epilepticus affected outcome only within the acute symptomatic group (P less than .05). Neurologic sequelae occurred in 29% of infants younger than 1 year of age, 11% of children 1 to 3 years of age, and 6% of children older than 3 years of age. However, this was a reflection of the greater incidence of acute neurologic disease in the younger age groups. Within each cause, age did not affect outcome. Of the 193 children, 61 (32%) had a history of prior unprovoked seizures. Of the 125 surviving children with no history of prior unprovoked seizures, 37 (30%) had subsequent unprovoked seizures.(ABSTRACT TRUNCATED AT 250 WORDS)     

Accidental epileptic seizures in children

The authors report a series of 71 children admitted to a general pediatric hospital for a first non febrile, non symptomatic seizure and observed for an average of 6 years and 5 months. Among these patients, 19 cases corresponded to an isolated unexplained seizure without paroxysmal E.E.G. abnormalities, which did not reappear without treatment in a mean follow-up period fo 5 years and 3 months. The typical features of these "accidental seizures" are compared with other types of epilepsy. Finally, these "accidental seizures" can be classified into 2 groups: atonic seizures in the young child (1-4 years) and partial seizures in older children. A statistical analysis was undertaken to define the risk factors for recurrence after the first epileptic attack. A low recurrence risk is expected for children between 1 to 4 years with atonic type of seizures without paroxysmal E.E.G. abnormalities while there is a high recurrence risk for children under 1 year with generalized seizures and paroxysmal E.E.G. intercritical abnormalities.     

Febrile status epilepticus

As part of a study of status epilepticus in children (Maytal J, Shinnar S, Moshe SL, Alvarez LA. Pediatrics. 1989; 83:323-331); 44 children with febrile convulsions lasting more than 30 minutes were followed for a mean of 28 months (range 4 to 72). Thirty children were followed prospectively. Children with prior afebrile seizures or evidence of acute central nervous system infection were excluded. Nine (20%) children had prior neurological deficits. The duration of the febrile seizure was 0.5 to 1 hour in 41 cases (85%), 1 to 2 hours in 5 (10%), and greater than 2 hours in 2 children (5%). No child died or developed new neurological deficits following the seizures. The risk of recurrent seizures was increased, but only in the group with prior neurological abnormality. Six (66%) of these children had subsequent febrile seizures compared with 12 (34%) of the normal children (P = .08). Three (33%) had recurrent febrile status epilepticus compared with only 1 (3%) normal child (P = .023). The 2 children in the prospective arm of the study with recurrent febrile status epilepticus were both neurologically abnormal (P = .035). All 3 of the children who subsequently had afebrile seizures (2 prospective) were neurologically abnormal (P = .006 overall, P = .035 for prospective only). It is concluded that the occurrence of febrile status epilepticus in a neurologically impaired child is a risk factor for subsequent febrile as well as afebrile seizures. The occurrence of febrile status epilepticus in an otherwise normal child does not significantly increase the risk for subsequent febrile (brief or prolonged) or afebrile seizures in the first few years following the episode.     

Recurrence risk after withdrawal of antiepileptic drugs in children with epilepsy: A prospective study

AimTo study recurrence risk after withdrawal of antiepileptic drugs in children with epilepsy.MethodsAll children younger than 14 with two or more unprovoked seizures 24 h apart who were seen at our Hospital between 1994 and 2004 were included consecutively and prospectively followed. Patients previously examined in other centres were excluded. All patients who entered a remission were proposed to stop medication and were followed.ResultsThree hundred and fifty three children with two or more unprovoked seizures were attended. A total of 238 entered a remission period and were proposed to stop medication, 216 accept. Mean seizure-free time before medication withdrawal was 2.2 years. Kaplan-Meier estimate of recurrence risk was 23% at 2 years (95% CI: 17–29) and 28% at 5 years (95% CI: 22–34). A remote symptomatic etiology, various seizure types and a history of prior febrile seizures or prior neonatal seizures were associated with a significant increase in recurrence risk in univariable and multivariable analyses using Cox proportional hazards model. Recurrence risk at 2 years was 17% (95% CI: 11–23) for idiopathic/cryptogenic epilepsies and 41% (85% CI: 28–54) for remote symptomatic epilepsies. Recurrence risks at 2 years by epileptic syndrome were West syndrome (0%), benign rolandic epilepsy (10%), epilepsy without unequivocal partial or generalized seizures (11%), benign infantile seizures (13%), absence epilepsy (16%), cryptogenic partial epilepsies (20%), symptomatic partial epilepsies (45%), symptomatic generalized epilepsies (54%).ConclusionsRecurrence risk after withdrawal of antiepileptic treatment in children is low. Etiology and syndromic diagnosis are the main predictive factors.     

婴儿良性癫癎的临床观察和远期随访研究

目的 研究婴儿良性癫痫的发作特征,脑电图及治疗反应,探讨早期诊断方法。方法 对出生后3-24个月内起病,排除热性惊厥,症状性癫痫及发育异常的婴儿惊厥进行临床观察及寻像脑电图（VEEG）监测,并随访治疗效果和远期预后,结果 42例经2年以上随访确诊为婴儿良性癫痫,其中3例有良性婴儿惊厥家族史,19%惊厥伴有轻微腹泻,67%为短期内频繁发作,无癫痫持续状态,3例VEEG监测证实分别为起源于颞区,枕区及多灶性的部分性发作,发作间期脑电图背景正常,24%睡眠中有Rolandic区小棘波,39例接受抗癫痫单药治疗,平均用药时间9个月,3例未用药物治疗,起病1年内发作均消失,结论 起病早期具有以下特征应考虑有婴儿良性癫痫的可能;（1）起病年龄在3-12个月,不超过24个月,可有婴儿良性惊厥家族史;（2）发病前后精神运动发育正常;（3）发作无诱因,或仅有轻度腹泻等非特异性感染;（4）以部分性发作为主,可继发全身性发作,起病时发作可以很频繁,但无癫痫持续状态;（5）发作间期脑电图背景正常,无典型癫痫样放电,可有睡眠期Rolandic区小棘波;（6）神经影像学正常。     

Prognosis of partial epilepsy.

The prognosis of partial epilepsy in childhood (excluding cases of benign partial epilepsy) was studied; the average follow up period was 7.4 years. Improvement rate of seizure status was 82.3%. We studied favourable prognostic factors and found that those most often associated with seizure improvement were familial convulsions and idiopathic forms, no generalised seizures before partial onset, low frequency of seizures after 12 months of treatment, short duration of epilepsy, and no background activity abnormalities on electroencephalography. We also observed such factors as mental retardation, neurological abnormalities, and behaviour and cognitive disorders. Factors that determined the prognosis for social adjustment were similar to those for seizure improvement. We discuss the favourable prognosis of partial seizures in childhood and the predictive factors.     

One vs. two years of anti-epileptic therapy in children with single small enhancing CT lesions

The duration of anti-epileptic drug (AED) therapy in children with seizures due to single small enhancing CT lesions (SSECTL) is controversial. We sought to determine whether there is any difference in the rate of seizure recurrence after 1 vs. 2 years of AED therapy and to identify the factors predictive of seizure recurrence. A total of 115 consecutive children with seizures and SSECTL were randomly assigned to two groups. Group A received AED(s) for 1 year and Group B for 2 years seizure-free interval. CT scan and EEG were done prior to AED withdrawal and children were followed-up for seizure recurrence for at least 1 year. Association between seizure recurrence and clinical and CT characteristics was analysed. Groups A and B consisted of 55 and 51 children, respectively (nine were lost to follow-up). There were 61 boys and 45 girls; mean age 9.33 years. Most (93 per cent) had focal seizures: 36 per cent complex partial, 22 per cent simple partial, 35 per cent partial with secondary generalization; 21 per cent had status epilepticus. The two groups were comparable in clinical, EEG and CT characteristics. CT scan and EEG prior to AED withdrawal were abnormal in 44 per cent and 33 per cent respectively. Six children, three from each group had seizure recurrence. Significant association was found between seizure recurrence and abnormal CT (persistence/calcification of lesion) and abnormal EEG prior to AED withdrawal (p < 0.01). The relative risk of seizure recurrence in a child with abnormal CT and EEG prior to AED withdrawal was 26.2 (95 per cent confidence interval 3.3-210.2, p = 0.0003). No association was found between seizure recurrence and any of the other variables. There was no difference in seizure recurrence after 1 vs. 2 years of AED therapy. Combination of persistent/calcified CT lesion and abnormal EEG prior to AED withdrawal was the best predictor of seizure recurrence.     

Risk factors for postencephalitic epilepsy in children: a hospital-based study in Taiwan.

To identify clinical, neurophysiological and neuroradiological features in acute encephalitis with predictive value for postencephalitic epilepsy (PEE) in children, a retrospective cohort study by following up 0-17-year-old children with the diagnosis of acute encephalitis was done. Total 330 children were enrolled. Of these, 54 (16.4%) developed epilepsy with a mean follow-up period of 6+/-4.6 years, and 79.6% had the diagnosis of epilepsy within six months after encephalitis. Significant risk factors for PEE include the presence of recurrent seizures, status epilepticus, severe disturbance of consciousness, the existence of focal neurological sign, and the presence of neurological deterioration during hospitalization. Patients with abnormal electroencephalogram, including focal (P<0.05), or profound cerebral dysfunction (P<0.001), and focal cortical abnormalities in neuroimaging (P<0.01), also have higher incidence of epilepsy. Furthermore, children with refractory status epilepticus at presentation also significantly increased the possibility of intractable PEE (P<0.01). We concluded that PEE is not a rare complication of acute encephalitis. Children with refractory status epilepticus or poor control of seizures are more likely to have intractable PEE.     

Actual insights into the clinical management of febrile seizures

Febrile seizures (FS) are a benign epileptic manifestation of infancy occurring between 3 months and 5 years of age and affecting an estimated 2–5 % of children. They have usually no important negative effects on motor and cognitive development. Simple FS (generalized seizures, lasting less than 10 min and single episodes during the same febrile event) have a benign prognosis in almost all cases and do not require an extensive diagnostic workup. In complex FS (focal semiology and lasting more than 10 min, more than one episode during the same febrile event), a more detailed clinical, electroencephalographic, laboratory, and neuroimaging evaluation is necessary because of a higher percentage of underlying detectable causes and a mildly higher risk for later development of epilepsy. Febrile status epilepticus is the most severe type of complex FS even if its morbidity and mortality is extremely low. Simple FS plus (more than one convulsive episode in 24 h) have the same benign prognosis of simple FS. Neither intermittent nor continuous prophylaxis is actually recommended both in simple and complex FS because its side effects outweigh its possible benefits. Conclusion: This review summarizes recent developments into the clinical management of FS including a suggested algorithm for simple and complex FS, the concept of simple FS plus, the controversies about the relationships between FS and hippocampal sclerosis, the relationships between FS and complex syndrome such as Dravet syndrome, genetic epilepsy with FS plus or febrile infection-related epilepsy syndrome, and the results of recent epidemiologic studies on febrile status epilepticus.     

Role of early EEG and neuroimaging in determination of prognosis in children with complex febrile seizure

BACKGROUND: The present study investigates the role of early use of EEG in children with no known neuropathology prior to the first CFS, and the contribution made by computed tomography (CT) and magnetic resonance imaging (MRI) to treatment and prognosis. METHODS: Over a period of 7 years, the authors evaluated 159 children (age range: 2 months-5 years) who were being treated for CFS at Haydarpasa Numune Training and Research Hospital, Pediatrics Clinic, Istanbul, Turkey, and who had no previously known neurological disorder. Patients who presented with febrile seizure were determined to have CFS if they fulfilled the following criteria: <3 months of age when seizure occurred, duration of seizure >/=15 min, more than one seizure occurred during a single episode of illness, or focal seizures and postictal neurological deficit was found. EEG was performed on all patients. CT was performed on the patients who had postictal neurologic deficit or focal seizures. Cranial MRI was performed on patients who had focal findings in their EEGs. RESULTS: Electroencephalogram abnormality was found in 71 cases; 51 of these were diagnosed with epilepsy during follow up. Six of the 16 cases whose EEGs were abnormal between days 2 and 6 were diagnosed with epilepsy. Twenty of the 30 cases whose EEGs were abnormal between days 7 and 10 were diagnosed with epilepsy. All 25 cases who had abnormal EEGs after day 11 were diagnosed with epilepsy. CT was performed for 36 patients, of which five were found to have pathological changes. Pathological changes were detected in two of the nine patients who had cranial MRI. Patients who received CT or MRI were all diagnosed with epilepsy during follow up. CONCLUSION: The results suggest that if neurological examination of CFS patients are normal after their clinical status has stabilised, EEG should be performed after 7 days at the earliest, however for the most accurate diagnosis EEG should be performed 10 days after CFS. The most important predictor for neuroimaging was found to be detection of postictal neurologic deficit. MRI had no advantages over CT in first treating CFS in the emergency unit.     

Risk of seizure recurrence following a first unprovoked seizure in childhood: a prospective study

In a prospective study, 283 children who presented with a first unprovoked seizure were followed for a mean of 30 months from the time of first seizure. Subsequent seizures were experienced by 101 children (36%). The cumulative risk of seizure recurrence for the entire study group was 26% at 12 months, 36% at 24 months, 40% at 36 months, and 42% at 48 months. The cumulative risk of recurrence in the 47 children with a remote symptomatic first seizure was 37%, 53%, and 60% at 12, 24, and 36 months, respectively, compared with a cumulative risk of 24%, 33%, and 36% at 12, 24, and 36 months, respectively, in the 236 children who had had an idiopathic first seizure (P less than .01). In children with an idiopathic first seizure, the electroencephalogram was the most important predictor of recurrence. The cumulative risk of recurrence in the 81 children with abnormal electroencephalograms was 41%, 54%, and 56% at 12, 24 and 36 months, respectively, but only 15%, 23%, and 26% at 12, 24, and 36 months, respectively, in the 138 children with normal electroencephalograms (P less than .001). A history of epilepsy in a first-degree relative was a significant risk factor only in idiopathic cases with abnormal electroencephalograms. In children with a remote symptomatic first seizure, either a history of prior febrile seizures or the occurrence of a partial seizure were significant predictors of recurrence. Age at first seizure and duration of seizure did not affect recurrence risk in either the idiopathic or remote symptomatic group. A total of 84% of the children were not treated with antiepileptic drugs or were treated for less than 2 weeks. Only 9% were treated for longer than 3 months. Treatment did not affect the risk of recurrence. The results suggest that, even without treatment, the majority of children with a first unprovoked seizure will not experiment a recurrence. Children with an idiopathic first seizure and a normal electroencephalogram have a particularly favorable prognosis.     

Electroencephalography in Pediatric Epilepsy

Surface electroencephalography (EEG) is a useful electrophysiological investigation for evaluating a paroxysmal event in children. It measures the electro potential difference between two points on the scalp. It is a non-invasive tool that analyzes neuronal maturation and abnormal cortical excitability. EEG helps in differentiating epileptic from non-epileptic clinical event and focal seizures from generalized seizure. This review is to discuss the rational use of interictal scalp EEG in diagnosis of epilepsy and different types of epilepsy syndromes in children. It further highlights its role in febrile seizure, first unprovoked seizure, status epilepticus and unexplained coma.     

Status Epilepticus

Status epilepticus is a common neurological emergency in childhood and associated with significant morbidity and mortality. Status epilepticus (SE) has been defined as continuous seizure activity lasting more than 30 min or 2 or more seizures in this duration without gaining consciousness between them. However, the operational definition has brought the time down to 5 min. Management can be broadly divided into initial stabilization, seizure termination, and evaluation and treatment of the underlying cause. Diagnostic evaluation and seizure control should be achieved simultaneously to improve outcome. Seizure termination is achieved by pharmacotherapy. Benzodiazepines are the first line drugs for SE. Commonly used drugs include lorazepam, diazepam, and midazolam. In children without an IV access, buccal or nasal midazolam or rectal diazepam can be used. Phenytoin as a second line agent is usually indicated when seizure is not controlled after one or more doses of benzodiazepines. If the seizures continue to persist, valproate, phenobarbitone or levetiracetam is indicated. Midazolam infusion is useful in refractory status epilepticus. Thiopentone, propofol or high dose phenobarbitone are considered for treatment of refractory status epilepticus. Prolonged SE is associated with higher morbidity and mortality. Long term neurological sequelae include epilepsy, behavioural problems, cognitive decline, and focal neurologic deficits.     

婴儿严重肌阵挛癫痫的临床特征和基因突变分析

目的 探讨婴儿严重肌阵挛癫癎(SMEI)的临床特点和基因诊断.方法分析13例SMEI患儿的临床和脑电图(EEG)特点及钠离子通道SCN1A基因突变筛查结果.结果男10例,女3例.8例有热性惊厥和癫痫家族史.惊厥起病年龄2～9个月,平均5.6个月.首次发作为热性惊厥9例.13例在病程早期均以反复发热诱发的全面性或一侧性阵挛或强直阵挛发作为主,其中8例有热性惊厥持续状态.出现无热惊厥的年龄为2～21个月.病程中均出现多种发作类型.发作均有热敏感的特点,诱发因素包括发热、洗热水澡和疫苗接种.起病后出现智力发育落后11例.共济失调5例,锥体束征阳性2例.EEG在1岁前多数正常,1岁后出现全导或局灶放电.头颅MBI检查异常2例.13例均应用多种抗癫痫药治疗,发作均未完全控制.卡马西平和拉莫三嗪使部分患儿发作加重.10例发现有SCN1A基因突变.结论 SMEI的临床特点是:1岁以内起病,首次发作常为热性惊厥;1岁以后出现多种发作形式和智力发育落后;发作具有热敏感的特点;EEG早期正常,以后出现全导或局灶放电.筛查SCN1A基因突变有助于早期明确诊断,指导选择抗癫癎药物.     

Risk of recurrent seizures after the first afebrile grand mal seizure in childhood]

A cohort of 74 children three months to 16 years-old who presented with a first unprovoked seizure were followed for five years to assess the risk of recurrence. Children with febrile convulsions, immediate posttraumatic seizures, meningitis and encephalitis were not included. The risk of recurrence was 68% for a second seizure. 47% of the patients developed an epilepsy. 85% of recurrences occurred within the first 6 months and 100% within 2 1/2 years. A history of epilepsy in a first degree relative, age at first seizure, duration of seizure, initial EEG or neurologic status were not associated with significantly higher risk of recurrence.     

Effective short-term diazepam prophylaxis in febrile convulsions

The efficacy of short-term diazepam prophylaxis in febrile convulsions was evaluated in a prospective, controlled study. A total of 289 consecutive children admitted with their first febrile seizure were randomized into two groups. One group received short-term prophylaxis for 18 months with rectally administered diazepam in solution whenever the temperature was greater than or equal to 38.5 degrees C. The control group received no prophylaxis, but diazepam rectally in the event of new seizures. The short-term prophylaxis, a mean of five doses of diazepam per child per year, afforded effective seizure control; the 18-month recurrence rate was reduced from 39% to 12% (P less than 0.001), the total number of recurrences from 77 to 23 (P less than 0.001), the long-lasting recurrences from 5.0% to 0.7% (P less than 0.05). The risk of subsequent epilepsy within the first 2 years was the same, regardless of receiving prophylaxis (3%) or not (3%); it was low after simple febrile convulsions (no cases of epilepsy in 230 children) but considerable after complex febrile seizures (20%) or seizures associated with severe interictal EEG abnormalities (50%).     

Immediate posttraumatic seizures: is routine hospitalization necessary?

OBJECTIVE: A recent Internet survey of pediatric neurosurgeons showed that 86% routinely admitted children with immediate posttraumatic seizures (PTS) for a brief period of observation. We wished to determine whether certain children meeting predefined criteria could instead be safely discharged from the emergency room. METHODS: We reviewed the records of children admitted during the past 5 years with a diagnosis of seizure and head injury. Children with a minor head injury, a PTS occurring within 24 h of injury and no intracranial abnormalities on admission CT scan were included. Children with previous neurological conditions, a history of prior seizures (other than PTS or febrile seizures), a prior history of anticonvulsant use, or intracranial abnormalities on the admission CT scan were excluded. Records were abstracted for child's age, gender, length of admission, previous history of PTS or febrile seizures, mechanism of injury, location of impact, time between impact and PTS, the number, length and type of PTS, Glasgow Coma Score (GCS) on admission, subsequent complications and hospital costs. RESULTS: Seventy-one children met the inclusion criteria. Eleven children presented to the emergency room with prolonged seizures, transient apnea or persistently low GCS and required admission to the intensive care unit (ICU). Among the 60 remaining children with simple PTS, none had further seizures during the follow-up period, and none had significant complications. The average cost of hospitalization was known for 58 children; after excluding the costs for 5 patients who were admitted to the ICU, the average hospital cost amounted to USD 1,615 per patient. CONCLUSIONS: Our data suggest that children with isolated minor head injuries and simple PTS who recover fully in the emergency room, whose CT scans show no intracranial abnormalities and who have no prior history of neurological disease, epilepsy or anticonvulsant use are at low risk for recurrent seizures or neurological complications, and could potentially be sent home to a reliable caretaker and a stable home situation. However, because of the limited sample size in this study, the statistical risk of a bad outcome may be as high as 9%; we therefore suggest that much larger studies are potentially needed before this becomes a standard policy.