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.     

Duration of recognized fever in febrile seizure predicts later development of epilepsy

Background: The current report examines the risk of and predictors for developing epilepsy in children with febrile seizure (FS). The present study addresses two factors that were previously identified as predictors of recurrent FS in previous reports: maximum temperature and duration of fever prior to the initial FS. Methods: Children aged 6 months–6 years with an initial simple FS were eligible for the study. The interview included questions about the child's illness, family history of seizures, and other exposure information. In particular, they were asked about the duration of recognized fever prior to the seizure. After the initial interview, parents were called every 3–4 months to ascertain whether any further seizures had occurred and the circumstances under which such seizures occurred. Follow up ≥3 years was attempted for all children. Statistical analysis was done with χ² test, Fisher's exact test, Mann–Whitney U‐tests and logistic regression analysis. Results: Five of 92 children (5.4%) experienced unprovoked seizures and were considered part of an epilepsy group. In four of these five patients, the duration of recognized fever prior to FS fell more than ±2.5 SD outside the distribution for the non‐epilepsy group. Either an unusually short or long recognized fever prior to the initial FS was associated with an increased risk of unprovoked seizures. Conclusions: The duration of recognized fever appears to provide useful information about the risk for the later development of epilepsy.     

616例小儿热性惊厥首次发作的临床特点及危险因素分析

目的了解热性惊厥患儿首次发作的临床特点及危险因素,指导临床医师对有危险因素的患儿采取相应干预措施,降低热性惊厥的发生。方法选取我院2016年8月至2018年8月收治的616例首次热性惊厥患儿为研究对象,回顾性分析患儿的临床特征及首次发作危险因素,并随机抽取同期发热但无惊厥发作(既往也无惊厥病史)的601例患儿为对照组。结果616例热性惊厥患儿,男344例,女272例,汉族584例,蒙古族32例。1岁以下126例(20.5%),~3岁405例(65.8%),3岁以上85例(13.7%)。发作病因中以急性上呼吸道感染[53.6%(330/616)]、疱疹性咽峡炎[25.9%(160/616)]及幼儿急疹[10.5%(65/616)]居前3位。惊厥发作时体温在38.0℃及以上者570例(92.5%),16例(2.6%)患儿惊厥发作后出现发热。534例(86.7%)患儿在发热24 h内出现惊厥发作。608例(98.7%)患儿表现为全面强直阵挛性发作。惊厥持续时间<5 min 548例(89.0%)、~14 min 48例(7.8%)、~29 min 16例(2.6%)及≥30 min 4例(0.4%)。572例(92.9%)患儿在单次热程中仅1次惊厥发作。临床类型中单纯性热性惊厥占88.3%(544/616),复杂性热性惊厥占11.0%(68/616),惊厥持续状态占0.7%(4/616)。危险因素分析显示首次惊厥时年龄、低钠、低铁、低锌、剖宫产、异常出生史、抽搐前1周疫苗接种史及热性惊厥家族史在热性惊厥组和对照组中差异有统计学意义(P<0.05)。Logistic回归分析发现首次发热惊厥年龄、低铁、剖宫产、低钠及热性惊厥家族史是热性惊厥首次发作的独立危险因素(P<0.05)。结论热性惊厥首次发作多见于3岁以内婴幼儿,以单纯性热性惊厥为主,惊厥发作时体温高,易发生于发热后24 h内,病毒感染是最常见病因。引起热性惊厥首次发作的危险因素依次为首次发作年龄、低铁、剖宫产、低钠及热性惊厥家族史,针对危险因素采取相应的干预措施可降低热性惊厥的发生。     

Febrile seizures

Febrile seizures (FS) are the most common seizure disorder in childhood, affecting 2–5% of children between the ages of 3 and 60 months. Differentiation of FS from acute symptomatic seizures secondary to central nervous system infection is essential. Those with a focal onset, prolonged duration or which occur more than once within the same febrile illness are considered complex and have an increase in risk of subsequent epilepsy development. The vast majority of febrile convulsions are simple, lasting only a few minutes and without need of drug intervention. They have an excellent outcome with no increased risk of decline in IQ, subsequent epilepsy or increased mortality. Febrile seizure can recur, and as it often is a frightening and anxiety-provoking event for parents and caregivers, an understanding of the natural history and prognosis should enable the physician to reassure the parents providing an appropriate counselling and reassurance.
Conclusion: Febrile seizure can recur, and as it often is a frightening and anxiety-provoking event for parent and caregivers. An understanding of the natural history and prognosis should enable the physician to reassure the parents providing an appropriate counselling and reassurance.     

Epilepsy and Mental Retardation Following Febrile Seizures In Childhood

ABSTRACT. In an unselected group of children who were seen following an initial febrile convulsion, the frequency of subsequent afebrile seizures was 3.5% and of mental retardation 1%. The most common afebrile seizure type was generalized major (86%). About 3/4 of the children who developed afebrile seizures did so by three years and all by five years following the initial febrile seizure. The children with afebrile seizures differed from those without afebrile seizures in the frequency of neonatal abnormality, family history of mental retardation, focal initial febrile convulsions, and delay in psychomotor milestones before the initial febrile seizure. Only about 1/3 of the children who developed afebrile seizures ever had a recurrent febrile convulsion and none had complex recurrent febrile seizures. Half the children with mental retardation had histories of delay in psychomotor milestones prior to the initial febrile seizure, and no child with mental retardation had any seizure longer than five minutes. The administration of daily phenobarbital did not reduce the frequency of epilepsy, in spite of a significant reduction in the incidence of recurrent febrile seizures. There remains no evidence that the prevention of recurrent febrile convulsions significantly decreases the frequency of afebrile seizures or mental retardation.     

Predictors of febrile seizure: a matched case-control study

In a prospective matched case-control study carried out to determine risk factors of febrile seizures among children in the United Arab Emirates, 84 patients with febrile seizure were identified and were matched with 84 control febrile patients without seizure in the same age range, who attended the same hospital during the same period of time. Logistic regression analysis showed that the age at first seizure, family history of febrile seizure, duration of fever, and height of temperature were the only significant predictors for febrile seizures.     

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.     

Pediatric seizures and their management in the emergency department

Seizures result from paroxysmal involuntary disturbance of brain function. The history and physical examination guide to management, and will help to differentiate seizures from non-epileptic disorders. The studies needed depend on the child’s age, presence or absence of fever, the duration of seizure activity, and clinical examination. Afebrile seizures in children older than 6 months of age require minimal investigation, while younger children are more likely to have an electrolyte abnormality or hypoglycemia that requires treatment in the emergency department. Children with febrile seizures are not at high risk for serious bacterial illness and routine diagnostic evaluation of simple febrile seizures is not indicated. Anti-epileptic drugs should not be routinely initiated in the emergency department in children whose seizures have resolved. We review the management of status epilepticus in this paper.     

Febrile seizures.

Febrile seizures are the most frequent of seizure disorders in childhood. Febrile seizures are most common in children between 6 months and 3 years of age, with a peak incidence at about 18 months. Approximately 30% to 40% of children who experience a febrile seizure will have a recurrence. The majority of febrile seizures occur within 24 hours of the onset of the fever. Febrile seizures can be simple or complex. Diagnostic studies are usually not necessary. Febrile seizures usually are self-limited, and intervention to stop the seizure often is unnecessary. When possible, the cause of the fever should be treated. Continuous preventative anticonvulsant therapy is not recommended for children with either simple or complex febrile seizures. The use of intermittent anticonvulsant therapy is not routinely indicated. Parental educational and counseling is important. The prognosis is excellent.     

Epilepsy and mental retardation following febrile seizures in childhood

In an unselected group of children who were seen following an initial febrile convulsion, the frequency of subsequent afebrile seizures was 3.5% and of mental retardation 1%. The most common afebrile seizure type was generalized major (86%). About 3/4 of the children who developed afebrile seizures did so by three years and all by five years following the initial febrile seizure. The children with afebrile seizures differed from those without afebrile seizures in the frequency of neonatal abnormality, family history of mental retardation, focal initial febrile convulsions, and delay in psychomotor milestones before the initial febrile seizure. Only about 1/3 of the children who developed afebrile seizures ever had a recurrent febrile convulsion and none had complex recurrent febrile seizures. Half the children with mental retardation had histories of delay in psychomotor milestones prior to the initial febrile seizure, and no child with mental retardation had any seizure longer than five minutes. The administration of daily phenobarbital did not reduce the frequency of epilepsy, in spite of a significant reduction in the incidence of recurrent febrile seizures. There remains no evidence that the prevention of recurrent febrile convulsions significantly decreases the frequency of afebrile seizures or mental retardation.     

An epidemiological study of febrile seizures with special reference to family history and HLA linkage.

One hundred and forty four cases of febrile seizures, 95 simple (typical) and 49 complex (atypical); were studied and compared for clinical and epidemiological data and family history of febrile and afebrile seizures. Major results were: maximum age of onset below three years (75%) in both simple and complex groups, male preponderance, respiratory infection as the commonest etiology (69.4%) and maximum seizure onset within 24 hours of fever (73%). The familial prevalence of all seizures was 29.1%, 23.2% in the simple and 40.8% in the complex group (p < 0.01). The familial prevalence of febrile seizures was 20%; similar in both groups. The familial prevalence of afebrile seizures was 13.9%; 6.3% in simple and 28.6% in complex group (p < 0.01). The commonest relative was a sibling (13.2%). The prevalence in parents was 4%. Families with two additional members with history of seizures revealed complex seizure patterns in two-thirds of index cases. There was no correlation between family history of seizures and age at onset or sex. No clear inheritance pattern emerged and polygenic inheritance is likely. One third of eighteen families had siblings with identical segregation of parental HLA-A and B haplotypes. Five families showed the presence of HLA All. This small though adequate sample size did not reveal an HLA marker for febrile seizures.     

Status epilepticus in children: Causes,clinical features and short‐term outcome

Background: We aimed to evaluate the cause, clinical profile, and short‐term outcome of status epilepticus cases that were admitted to our pediatric emergency unit between 1 January and 31 December 2008. Methods: We studied the clinical features of 59 seizures that occurred in 56 patients aged between 3 months and 15 years with the diagnosis of status epilepticus. We observed the clinical course and outcome of 53 cases for 6 to 18 months. The correlation between the cause of the seizure and the patient's age at the time of status epilepticus was evaluated as well as the correlation between the risk of seizure recurrence and family history of seizure, the neurological status of the patient prior to seizure and the presence of epilepsy. Results: The most common cause of status epilepticus is febrile illness in children younger than 2 years and idiopathic/cryptogenic and remote symptomatic causes in children older than 2 years. The rate of recurrence of seizure was significantly higher in cases with existing neurological abnormalities, prior epilepsy and seizures with remote symptomatic causes. The most common triggering factors of status epilepticus development in cases with epilepsy were noncompliance for anti‐epileptic drugs and infectious fever. Conclusions: In our study, the risk factors for seizure recurrence were the presence of prior epilepsy, existence of neurological abnormalities and remote symptomatic causes. We argue that improving the compliance of patients and their families to take medicine appropriately and training them in how to cope with febrile illnesses may decrease the recurrence of seizures.     

Reflex anoxic seizures ('white breath-holding'): nonepileptic vagal attacks.

From clinical history 58 children were diagnosed as having reflex anoxic seizures secondary to provoked cardioinhibition (also known as white breath-holding attacks). Before referral, these seizures were commonly misdiagnosed as epileptic either because the provocation was ignored, not recognised, or was a febrile illness, or because there was no crying, no obvious breath-holding, little cyanosis, and often no pallor to suggest syncope and cerebral ischaemia. The duration of cardiac asystole after ocular compression was measured in these children and in 60 additional children with other paroxysmal disorders. In 45 (78%) of the 58 with reflex anoxic seizures asystole was 2 seconds or over, and in 32 (55%) it was 4 seconds or greater, an abnormal response. Review of the literature supports the concept that these seizures result from vagal-mediated reflex cardiac arrest which can if necessary be prevented by atropine. The simple name 'vagal attack' is proposed. Ocular compression under EEG and ECG control supports the clinical diagnosis if asystole and/or an anoxic seizure is induced; the procedure described is safe and should be routine in seizure or syncope evaluation, when a meticulous history still leaves room for doubt.     

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。     

Seizures associated with fever: clinical data as predictors for normal biochemical blood levels

We developed a predictive model to assess the probability of normal biochemical blood test results in children presenting with a seizure associated with fever. The models were based on various combinations of patient characteristics of the history and physical examination of 203 children. The characteristics included gender, age in years, previous history of febrile seizures, family history of febrile seizures, fever previous to the seizure, vomiting and diarrhoea previous to the seizure. Further, clinical characteristics of the seizure were considered: focal seizure signs, multiple seizure, seizure duration and rectal temperature at seizure. The outcome was defined as normal test results of serum levels of sodium (n=115, 68%), calcium (n=149, 89%) and glucose (n = 173, 100%), according to the hospital reference values. The prevalence of abnormal test results was rather low and the abnormalities were outside the morbidity range. We used logistic regression to relate the outcome to the several clinical characteristics. The discriminative ability of the models was 0.63 (area under the receiver operating characteristic curve of the model predicting normal sodium), 0.66 (normal calcium) and 0.66 (both normal). The score chart we constructed is an additional tool to a carefully performed patient history and physical examination and it may help to decide if a biochemical test is indicated for the individual patient. Conclusion In children with seizures associated with fever, abnormal biochemical blood test results are rare and outside the morbidity range. The biochemical tests are generally not required. In children with a low probability of a normal result as calculated by the score chart, the test may be indicated. Received: 8 October 1997 / Accepted in revised form: 19 January 1998     

Parents'fear regarding fever and febrile seizures

In order to improve the effectiveness of information, we studied parents' perceptions and knowledge about fever and febrile seizures. A questionnaire study was carried out among the parents whose children (n = 230) participated in a randomized controlled trial of ibuprofen to prevent recurrent febrile seizures. Of the 230 parents, 181 (79%) responded to the questionnaire. Of all parents, 45% were afraid or very afraid of fever, which was strongly associated with being afraid of recurrent febrile seizures. Parents of children with a non-West European background were more afraid. The consequences of parental fear included frequent temperature measurements (25% measured five times per day or more), sleeping in the same room (24%) and 13% remained awake at night. Witnessing a febrile seizure is a frightening experience for parents; a majority thought that febrile seizures were harmful, because they look dangerous. Forty-seven percent thought that their child was dying during the initial febrile seizure. On the other hand, reassuring information may be helpful: 21% mentioned it as their reason to consider febrile seizures not harmful. We conclude that parental fear of fever and febrile seizures is a major problem with several negative consequences for daily family life. Adequate provision of information may reduce parental fear. We suggest that information about fever and febrile seizures should be provided to all parents, preferably during their contact with the providers of preventive healthcare. The parents of children with a non-West European origin need extra attention.     

Sudden death in toddlers associated with developmental abnormalities of the hippocampus: a report of five cases.

Sudden unexplained death in childhood (SUDC) is the sudden death of a child older than 1 year of age that remains unexplained after review of the clinical history, circumstances of death, and autopsy with appropriate ancillary testing. We report here 5 cases of SUDC in toddlers that we believe define a new entity associated with hippocampal anomalies at autopsy. All of the toddlers died unexpectedly during the night, apparently during sleep. Within 48 hours before death, 2 toddlers had fever, 3 had a minor upper respiratory tract infection, and 3 experienced minor head trauma. There was a history of febrile seizures in 2 (40%) and a family history of febrile seizures in 2 (40%). Hippocampal findings included external asymmetry and 2 or more microdysgenetic features. The incidence of certain microdysgenetic features was substantially increased in the temporal lobes of these 5 cases compared with the temporal lobes of 39 (control) toddlers with the causes of death established at autopsy (P < 0.01). We propose that these 5 cases define a potential subset of SUDC whose sudden death is caused by an unwitnessed seizure arising during sleep in the anomalous hippocampus and producing cardiopulmonary arrest. Precipitating factors may be fever, infection, and/or minor head trauma. Suggested risk factors are a history of febrile seizures and/or a family history of febrile seizures. Future studies are needed to confirm these initial findings and to define the putative links between sudden death, hippocampal anomalies, and febrile seizures in toddlers.     

Theophylline-associated seizures and their clinical characterizations

BACKGROUND: To elucidate the basic mechanism of theophylline-associated seizures (TAS), the clinical symptoms, electroencephalogram (EEG) and neuroradiological imaging of eight pediatric patients were all retrospectively evaluated. METHODS: Patients whose seizures represented their first episode were selected, while patients with cerebrospinal fluid abnormalities including pleocytosis and protein elevation, present illness of head trauma, epilepsy, febrile convulsion or any psychomotor retardation were excluded although they were given theophylline. RESULTS: Eight patients, 3.5 +/- 1.7 years of age, thus fulfilled the definition of TAS in the past 5 years. Based on their seizure patterns, EEG findings, brain single-photon emission computed tomography and head magnetic resonance imaging, a total of seven of eight patients had either localized or unilateral dominant lesions. They all had fever, > or =38 degrees C, and six of eight patients had a family history of febrile convulsions and/or idiopathic epilepsy. Thereafter none of them had convulsions after the cessation of theophylline administration. Through the TAS event, a 6-year-old female patient was found to have a right deep lateral cerebral venous angioma. CONCLUSION: In infants with idiopathic low seizure threshold and fever, theophylline administration might possibly trigger a seizure. Moreover, based on these patients' clinical findings, some kind of cerebral vascular involvements is speculated to be related with TAS.     

Atrioventricular conduction in children with acute rheumatic fever.

Atrioventricular conduction was quantitatively evaluated in 118 children with acute rheumatic fever. The mean PR index in children with acute rheumatic fever, 1.06 +/- 0.38, was significantly higher than normal children or children who had febrile illness of nonrheumatic or nonstreptococcal origin (P is less than .001). Among 35 children with rheumatic fever and an abnormal PR index, the disease presented as carditis in 21, arthritis in ten, and chorea in four. The mean PR index and the frequency distribution of abnormal PR indices were significantly higher in children with carditis (P is less than .001). Five children who initially had an abnormal PR index and arthritis or chorea subsequently developed carditis. These observations suggest that children with acute rheumatic fever and abnormal PR index warrant close observation for possible clinical evidence of myocardial involvement during subsequent course of the illness.