Adolescent Onset of Idiopathic Photosensitive Occipital Epilepsy After Remission of Benign Rolandic Epilepsy

Summary: Purpose: We describe 2 girls, aged 19 years, who experienced a rolandic seizure at ages 4 and 5, respectively, together with the interictal EEG features of benign rolandic epilepsy (BRE). In adolescence both patients developed photosensitive occipital seizures accompanied by spontaneous and photic-induced occipital EEG paroxysms.
Methods: We have been following 33 patients with a history of BRE, between ages 12 and 28 years (mean 17 years). Twenty-one of these patients had experienced their last rolandic seizure before the age of 10 years and 9 of them had been without treatment since age 11 or earlier. In 2 of these 9 patients, other types of seizures recurred after remission of BRE. Clinical, EEG, and evoked potential findings on these 2 patients are presented.
Results: After having experienced BRE, both patients suffered partial seizures from age 12, with elementary visual hallucinations, visual blurring, slow head turning, cephalic pain, epigastric discomfort, unresponsiveness, and vomiting. Seizure onset was related to watching TV or exposure to bright light. EEG showed interictal occipital spikes, and a photoparoxysmal response limited to the occipital lobes. Visual evoked potentials were greatly increased in amplitude. One patient had two visual attacks only and remained seizure free after 4 years of follow-up, while the other had seizures controlled by an association of valproate and carbamazepine.
Conclusions: Clinical and neurophysiological characteristics suggest that these two patients may have presented different age-related expressions within the spectrum of a benign seizure susceptibility syndrome rather than sharply distinct epilepsy syndromes.     

Benign Focal Epilepsies of Childhood

Until relatively recently, genetic influences in partial seizures were thought to be of minimal importance. However, with further identification of childhood benign partial seizures it is becoming clear that inheritance plays a major role in the pathogenesis of these seizures. Diagnostic criteria proposed for benign partial seizures include absence of neurologic or intellectual deficits, family history of epilepsy, onset of seizures after age 2 years, stereotyped brief seizures, frequent nocturnal occurrence, spontaneous remission in adolescence, and electroencephalograms (EEGs) demonstrating spikes with a distinctive morphology and localization superimposed on normal background activity. The two most commonly described benign partial epilepsies of childhood are benign Rolandic epilepsy (BRE) and benign occipital epilepsy (BOE). Both disorders begin in childhood, are associated with characteristic EEG patterns, have seizures that are easily controlled with medication, often are familial, and have an excellent prognosis. The other benign partial seizure disorders in children that have been described are not as well studied as BOE and BRE, and the role of inheritance pattern, if any, is less clear.     

Rolandic Epilepsy: An Incidence Study in Iceland

Summary: Purpose: We wished to determine incidence, clinical features, and prognosis of benign rolandic seizures (BRS) and benign rolandic epilepsy (BRE) in a total population.
Methods: Cases were ascertained through review of all EEG records, and diagnosis was verified by review of medical records. Follow-up information regarding seizures and treatment was obtained from parents and treating physicians.
Results: In the Icelandic population aged 3–15 years, the incidence of BRS is 6.2 and BRE 4.7 in 100,000. Five years after onset 95% were seizure-free. At last follow-up, all were seizure free and had not been treated with antiepileptic drugs (AEDs) for at least 1 year.
Conclusions: Our study demonstrates that BRS is a common entity in children. The prognosis is excellent and treatment is not necessary in all cases. It is important to identify BREBRS correctly and distinguish it from other types of epilepsy.     

Panayiotopoulos-type benign childhood occipital epilepsy: a prospective study

OBJECTIVE: To characterize the clinical and EEG features of the syndrome of benign childhood partial seizures with ictal vomiting and EEG occipital spikes (Panayiotopoulos syndrome [PS]). METHODS: Prospective study of children with normal general and neurologic examinations who had seizures with ictal vomiting and EEG with occipital spikes. RESULTS: From February 1990 to 1997, the authors found 66 patients with PS and 145 children with benign childhood epilepsy with centrotemporal spikes. Peak age at onset of PS was 5 years. Ictal deviation of the eyes and progression to generalized seizures were common. One-third had partial status epilepticus. During sleep, all had seizures. While awake, one-third also had seizures. Five children with PS had concurrent symptoms of rolandic epilepsy and another five developed rolandic seizures after remission of PS. Prognosis was excellent: one-third had a single seizure, one-half had two to five seizures, and only 4.5% had frequent seizures. CONCLUSIONS: Panayiotopoulos-type benign childhood occipital epilepsy is less common than benign childhood epilepsy with centrotemporal spikes but is well defined and recognizable by clinical and EEG features.     

伴中央颞区棘波的儿童良性癫痫典型及非典型病例探讨

目的 探讨ＢＥＣＣＴ典型与非典型特征,以提高对该病的认识。方法 分别５６例ＢＥＣＣＴ临床发作形式、ＥＥＧ表现、影像学及治疗资料。结果 ５６例中典型者２３例（４１．０７％）;不典型病例３３例（５８．９３％）,其中临床发作形式不典型１６例（２８．５７％）,ＥＥＧ不典型２５例（４４．６４％）。头ＣＴ及ＭＲＩ检查正常４６例,轻度异常１０例。卡马西平及丙戊酸对治疗典型和非典型的ＢＥＣＣＴ均有效,以卡马西平为优。结论 ＢＥＣＣＴ的诊断应建立在对发病年龄、发作时间及形式、发作间期ＥＥＧ综合分析基础上,可以有不典型的发作。曾接受ＡＥＤｓ治疗者ＥＥＧ可能不典型。     

The effect of lamotrigine on the EEGs of children and adolescents with epilepsy

Lamotrigine (LTG) is one of the newer-generation antiepileptic drugs (AEDs) with broad-spectrum efficacy against a variety of seizures and epileptic syndromes. We retrospectively evaluated the effects of LTG as add-on therapy on EEGs of children and adolescents. The EEGs of 53 patients (mean age: 12.5 years) with primarily pharmacoresistant epilepsy were reviewed prior to and after LTG add-on therapy. Multiple seizure types were seen in 25, generalized seizures in 15, and complex partial seizures in 13 of the patients. Preceding LTG therapy, the baseline EEG was abnormal because of slow background in 60.3% and localized spikes in 35.8%, generalized spikes in 28.3%, or both in 24.5%. The EEG analysis during the 2-year follow-up period showed improvement in the background in 21.9%, interictal activity in 37.8%, and ictal pattern in 41.1% of the EEG recordings. Overall, LTG resulted in improvement in electrographic features which paralleled the clinical improvement.     

Spike persistence and normalization in benign epilepsy with centrotemporal spikes – Implications for management

Purpose

This study was performed 1) to determine the timing of spike normalization in patients with benign epilepsy with centrotemporal spikes (BECTS); 2) to identify relationships between age of seizure onset, age of spike normalization, years of spike persistence and treatment; and 3) to assess final outcomes between groups of patients with or without spikes at the time of medication tapering.

Benign childhood epilepsy with centro-temporal spikes (BCECTS): early onset of seizures is associated with poorer response to initial treatment.

AIM: Benign childhood epilepsy with centro-temporal spikes (BCECTS) is the most common idiopathic partial epilepsy in children. Treatment attitudes remain a controversial issue. We examine features that could suggest refractoriness at onset. METHODS: We retrospectively reviewed the medical records of 144 children with BCECTS diagnosed at the Division of Pediatric Neurology, Asan Medical Center, from March 1, 1995, to April 30, 2002 and treated with AEDs. The patients were subdivided into two groups according to the number of antiepileptic drugs used for effective seizure control. RESULTS: Of the 144 patients, 75 were male and 69 were female, with a mean age at seizure-onset of 7.2 +/- 2.3 years (range, 2.1-14.3 years); 119 children were taking one antiepileptic drug (AED) (Group A), and 25 were taking more than one (Group B). There were no significant group differences in female-to-male ratio, prescribed AEDs, number of seizures before the start of treatment, interval between seizure-onset and start of treatment, presence of secondarily generalized seizures, or presence of bilateral EEG abnormalities. The groups differed however, in mean age at seizure onset (7.6 +/- 2.2 years versus 5.1 +/- 1.9 years, p < 0.05) and percentage of patients with seizure-onset before 3 years (p < 0.05). CONCLUSIONS: When treated with AEDs, children with BCECTS usually respond well. However, an earlier onset of seizures is associated with more frequent seizures and initial refractoriness to medical treatment.     

Rolandic epilepsy: report of 53 cases]

The clinical and EEG features of 53 out-patients with benign partial epilepsy of childhood with rolandic spikes were studied. The age mean (years) of seizures onset was 5.5 +/- 3.2. Simple partial seizures with speech arrest were more frequent than other seizure types. In seven cases (13.2%) cognitive disabilities were present. In the left-side foci a expected correlation between the interictal EEG focus and clinical lateralization of seizure was observed; in the right-side foci, the right-side interictal focus was correlated with ipsilateral seizures.     

Functional partial epilepsies in childhood

Out of the 314 children with partial epilepsies below the age of 15 who had been followed for more than 5 years, 122 children with functional partial epilepsies (FPE) were the subjects of this study. The specific modality of interictal EEG findings reminiscent of that of the Rolandic spikes (Dalla Bernardina, 1982, 1984) was compared between functional and organic partial epilepsies. The results were: 1) One hundred and twenty-two patients were found to have FPE out of a total of 314 patients (39%). 2) Fifty-five patients were diagnosed as typical benign epilepsy of children with centro-temporal EEG foci (BECCT) of the 122 functional cases (45%). The remaining 67 patients who defied the diagnostic criteria of BECCT were; 9 cases with frontal EEG focus (7%), 11 cases with central and/or midtemporal EEG foci (9%), 10 cases with occipital EEG focus (8%) and 14 cases with multiple foci (11%). 3) Unfavorable seizure control was observed in cases with frontal foci (44%) and with multiple EEG foci (64%) compared with those whose EEG focus was located in the centro-midtemporal area, (including BECCT; 98%) and in the occipital region (80%). 4) It was noteworthy that the recurrent interictal spikes similar to those of the Rolandic spikes (Dalla Bernardina) were found in more than 50% of the patients not only with functional but with organic partial epilepsies. Our results, at least at the present time, appear to indicate that the recurrent spikes never did signify the benignancy of the seizure prognosis. The importance of differential diagnosis of BECCT from FPE was specifically stressed.     

Functional Partial Epilepsies in Childhood

Abstract: Out of the 314 children with partial epilepsies below the age of 15 who had been followed for more than 5 years, 122 children with functional partial epilepsies (FPE) were the subjects of this study. The specific modality of interictal EEG findings reminiscent of that of the Rolandic spikes (Dalla Bernardina, 1982, 1984) was compared between functional and organic partial epilepsies.
The results were: 1) One hundred and twenty-two patients were found to have FPE out of a total of 314 patients (39%). 2) Fifty-five patients were diagnosed as typical benign epilepsy of children with centra-temporal EEG foci (BECCT) of the 122 functional cases (45%). The remaining 67 patients who defied the diagnostic criteria of BECCT were; 9 cases with frontal EEG focus (7%), 11 cases with central and/or mid-temporal EEG foci (9%), 10 cases with occipital EEG focus (8%) and 14 cases with multiple foci (11%). 3) Unfavorable seizure control was observed in cases with frontal foci (44%) and with multiple EEG foci (64%) compared with those whose EEG focus was located in the centro-midtemporal area, (including BECCT; 98%) and in the occipital region (80%). 4) It was noteworthy that the recurrent interictal spikes similar to those of the Rolandic spikes (Dalla Bernardina) were found in more than 50% of the patients not only with functional but with organic partial epilepsies. Our results, at least at the present time, appear to indicate that the recurrent spikes never did signify the benignancy of the seizure prognosis. The importance of differential diagnosis of BECCT from FPE was specifically stressed.     

Sulthiame add-on therapy in children with focal epilepsies associated with encephalopathy related to electrical status epilepticus during slow sleep (ESES)

Purpose: In children with symptomatic or idiopathic focal epilepsies, their disease may evolve into an epileptic encephalopathy related to continuous spike and wave during slow sleep (CSWS) or electrical status epilepticus during slow sleep (ESES). ESES syndrome implies serious risks of neuropsychologic impairment, and its treatment has frequently been disappointing. The aim of this study is to present our experience using sulthiame as add‐on treatment in 53 patients with ESES syndrome that was refractory to other antiepileptic drugs (AEDs). Methods: Neurologic examinations, cerebral magnetic resonance imaging (MRI), and repeated prolonged sleep electroencephalography (EEG) studies were performed in all cases. Data about school achievements and or neuropsychological evaluations were obtained repeatedly during the follow‐up of 1.5–16 years. Sulthiame was added in doses ranging between 5 and 30 mg/kg/day. Key Findings: Since add‐on of sulthiame, 10 of 28 patients in the symptomatic group became seizure free: 4 patients with normal EEG studies and 6 with residual spikes. Nine of 28 patients showed a significant reduction in number of seizures and presented spikes but no ESES on EEG. The other nine cases showed neither clinical nor EEG improvement. A striking result was that 3 of 11 children with unilateral polymicrogyria and ESES syndrome became seizure free, and in another six a significant improvement in frequency of seizures and in EEG abnormalities seemed to be related to the add‐on of sulthiame. Twenty‐one of the 25 patients in the idiopathic group became seizure free and without ESES in <3 months after add on of sulthiame. In two of the patients the changes were seen in a few days. Significance: We understand that sulthiame may be effective as add‐on treatment in children with ESES syndrome.     

结节性硬化致癫痫发作的临床特点及治疗观察

目的　分析结节性硬化 (TS)所致癫痫的临床特点及治疗效果。方法　对 2 0例TS所致癫痫患者的临床发作特点 ,电生理、影像学资料及抗癫痫药物 (AEDs)的治疗效果进行分析。结果　本组癫痫发作均出现在 1 0岁之前 ,其中 2岁之前发病 1 5例 (75 .0 % ) ,主要发作类型为部分性发作 1 0例 (50 .0 % )。 5例患者在疾病发展过程中 ,从部分发作转化为全身强直 阵挛发作。 2例结节位于皮质且直径 >1 0mm ,其位置与脑电图 (EEG)主要放电的部位相符。 1 1例 (61 .1 1 % )AEDs治疗有效。 1 0例随诊 1年以上 ,其中 3例恶化。结论TS所致癫痫患者发病年龄低 ,主要发作类型为部分性发作 ,在疾病的发展过程中 ,发作形式可发生转变。皮质结节的大小与致痫灶有关。AEDs治疗有效 ,但随年龄增长 ,疗效下降     

Early-Onset Benign Childhood Occipital Seizure Susceptibility Syndrome: A Syndrome to Recognize

Early-onset benign childhood occipital seizures (EBOS) described by Panayiotopoulos constitute the commoner after the rolandic phenotype of a childhood seizure susceptibility syndrome. EBOS are the clinical representative of occipital spikes. Their cardinal features are infrequent (often single) partial seizures manifested with deviation of the eyes and vomiting, frequently evolving to hemi- or generalized convulsions. Ictal behavioral changes, irritability, pallor, and rarely cyanosis, and eyes wide open are frequent. Retching, coughing, aphemia, oropharyngolaryngeal movements, and incontinence may occur. Consciousness is usually impaired or lost, either from the onset or the course of the fits, but in a few children, it may be preserved. Duration varies from a few minutes to hours (partial status epilepticus). Seizures are usually nocturnal, but semiology is similar in nocturnal or diurnal fits. Onset is between 1 and 12 years with a peak at 5 years. One third of children have a single seizure, the median total number of fits is two to three, and the prognosis is invariably excellent, with remission usually occurring within 1 year from onset. A few children may later develop rolandic or other benign partial seizures. The likelihood to have seizures after age 12 years is exceptional and rarer than that of febrile convulsions. EEG shows occipital paroxysms demonstrating fixation-off sensitivity, but random occipital spikes, occipital spikes in sleep EEG alone, or normal EEG may occur. Centrotemporal and other spike foci may appear in the same or more frequently in subsequent EEGs. The EEG does not reflect clinical course and severity.     

Abstracts of the 38th Congress of the Japan Epilepsy Society, Shizuoka, Japan, September 30-October 1, 2004

Diagnosis and Treatment of Idiopathic Focal Epilepsies (Benign Partial Epilepsies) in Infancy and Childhood. ¹ Tamiko Negoro ( ¹ Department of Laboratory Medicine, Nagoya University Hospital, Nagoya, Japan ). Introduction: According to the revised classification of epilepsies and epileptic syndromes proposed by the Commission on Classification and Terminology of the International League Against Epilepsy (1989), benign childhood epilepsy with centrotemporal spikes (BCECT), childhood epilepsy with occipital paroxysms (Gastaut‐type late‐onset CEOP), and primary reading epilepsy were included in the idiopathic localization‐related epilepsies (ILRE). Since then, new epileptic syndromes such as Panayiotopoulos‐type early‐onset benign childhood occipital epilepsy (also known as benign childhood epilepsy with occipital paroxysms or BCEOP) and benign partial epilepsies in infancy (BPEI) have been additionally included as ILRE. The diagnostic criteria for benign partial epilepsies include (1) normal neurological examination; (2) normal intelligence; (3) normal neuroimaging; (4) a family history of benign‐type seizures; (5) brief stereotyped seizures; (6) frequent nocturnal occurrence; (7) easy control with antiepileptic drugs (AEDs), except ethosuximide; and (8) remission before adolescence. The EEG features include (1) normal background activity; (2) spikes with a characteristic morphology and location; (3) sleep activation; and (4) occasional generalized paroxysms. In our 114 cases of childhood onset localization‐related epilepsies (LRE) diagnosed between 1997 and 2000, 48 cases (42%) were diagnosed as ILRE and 66 were cryptogenic or symptomatic LRE. Among the 48 ILRE cases, 28 (58%) were classified as BCECT, six (13%) as BPEI, five (10%) as BCEOP, two (4%) as atypical benign partial epilepsy, and seven (15%) as unclassified. Carbamazepine (CBZ), valproate (VPA) and clonazepam (CZP) were equally effective in controlling the seizures in our ILRE patients. This review describes briefly the diagnosis and treatment of BCECT, BCEOP, and BPEI. Benign Childhood Epilepsy with Centrotemporal Spikes: BCECT is the most common ILRE (also known as idiopathic focal epilepsies) in infancy and childhood. Sylvian seizures (simple partial seizures or focal motor or sensory seizures beginning unilaterally at the lower part of the face) and EEG findings (central‐midtemporal high‐amplitude, repetitive sharp waves) are quite stereotyped, and diagnosis of typical cases is relatively easy. Frontal lobe seizures with bilateral perioral twitches or unilateral eyelid twitches may sometimes be misdiagnosed as Sylvian seizures. Since status epilepticus is very rare and many patients only have occasional seizures, many physicians choose not to treat the disorder. If treatment is necessary, the seizures are usually easily controlled with carbamazepine, valproic acid, or clonazepam. Continuation of AEDs is not necessary after the EEG has normalized around puberty. The prognosis is excellent. However, a small percentage of patients may show atypical evolutions such as atypical benign partial epilepsy, epilepsy with continuous spike‐and‐waves in slow wave sleep, or epilepsy with centrotemporal spikes and oromotor deficit. Seizure and EEG exacerbations by AEDs, especially carbamazepine, have to be considered. Early recognition and proper treatment are necessary for these conditions. Benign Childhood Epilepsy with Occipital Paroxysms: BCEOP is also known as Panayiotopoulos‐type early‐onset benign childhood occipital epilepsy. The mean age at first seizure is around 5 years. Seizures comprise an unusual constellation of autonomic, mainly emetic, syndromes with unilateral deviation of the eyes and impairment of consciousness. Visual symptoms, which are the main symptoms in Gastaut‐type, late‐onset CEOP, are exceptions in BCEOP. According to our 41 cases examined between 1984 and 1993, two‐thirds of the patients show hemi‐ or generalized convulsions and one‐fourth experience prolonged seizures for longer than 30 minutes. EEG findings (bilateral occipital paroxysms) consist of some extraoccipital abnormalities especially in the frontal area (48% of our cases) or generalized paroxysmal discharges (32% of ours cases), together with various migrations of spike foci. EEG findings seem to normalize around the age of 10 years. Many patients only have occasional seizures and these seizures are usually easy to control with carbamazepine or valproic acid. The prognosis is excellent. Prevention of status epilepticus is the major problem in this disorder. Recently, new concepts including early‐onset benign occipital seizure susceptibility syndrome (EBOSS) and Panayiotopoulos syndrome (idiopathic susceptibility to early‐onset benign childhood seizures with mainly autonomic symptoms), have been introduced for BCEOP. Benign Partial Epilepsies in Infancy: BPEI comprise two forms. One is partial epilepsy with complex partial seizures (CPS) and the other is partial epilepsy with secondarily generalized seizures (SGS). Onset is mostly during the first year of life. Seizures often occur in clusters and are characterized by motion arrest, decreased responsiveness, staring or blank eyes often with automatisms, and mild convulsive movements in the CPS form; and motion arrest or opening of eyes with staring or blank eyes followed by generalized tonic–clonic seizures in the SGS form. Interictal EEGs mostly show no abnormal findings. According to the ictal EEGs, the most frequent site of seizure origin is in the frontal or temporal area in the CPS form; and central, parietal, or occipital area in the SGS form. Treatment with carbamazepine, phenobarbital, zonisamide or valproic acid immediately stops the cluster of seizures. The prognosis is excellent. Recently, sleep‐related and low‐voltage Rolandic and vertex spikes have been reported as an EEG marker of benignity in this disorder. Most of benign infantile convulsions may belong to partial epilepsy with SGS, although confirmation with ictal EEG recording is necessary for accurate diagnosis.     

Epileptic encephalopathy with continuous spikes and waves during sleep in children with shunted hydrocephalus: a study of nine cases

Purpose: We present a series of nine patients with early‐onset hydrocephalus who had seizures and continuous spikes and waves during slow sleep (CSWS) associated with neurocognitive and motor deterioration. Methods: Six boys and three girls aged 9–16 years (mean 11.3 years) were studied. [Correction added after online publication 12‐Apr‐2008: Number of girls and boys has been updated.] All patients underwent clinical examinations, electroencephalographic evaluations, neuroradiological imaging and neuropsychological assessment at first examination. Antiepileptic drugs (AEDs) were given in all cases and changed according to clinical and EEG evolution. Results: Onset of epilepsy occurred from age 8 to 60 months (mean 19.6 months and median 14 months) with focal seizures with or without secondary generalized tonic–clonic seizures. Between ages 6 and 13 years (mean 10.4 years and median 8 years), hyperkinesia, aggressiveness, and poor socialization appeared in all nine cases. Reduced attention span, deterioration of language, and temporospatial disorientation were found in three of them. Negative myoclonus was found in two patients. The EEG showed CSWS. Response to change in treatment was good in all patients. None of the patients had relapses, seven of them have remained seizure free, and two continued having sporadic focal motor seizures during 2–5 years (mean 3 years) of follow‐up. Conclusion: In children with early‐onset hydrocephalus, particularly with behavioral and language disturbances and/or motor deterioration, CSWS should be considered. Periodic EEG recordings during sleep should be done in these children. The early identification of this particular electroclinical picture is crucial to start adequate treatment to avoid progressive cognitive deterioration.     

Stimulant therapy and seizure risk in children with ADHD

Stimulants are an effective treatment frequently prescribed for attention-deficit-hyperactivity disorder (ADHD), but they commonly are believed to lower the threshold for seizures. Although several studies have revealed that stimulants do not exacerbate well-controlled epilepsy, there is a paucity of data about seizure risk in nonepileptic children treated with stimulants. Two hundred thirty-four children (179 males, 9.1 +/- 3.6 years of age; 55 females, 9.6 +/- 3.9 years of age) with uncomplicated ADHD received electroencephalograms (EEGs) performed in our institution. Thirty-six patients (15.4%) demonstrated epileptiform abnormalities, and 198 (84.6%) demonstrated normal or nonepileptiform EEGs. Rolandic spikes accounted for 40% of the abnormal EEGs and 60% of those with focal abnormalities. Stimulant therapy was elected by 205 of 234 patients (87.6%). Seizures occurred only in the treated group, in one of 175 patients with a normal EEG (incidence 0.6%, 95% confidence intervals 0%-1.7%) and three of 30 treated patients with epileptiform EEGs (incidence 10%, 95% confidence interval 0%-20.7%). Seizures occurred in two of 12 children (16.7%) with rolandic spikes. These data suggest that a normal EEG can be used to assign children with ADHD to a category of minimal risk for seizure. In contrast, an epileptiform EEG in neurologically normal children with ADHD predicts considerable risk for the eventual occurrence of seizure. The risk, however, is not necessarily attributable to stimulant use.     

Benign Rolandic Epilepsy: High Central and Low Central Subgroups

Summary: The spikes in benign rolandic epilepsy (BRE) typically involve both the central and midtemporal regions as recorded on standard EEG montages, but the seizures are characterized by sensorimotor manifestations that are rarely referable to the temporal lobe. To study this apparent disparity, we evaluated the field distribution of interictal spikes in 33 BRE patients using closely spaced electrodes (AEEGS 1990) arranged over perisylvian cortex. None of the 33 patients showed maximum negativity in the midtemporal regions (T3/T4). Instead, maximum negativity was evident in the high central region (C3/C4) in 10 children (30.3%) and in the low central region (C5/C6) in 23 (69.7%). Hand involvement was significantly frequent (50%) in the high central group, and drooling with oromotor involvement was a distinctive symptom (65.2%) in the low central group. Our findings indicate that the spikes in patients with BRE are exclusively suprasylvian in origin and correlate with two electroclinical subgroups.     

中央-颞部棘波的临床价值

目的 探讨脑电图出现的阵发性中央-颞部棘波的临床价值。方法 观察38例脑电图表现为中央-颞部局限性棘波且并非仅表现为中央沟发作患儿的临床发生经过及脑电图表现的变化。其中28例以局限性运动性发作为主,4例表现为不固定的单侧或双侧发作性头痛;2例于5岁前因发热而出现双侧肢体抽搐;余4例无神经系统病症。患儿首次接受脑电图检查的年龄为4～9岁,随访4～7年。结果38例患儿中,癫痫发作者的临床类型呈多种形式;而无癫痫发作病史者中,既有头痛、头晕主诉者,亦有无神经系统疾病病史者。所有患儿的脑电图均表现为不恒定,多次复查脑电图可出现不同部位的局限性慢波或尖波等内容。结论 中央-颞部局限性棘波并非伴中央-颞部棘波的儿童期良性癫痫的特异性脑电图表现。     

Encephalopathy related to status epilepticus during slow sleep (ESES) as atypical evolution of Panayiotopoulos syndrome: an EEG and neuropsychological study

Aim: We report two patients with Panayiotopoulos syndrome (PS) who developed encephalopathy related to status epilepticus during slow sleep (ESES) at the peak of their clinical course. Methods: Clinical charts and EEG data were reviewed. Results: The patients exhibited nocturnal autonomic seizures and occipital EEG foci, the latter of which later evolved into multifocal EEG foci with synchronous frontopolar and occipital spikes (Fp‐O EEG foci), and finally into continuous spikes‐waves during sleep (CSWS; spike‐wave index >85% based on whole‐night sleep recording) at eight years and seven years of age, respectively. The occipital spikes always preceded frontopolar spikes by 30～50 mseconds based on the analysis of CSWS. Neuropsychological ability, including IQ, deteriorated during the CSWS period in both patients. The autonomic seizures and focal to bilateral tonic‐clonic seizures were initially resistant to antiepileptic drugs (AEDs), and occurred more than 10 times in both patients. However, the seizures and EEG findings gradually resolved, and AEDs were successfully terminated in both patients. Conclusion: PS can progress to ESES if the clinical course exhibits atypical evolution. The initial autonomic symptom of the seizures and interictal Fp‐O EEG foci should be carefully monitored in patients with CSWS or ESES.