Early clinical features and diagnosis of Dravet syndrome in 138 Chinese patients with SCN1A mutations

Objective: To summarize the early clinical features of Dravet syndrome (DS) patients with SCN1A gene mutations before the age of one. Methods: SCN1A gene mutation screening was performed by PCR–DNA sequencing and multiple ligation-dependent probe amplication (MLPA). The early clinical features of DS patients with SCN1A mutations were reviewed with attention to the seizures induced by fever and other precipitating factors before the first year of life. Results: The clinical data of 138 DS patients with SCN1A gene mutations were reviewed. The median seizure onset age was 5.3 months. Ninety-nine patients (71.7%) experienced seizures with duration more than 15 min in the first year of life. Two or more seizures induced by fever within 24 h or the same febrile illness were observed in 93 patients (67.4%). 111 patients (80.4%) had hemi-clonic and (or) focal seizures. Seizures had been triggered by fever of low degree (T < 38 °C) in 62.3% (86/138) before the first year of life. Vaccine-related seizures were observed in 34.8% (48/138). Seizures in 22.5% (31/138) of patients were triggered by hot bath. Carbamazepine, oxcarbazepine, lamotrigine, phenobarbital and phenytoin showed either no effect or exacerbating the seizures in our group. Conclusion: The seizure onset age in DS patients was earlier than that was in common febrile seizures. When a baby exhibits two or more features of complex febrile seizures in the first year of life, a diagnosis of DS should be considered, and SCN1A gene mutation screening should be performed as early as possible. Early diagnosis of DS will help clinicians more effectively prescribe antiepileptic drugs for stronger prognosis.     

Refractory grand mal seizures with onset during infancy including severe myoclonic epilepsy in infancy.

In 1978, Dravet proposed a clinical entity called severe myoclonic epilepsy in infancy (SMEI). In the same year, a patient group, which was later called high voltage slow wave-grand mal syndrome (HVSW-GM), is reported in Japan. Both syndromes are very similar, except for seizure manifestation: generalized tonic-clonic convulsions (GTC) with myoclonic and other polymorphic seizures in SMEI vs. GTC only in HVSW-GM. To study the pathophysiology of these refractory epilepsies, the author formulated new clinical diagnostic criteria common to both syndromes as follows: GTC with onset before the age of 1 year as the principal seizure type; an epilepsy entity unclassifiable either as partial or generalized by all the clinical data including EEG findings; mental and motor dysfunction absent prior to seizure onset but appearing later; absence of epileptiform activities on EEG in the initial stage; stubborn refractoriness to conventional antiepileptic medication. Twenty-two patients meeting all of five clinical criteria above mentioned were recruited in the study. Detailed analysis of clinico-electrical features and long-term follow-up of these patients led the author to the conclusion that GTC in combination with seizures of other types will contribute to an unfavorable pathophysiological or prognostic conditions, and, especially when GTC exists in combination with myoclonic seizures, the severity of epilepsy will increase. The author claimed that the three clinical entities, SMEI, HVSW-GM, and their variant form, share certain characteristics in common and may constitute a unique epilepsy syndrome for which a new name of infantile refractory grand mal syndrome (IRGMS) was offered. This is a more basic concept with broader spectrum than SMEI, encompassing not only SMEI but also related borderlands like HVSW-GM. More recently, the author observed that early zonisamide medication within 1 year after seizure onset may improve seizure prognosis in IRGMS, by preventing the development of myoclonic seizures.     

Early diagnosis of severe myoclonic epilepsy in infancy.

Of 329 epileptic patients referred in a six year period with the first seizure occurring in the first year of life, 20 met the following criteria: generalized seizures excluding infantile spasms, myoclonic, tonic or absence seizures, at least one afebrile seizure, normal development prior to the first seizure, normal CT scan, and no etiology. Seventeen of these 20 patients developed the full pattern of severe myoclonic epilepsy in infancy (SMEI). This syndrome was recognizable from the second or third seizure in the first year of life, although epileptiform EEG abnormalities were lacking until the age of 11 to over 30 months. Therefore, based on the clinical pattern, the diagnosis of SMEI can be made with quite good reliability by the end of the first year of life.     

Long-term neurological outcome in children with early-onset epilepsy associated with tuberous sclerosis

In tuberous sclerosis complex, early seizure onset is associated with high risk of intractable epilepsy and cognitive/behavioral impairment. We retrospectively evaluated the long-term outcome of 44 infants presenting with seizures in the first 12 months who received vigabatrin, and were followed up for at least 3.5 years. At the final evaluation 55% of patients were still having seizures, 80% had intellectual disability, and 30% had autism. Sixty-five percent of children who had been treated earlier with vigabatrin after seizure onset achieved seizure freedom, compared with 24% of subjects who received vigabatrin treatment later (P < 0.01). Intellectual disability was present in 61% of the children treated early (group A) and in 100% of the children treated later (group B). Nine percent of group A and 52% of group B had autism (P ≈ 0.001).A shorter gap between seizure onset and start of treatment could reduce the risk of epileptic encephalopathy, minimizing the deleterious effect of seizures, but is not able to completely reverse the tuberous sclerosis complex-associated cognitive impairment.     

Passive toothbrushing-induced seizures: Report of a severely disabled girl

Toothbrushing-induced seizures are rare reflex seizures triggered by the brushing of one’s own teeth. We encountered an 11-year-old girl with severe mental retardation, hypotonic cerebral palsy and epilepsy who presented with toothbrushing-induced seizures. She had had spontaneous brief tonic seizures several times a day since the age of 1 year and 2 months and started presenting with the same type of seizures induced by toothbrushing from the age of 8 years. As she could not brush her teeth by herself due to her disabilities, her mother brushed her teeth daily for her. The interictal EEG showed spike-and-wave complexes in the frontal regions bilaterally. The [Tc-99m]HMPAO-SPECT at the time of the seizure induced by toothbrushing suggested that the seizures originated from the left perisylvian cortex. This is the first report of toothbrushing-induced seizures triggered by the brushing of the patient’s teeth by another person (‘passive toothbrushing’).     

Five-year follow-up of patients with partial epilepsies in infancy

The aim of this study is to clarify the long-term outcome of patients with partial epilepsies that begin in the first 2 years of age. Thirty-nine infants who experienced partial epilepsies with onset by 2 years of age were followed until they were at least 5 years of age. Seizure and developmental outcome were investigated in relation to the following items: delayed psychomotor development before onset of seizures; interictal electroencephalogram (EEG) abnormalities; abnormal neuroimaging; family history of epilepsy; and history of neonatal seizures. Thirty-three infants achieved freedom from seizures and 27 achieved normal development. Delayed development before the onset and abnormal interictal EEG correlated with both poor seizure and developmental outcome. Abnormal neuroimaging findings correlated with a poor seizure outcome. A history of neonatal seizures and lack of a family history of epilepsy correlated with poor developmental outcome. The combination of the first four factors predicted a normal outcome with a sensitivity of 0.79 and a specificity of 0.87. The seizure and developmental outcomes were better in this study than those in previous studies. The combination of the four factors will be useful for predicting the outcomes in patients with partial epilepsies in infancy.     

Clinical and ictal characteristics of infantile seizures: EEG correlation via long-term video EEG monitoring

Purpose

The semiology of infantile seizures often shows different characteristics from that of adults. We performed this study to describe clinical and ictal characteristics of infantile seizures at less than two years of age.

Methods

A retrospective study was done for infants with epilepsy (ages: 1–24 months) who underwent long-term video electroencephalography (EEG) monitoring at Samsung medical center between November 1994 and February 2012. We analyzed the clinical and ictal characteristics of the 56 cases from 51 patients.

Results

In 69% of the patients, the seizure onset was before six months of age and the etiology was symptomatic in one third of the patients. Twelve seizure types were identified; spasms (24%), unilateral motor seizures (18%), and generalized tonic seizures (15%) were the three frequent types of seizure.All partial seizures were well correlated with the partial-onset ictal EEG, however 19.4% (7/36) of clinically generalized seizures revealed partial-onset ictal EEG. About one-thirds (4/11) of generalized tonic seizures had its ictal onset on unilateral or bilateral frontal areas and two out of seven generalized myoclonic seizures showed unilateral frontal rhythmic activities. Hypomotor seizures mainly arose from the temporal areas and hypermotor seizures from the frontal regions.

Conclusions

Even though most of the seizure semiology of infants is well correlated with ictal EEG, some of the generalized tonic seizures or myoclonic seizures revealed partial-onset ictal EEG suggesting localized epileptic focus. Accurate definition of seizures via video EEG monitoring is necessary for proper management of seizures in infancy, especially in some clinically generalized seizures.     

Benign familial and non-familial infantile seizures (Fukuyama-Watanabe–Vigevano syndrome): A study of 14 cases from Saudi Arabia

Purpose: Benign infantile seizures [BIS], familial and non-familial, represent a benign, age-related idiopathic syndrome of infancy. The aim of the current paper is to document the presence of the syndrome in Saudi Arabia and in Arab populations and to discuss the characteristic electroclinical features and the benign nature of this syndrome. Patients and methods: A case series of 275 patients with epileptic seizures (age range: 2 months–13 years) were followed over a period of 3 years and 7 months. The inclusion criteria for BIS were as follows (1) age of seizure onset between 2 and 24 months, (2) normal development before, during and after the onset of seizures, (3) normal interictal EEG, (4) normal brain imaging, and (5) good response to treatment. We analyzed these infants with respect to age at seizure onset, sex, physical and neurological examination, consanguinity, frequency and type of convulsions, associated conditions and laboratory and radiological investigations. A waking and sleeping interictal EEG was performed on all patients, and for one patient (No. 1), ictal EEG and video clips were recorded. Results: Fourteen infants (12.0%) showed electroclinical features consistent with BIS. Eleven patients fulfilled the criteria of benign non-familial infantile seizures (BNFIS), and for three patients, their family pedigrees showed the possibility of benign familial infantile seizures (BFIS). All of the patients responded to anti-epileptic treatment, and 50% of them responded within 3 months. Conclusions: To our knowledge, this is the first study to document the presence of BIS (Fukuyama-Watanabe–Vigevano syndrome) in Saudi Arabian and Arab populations. We highlighted the characteristic features of BIS and demonstrated the benign nature of the syndrome.     

Dravet syndrome: From electroclinical characteristics to molecular biology

The onset of Dravet syndrome typically occurs within the first year, with prolonged, generalized, or unilateral clonic seizures triggered by fever. In the early stages, other types of refractory seizures usually present that include myoclonic seizures, atypical absences, and partial seizures. Electroencephalography (EEG) findings are not pathognomonic, and signs of cognitive arrest or deterioration progressively appear. In contrast, in adults, myoclonic seizures, atypical absences, and focal seizures tend to disappear, and short tonic–clonic seizures, often associating a focal component, persist particularly during sleep. The sensitivity to fever persists into adulthood, and although mental deterioration occurs in infancy, usually leaving patients with severe mental impairment, further deterioration does not occur. The identification of genes associated with Dravet syndrome and related syndromes hints at the complexity of the etiology of such epilepsies. Identifying SCN1A mutations has become useful as a means to support an early diagnosis of Dravet syndrome, to benefit counseling, and to avoid use of antiepileptic drugs (AEDs) that may have adverse effects. However, the defining characteristics of seizure type and EEG patterns initially identified by Dravet remain fundamental to diagnosis.     

Clinical Electroencephalographic Biomarker for Impending Epilepsy in Asymptomatic Tuberous Sclerosis Complex Infants

BackgroundWe assessed the clinical utility of routine electroencephalography (EEG) in the prediction of epilepsy onset in asymptomatic infants with tuberous sclerosis complex.MethodsThis multicenter prospective observational study recruited infants younger than 7 months, seizure-free and on no antiepileptic drugs at enrollment, who all underwent serial physical examinations and video EEGs throughout the study. Parental education on seizure recognition was completed at the time of initial enrollment. Once seizure onset occurred, standard of care was applied, and subjects were followed up until 24 months.ResultsForty patients were enrolled, 28 older than 12 months with completed EEG evaluation at the time of this interim analysis. Of those, 19 (67.8%) developed seizures. Epileptic spasms occurred in 10 (52.6%), focal seizures in five (26.3%), generalized tonic-clonic seizure in one (5.3%), and a combination of epileptic spasms and focal seizures in three (15.7%). Fourteen infants (73.6%) had the first emergence of epileptiform abnormalities on EEG at an average age 4.2 months, preceding seizure onset by a median of 1.9 months. Hypsarrhythmia or modified hypsarrhythmia was not found in any infant before onset of epileptic spasms. All children with epileptiform discharges subsequently developed epilepsy (100% positive predictive value), and the negative predictive value for not developing epilepsy after a normal EEG was 64%.ConclusionsSerial routine EEGs in infants with tuberous sclerosis complex is a feasible strategy to identify individuals at high risk for epilepsy. The most frequent clinical presentation was epileptic spasms followed by focal seizures, and then a combination of both seizure types.     

Early onset and focal spike discharges as indicators of poor prognosis for myoclonic-astatic epilepsy

Background: Myoclonic-astatic epilepsy (MAE) is an epileptic syndrome characterized by unique myoclonus, myoclonic-astatic, or astatic seizures in childhood. MAE prognosis vary from spontaneous remission to intractable seizures with profound mental retardation. Aim: Identifying early risk factors may optimize the treatment of children with MAE. Our hypothesis is early onset age and focal spike discharges on EEG indicate a poor MAE prognosis. Methods: Using the medical records of 9 children with MAE, we analyzed their clinical histories, EEG findings, and seizure symptoms. All patients were given follow-up observations/treatments by our department for at least 2 years after MAE onset. Results: Five of the patients were given favorable prognoses because their seizures disappeared within 2 years of onset; the other 4 received poor prognoses because their seizures continued more than 2 years. MAE onset in patient with refractory seizures was earlier than that in those with a favorable prognosis (7–24 months vs. 23–38 months). All the patients with refractory seizures showed moderate or severe mental retardation. Among the 5 patients with good prognosis, EEGs showed two with focal spike discharges and three with only generalized spike discharges. In contrast, all cases with a poor prognosis had focal spike discharges. Conclusions: MAE onset in patients with refractory seizures occurs earlier than in those with favorable prognosis. Prognosis was excellent when EEG findings show no focal spike discharges. Both early seizure onset and the focal spike discharges associated with MAE are indicators of poor prognosis.     

Improvement of intractable childhood epilepsy following acute viral infection

In this study, we report 11 patients with intractable childhood epilepsy that improved following acute viral infection. The patients were 8 boys and 3 girls. Six of the 11 children were diagnosed as West syndrome (5 of the symptomatic type and 1 of the cryptogenic type). The remaining 5 children were myoclonic seizures. The patients became seizure free within 6 days following acute viral infections without an exchange or addition of antiepileptic drugs (AEDs). The types of acute viral infections were Exanthema subitum (Roseola infantum) in 5 patients, Rotavirus gastroenteritis in 2 patients, Measles infection in 2 patients, Herpetic stomatitis in 1 patient and Common cold in the remaining patient. Salaam seizures and/or tonic spasms disappeared within 6 days after the onset of viral infections, and hypsarrhythmia evolved to localized spikes on electroencephalography (EEG) in the patients with West syndrome. Epileptic seizures disappeared rapidly and EEG gradually normalized or improved in patients with myoclonic seizures. Four patients became seizure free for 5 years to 20 years. In 6 patients, seizures relapsed within 14 days to 1 month after the disappearance of seizures. One child remained seizure free for 12 months after viral infection. Common factors in 4 children who were continuously seizure free include (1) normal or almost normal findings of brain CT/MRI, (2) normal development prior to the onset of epileptic seizures, and (3) a short time interval between the onset of seizures and the acute viral infection. We propose several hypotheses including an immunological effect for the improvement of intractable childhood epilepsy following acute viral infection. Further study may provide important information concerning the mechanism of seizure control and the applicable to treatment for intractable childhood epilepsy.     

Clinical features of epilepsy with pervasive developmental disorder

Purpose

To clarify the clinical features of patients with epilepsy and pervasive developmental disorder (PDD). Methods: We examined 12 outpatients with epilepsy as well as PDD at Seiai Rehabilitation Hospital. Results: The patients comprised 7 males and 5 females. The initial neurological symptoms appeared between 5 months and 4 years of age. The interval between the initial neurological symptoms/developmental delay and seizure onset ranged from several months to twenty years. The seizures started at 10–19 years of age in 8 out of the 12 cases. The types of seizures were astatic-drop in 2 cases, tonic-to-astatic in one, atypical absence (decreased consciousness) and generalized tonic clonic seizures (GTCS) in 3 cases, GTCS in 4 cases, or myoclonic and psychomotor in 2 cases. The mental development distributed from normal to extremely severe retardation. Paroxysmal abnormalities on eegs were focal at the frontal area in 7 cases (58%) and other findings in 5 cases. Presumptive risk factors were prenatal in 6 cases (family history for PDD in 1 case, for epilepsy in 1, twin pregnancy in 2 cases, and other in 2 cases), perinatal in 2 patients, postnatal in 1, and unknown in 3 cases. Conclusions: The seizures occurred most frequently after the onset of neurological symptoms or developmental delay. The frontal lobe dysfunction was associated with seizure onset in 58% of the cases based on the EEG findings. The risk factors were prenatal in 50% of the cases.     

Dravet综合征临床和脑电图特征及演变过程

目的 分析Dravet综合征的临床和脑电图特征及演变过程,以改善对本病的早期诊断与合理治疗.方法 分析50例Dravet综合征儿童各种发作类型的起病年龄、诱因、各年龄段发作特征及其与脑电图演变过程的关系.结果 患儿平均起病年龄(5.5±1.9)个月,病程中持续存在热敏感现象.早期以全面性、一侧性或局灶性惊厥发作为主,以后出现肌阵挛、失神等多种发作类型.出现肌阵挛发作的平均年龄(M50)为16个月,13例(26%)无肌阵挛发作.患儿1岁以内脑电图36例次(76%)正常,在1～2岁期间尽管临床出现多种形式发作,但脑电图异常放电出现率仅在50%左右.3岁以后脑电图背景和异常放电出现率均在90%以上.5/18例(28%)有光敏性反应伴肌阵挛发作.结论 Dravet综合征病程早期脑电图与临床发作呈现不平行的进展过程,2岁之前常表现为临床发作严重而脑电图异常率较低.3岁以后各种脑电图异常特征逐渐充分表现.认识Dravet综合征这种临床和脑电图特征有助于早期做出临床诊断,筛选进行SCN1A基因检测的病例.     

Ictal electroencephalographic findings of neonatal seizures in preterm infants

The aim of this study is to clarify the characteristics of ictal EEG findings of neonatal seizures in preterm infants. Seizures associated with ictal EEG changes were recognized in nine infants with gestational age of less than 37 weeks. Propagation, migration, shifting, changes in morphology of ictal EEG discharges were evaluated. Seizure manifestation was divided into the following categories; motor seizure, apneic seizure, automatic seizure and seizure without clinical symptoms. The types of the seizures were motor seizures in five infants, apneic in two, automatic in one and those without clinical symptoms in five. All seizures were of focal onset. The foci of seizures were temporal in six infants, occipital in two, central in one, and frontal in one. The morphology of ictal discharges was low voltage spikes or sharp waves in six infants, spikes in two, theta waves in one and high-voltage spiky theta in one. The propagation of ictal discharges was focal in five infants and regional in five. The migration of ictal discharges was observed in two infants and a shift in two. There was no clear relation between seizure manifestation and ictal EEG foci, duration of seizures and morphology or propagation of ictal discharges.     

Idiopathic epilepsies with seizures precipitated by fever and SCN1A abnormalities

PURPOSE: SCN1A is the most clinically relevant epilepsy gene, most mutations lead to severe myoclonic epilepsy of infancy (SMEI) and generalized epilepsy with febrile seizures plus (GEFS+). We studied 132 patients with epilepsy syndromes with seizures precipitated by fever, and performed phenotype-genotype correlations with SCN1A alterations. METHODS: We included patients with SMEI including borderline SMEI (SMEB), GEFS+, febrile seizures (FS), or other seizure types precipitated by fever. We performed a clinical and genetic study focusing on SCN1A, using dHPLC, gene sequencing, and MLPA to detect genomic deletions/duplications on SMEI/SMEB patients. RESULTS: We classified patients as: SMEI/SMEB = 55; GEFS+= 26; and other phenotypes = 51. SCN1A analysis by dHPLC/sequencing revealed 40 mutations in 37 SMEI/SMEB (67%) and 3 GEFS+ (11.5%) probands. MLPA showed genomic deletions in 2 of 18 SMEI/SMEB. Most mutations were de novo (82%). SMEB patients carrying mutations (8) were more likely to have missense mutations (62.5%), conversely SMEI patients (31) had more truncating, splice site or genomic alterations (64.5%). SMEI/SMEB with truncating, splice site or genomic alterations had a significantly earlier age of onset of FS compared to those with missense mutations and without mutations (p = 0.00007, ANOVA test). None of the remaining patients with seizures precipitated by fever carried SCN1A mutations. CONCLUSION: We obtained a frequency of 71%SCN1A abnormalities in SMEI/SMEB and of 11.5% in GEFS+ probands. MLPA complements DNA sequencing of SCN1A increasing the mutation detection rate. SMEI/SMEB with truncating, splice site or genomic alterations had a significantly earlier age of onset of FS. This study confirms the high sensitivity of SCN1A for SMEI/SMEB phenotypes.     

A comparative study of febrile and afebrile seizures associated with mild gastroenteritis

Purpose: Seizures associated with mild gastroenteritis have been increasingly reported. We analyzed the clinical characteristics of febrile and afebrile seizures associated with mild gastroenteritis, and attempted to determine the influence of fever in these two groups. Methods: We reviewed the medical records of 59 children presenting with seizures during a mild gastroenteritis episode. They were classified into an afebrile group (n = 27) and a febrile group (n = 32). We compared the age of onset, sex, seizure semiology, frequency, duration, family history, and prior history of seizures between the two groups. Results: The mean age, family history, seizure semiology, and frequency of seizures were not significantly different between the two groups. However, more patients in the afebrile group experienced ?2 seizures/day than in the febrile group (63% vs. 38%, p = 0.051). The febrile patients had a tendency of experiencing prolonged seizures lasting ?5 min compared with the afebrile group (34% vs. 11%, p = 0.063). Prior febrile seizures were noted in 5 of the 32 patients (15.6%) in the febrile group, while none of the 27 patients in the afebrile group had a history of prior seizures (p = 0.056). Conclusions: It seems that the presence of fever may influence the clinical characteristics of seizures associated with mild gastroenteritis. We suggest that afebrile seizures associated with gastroenteritis may be regarded as a distinct condition from those associated with fever, and it needs to be clarified by a further large sample study.     

Seizures with Fever After Unprovoked Seizures: An Analysis in Children Followed from the Time of a First Febrile Seizure

Summary: Purpose: To determine how the onset of unprovoked seizures influences recurrence of seizures with fever in children followed from the time of a first febrile seizure.
Methods: In a prospective cohort of children (n = 428) identified at the time of a first febrile seizure, predictors of a second seizure with fever were identified. The occurrence of a first unprovoked seizure was treated as a time-dependent covariate in a Cox regression model rather than as a censoring point as it traditionally has been in the past.
Results: One hundred forty-three (33.4%) children had further seizures. Seven had further seizures with fever only after onset of unprovoked seizures. After adjustment was made for the four previously described predictors of recurrent febrile seizures (age at onset, family history, height of fever, and duration of fever), the onset of unprovoked seizures was associated with a rate ratio of 3.47 (p = 0.0015), indicating a large increase in the risk of further seizures with fever after onset of unprovoked seizures.
Conclusions: Young children who develop unprovoked seizures after a febrile seizure are at substantial risk for further seizures with fever. This may represent part of the spectrum of benign febrile seizures or it may represent the so-called "epilepsy triggered by fever" spectrum. It affects only a small proportion of children with febrile seizures; however, in some children, it may be useful information to consider when making treatment decisions.     

Reversible EEG changes during rotavirus gastroenteritis

Rotavirus can cause seizures and encephalopathy in infants and young children. We report reversible EEG abnormalities in a 2 year and 7 month old boy who had a single seizure during rotavirus gastroenteritis. His EEG showed bilateral independent temporoparietal sharp waves. The EEG was normal 10 days after the first EEG and the patient has been followed with no neurological sequelae. The EEG literature in children developing seizures secondary to rotavirus infection including ours suggest the involvement of mostly posterior cerebral areas. The localization and reversibility of the process can enlighten the pathophysiology underlying this disorder.     

Five pediatric cases of ictal fear with variable outcomes

Purpose: Ictal fear is an uncommon condition in which fear manifests as the main feature of epileptic seizures. The literature has suggested that ictal fear is generally associated with poor seizure outcomes. We wanted to clarify the variability in seizure outcome of children with ictal fear. Subjects and methods: We identified five pediatric patients with ictal fear who were followed up on at Okayama University Hospital between January 2003 and December 2012. We retrospectively reviewed their clinical records and EEG findings. Results: The onset age of epilepsy ranged from 8 months to 9 years and 10 months. The common ictal symptoms were sudden fright, clinging to someone nearby, and subsequent impairment of consciousness, which were often accompanied by complex visual hallucinations and psychosis-like complaints. Ictal fear, in four patients, was perceived as a nonepileptic disorder by their parents. Ictal electroencephalograms (EEG) of ictal fear were obtained in all patients. Three showed frontal onset, while the other two showed centrotemporal or occipital onsets. Two patients were seizure free at last follow-up, while seizures persisted in the other three. A patient with seizure onset during infancy had a favorable outcome, which was considered to be compatible with benign partial epilepsy with affective symptoms. Conclusion: Ictal fear is not always associated with a symptomatic cause or a poor seizure outcome. It is quite important to make a correct diagnosis of ictal fear as early as possible to optimize treatment.