Temporal intermittent rhythmic delta activity (TIRDA) in the diagnosis of complex partial epilepsy: sensitivity, specificity and predictive value

Temporal interictal rhythmic delta activity or TIRDA was found in 45 of the 127 recordings of patients with complex partial epilepsy (CPE) referred for both awake and sleep EEG. TIRDA was more abundant during drowsiness and light sleep; it occurred more characteristically as trains of 50-100 microv sinusoidal or saw-toothed 1-4Hz activity, recorded predominantly from anterior temporal regions. When occurring bilaterally and independently, TIRDA varied from side to side. TIRDA is often found in association with anterior temporal spikes or sharp waves (TS) particularly during sleep, as observed in 43 out of 45 EEGs. TIRDA can nevertheless occur as an isolated abnormality, as noted in two sleep and 12 awake study recordings. Because of its high specificity and positive predictive value over a large range of prevalence, TIRDA should be singled out as an accurate interictal indicator of CPE. In patients with isolated TIRDA, the cost of prolonged EEG recording during sleep for the purpose of activating TS has to be weighed against the yield of eventually confirming the obvious.     

Musicogenic epilepsy with ictal single photon emission computed tomography (SPECT): could these cases contribute to our knowledge of music processing?

Musicogenic epilepsy has a strong correlation with the temporal lobe with a right-sided preponderance. We report the case of a 48-year-old woman whose seizures began at the age of 32 years. Her prenatal, natal and childhood histories were unremarkable and her family history was negative for epilepsy. She had typical complex partial seizures with chewing automatisms. Cranial computed tomography, magnetic resonance imaging (MRI) and interictal SPECT showed no abnormality. Interictal EEG showed paroxysmal bitemporal sharp wave discharges predominant on the right side. Ictal EEG showed a combination of high voltage sharp and slow sharp waves and spikes that originated from the right temporal leads and then became generalized. Ictal activity on EEG started 4-5 min after the music stimulus. For the ictal SPECT study, i.v. injection of 20 mCi of HMPAO was administered approximately 30 s after the ictal activity started. Ictal SPECT demonstrated a right anterior and mesial temporal hyperperfusion. These results seem to support the dominant role of the right temporal lobe and the possible relation of mesial temporal structures to the affective content of music in musicogenic epilepsy.     

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.     

外伤性癫痫的临床特征分析及手术治疗

目的 总结外伤性癫痫的临床特征及手术效果。方法 对病人进行神经学检查及EEG、CT、MRI及ECT检查，确定致痫灶后手术治疗，手术中行皮层及深部脑电监测。结果 外伤性癫痫病人32例，年龄10至45岁。临床主要表现有全身强直痉挛性发作、部分性发作、精神运动发作、失神发作。头皮脑电图显示32例病人中30例患者有与损伤部位或对冲部位相符的恒定局限性高波幅尖波、棘波和棘慢波。所有病人均在皮层及深部脑电监测下切除病灶。术后15例已完全停止发作，16例已明显好转，1例无明显改变。结论 外伤性癫痫的临床特点包括局灶性发作多见，癫痫发作形式多样和致痫灶在影像学改变附近。手术切除病灶能获良好效果。     

Clinical and EEG characteristics of benign rolandic epilepsy in Chinese patients

We retrospectively evaluated the clinical and electroencephalogram (EEG) characteristics of benign rolandic epilepsy (BRE) in Chinese children. Two hundred and seventy-six patients with BRE were enrolled in this study. All patients had their first seizure between the ages of 3 and 12 years. 39.5% (109 cases) of patients ceased to have further BRE seizures by the age of 6 years, 93.1% (257 cases) recovered by the age of 12 years and 96.7% (267 cases) recovered by the age of 18 years. Two hundred and twenty-seven patients suffered only simple partial seizures, whereas 49 patients suffered generalized seizures from onset of BRE. The EEG scans of 239 patients showed repetitive diphasic spikes or sharp waves with high amplitude, which were most dominant in the central or centrotemporal areas. The spikes were confined to one hemisphere in 180 patients and occurred bilaterally in 59 patients. Ninety-eight patients were treated with antiepileptic drugs (AEDs): carbamazepine (CBZ) or valproate (VPA). The study showed that, in Chinese children, BRE is remarkably characteristic in its clinical and EEG presentation. Although BRE is usually benign in terms of ease of control with AEDs and spontaneous seizure remission, for those patients with a high frequency of seizures, AEDs should be prescribed positively.     

The EEG Findings in Extratemporal Seizures

Summary: Extratemporal seizures originate from the frontal, central, parietal, occipital, and midline regions of the brain. The scalp EEG can show various types of interictal and ictal discharges consisting of spikes, spike and wave sharp waves, paroxysmal fast activity, or rhythmic activity in the β, α, θ, or δ frequency ranges. The discharges can occur as focal, regional, lateralized, or secondarily generalized discharges. Discharges arising from the frontal region are varied and at times complex. Centro-temporal spikes associated with benign epilepsy of childhood have a characteristic blunt spike and wave appearance. Centro-parietal spikes can occur in children with benign childhood epilepsy or in association with symptomatic epilepsies at any age. Occipital spike discharges have been seen in young children with visual problems, benign occipital epilepsy of childhood, the Sturge-Weber syndrome, and other symptomatic or structural lesions involving the occipital lobe. There may be problems with detection of the source of origin of seizures secondary to the anatomy of the various regions, deep foci, small restricted foci, rapid spread of epileptiform discharges, and contaminating effects of muscle and movement artifact. Depth or intracranial recordings may help in further localization of foci.     

Spectrum of epilepsy in terminal 1p36 deletion syndrome

PURPOSE: Previous reports have summarized the seizures types occurring in 1p36 deletion syndrome. To better define the spectrum of epilepsy, we studied 91 patients (median age 7.8 years) with confirmed 1p36 deletion. METHODS: Based on clinical charts, we retrospectively analyzed the evolution of both the EEG findings and seizures. RESULTS: Epilepsy occurred in 53 patients (58.2%), with onset at a median 2.75 months. First seizures were generalized tonic (8 cases), tonic and clonic (6) or myoclonic (12), simple partial (6), or complex partial (14). Thereafter, 20 patients (21.9%) developed infantile spasms with hypsarrhythmia, at a median age of 5 months. High doses of oral steroids were tried in nine cases, with a prompt remission of seizures in six. Among them, five were seizure-free at the time of evaluation. Conversely, two of three nonresponders to steroids developed severe and refractory epilepsy. At the time of evaluation, 32 patients were seizure-free, from a median age of 1.8 years. Nineteen patients (20.9%) had developed refractory epilepsy with polymorphic seizures, including generalized tonic and tonic-clonic seizures (13) combined with myoclonic seizures (11) and atypical absences (3), atonic seizures (2), or complex partial seizures (3). The EEG showed focal, multifocal or generalized spikes, polyspike, and waves, with poverty of the usual background rhythmic activities. CONCLUSIONS: Early epilepsy is a frequent finding in 1p36 deletion syndrome with infantile spasms as of the most common features that can contribute to a poor clinical outcome. Early diagnosis and management of infantile spasm in this condition is mandatory.     

Postnatal Epilepsy After EEG-Confirmed Neonatal Seizures

We examined infants whose neonatal seizures were confirmed by randomly recorded ictal EEG tracings to determine the types and frequency of postnatal epilepsy (PNE)--unprovoked, recurring postnatal seizures. Perinatal and postnatal clinical and EEG variables were also examined for their relevance to PNE. Forty infants with EEG-documented neonatal seizures of diverse etiologies were studied. The 27 survivors were followed for a mean of 31 months. PNE developed in 56% (15 of 27) of the cohort. The first seizure appeared at a mean-corrected age of 12.7 months and occurred despite ongoing antiepileptic medication in 60% (9 of 15) of the group. Seizures were classified as infantile spasms or minor motor (7 patients), complex partial (4 patients), or generalized tonic-clonic (4 patients). Perinatal variables that significantly correlated with PNE included the presence of coma but not the age at seizure onset, the estimated gestational age, or Apgar scores. PNE occurred in 68% (13 of 19) of patients with moderately or markedly abnormal EEG backgrounds but in only 25% (2 of 8) without (p = 0.035). There was a strong trend for PNE to develop in patients with greater than 10 electrographic seizures per hour but in only 45% (9 of 20) of infants with fewer seizures (p = 0.058). Several postnatal variables were significantly related to PNE--the presence of cerebral palsy (CP), mental retardation (MR), CP with MR, and follow-up EEGs. PNE occurred in only 27% (3 of 11) of patients without spikes or sharp waves on postnatal EEGs performed at age 3 months but in 100% (3 of 3) of patients with spikes or sharp waves (p = 0.022).(ABSTRACT TRUNCATED AT 250 WORDS)     

Absence seizures with evolution into generalized tonic-clonic activity: clinical and EEG features

PURPOSE: To report the clinical and electrographic features of absence seizures evolving into generalized tonic-clonic (GTC) activity in six patients with idiopathic generalized epilepsy. METHODS: All patients were referred for evaluation of refractory seizures and underwent video-EEG monitoring after discontinuation of their antiepileptic drugs (AEDs). We analyzed the video-EEG recordings for seizure semiology as well as ictal and interictal activity. We also reviewed the initial clinical data in all patients. RESULTS: All patients were women, with a mean age of 27 years (range, 14-43 years). The mean age at seizure onset was 12 years (range, 5-15 years). Family history was positive for epilepsy in four patients. All patients had recorded seizures with an onset that was characteristic of generalized absence clinically and electrographically, with evolution into GTC activity. The EEG onset was with generalized 2.5-to 5-Hz spike-and-wave discharges, with evolution into faster rhythmic activity. Interictal EEG recordings showed generalized 2-to 5-Hz spike-and-wave discharges. All had normal background activity. All patients were treated with divalproex monotherapy. Five patients have been seizure free, and one had a single breakthrough GTC seizure during a follow-up period of 12-36 months. CONCLUSIONS: GTC activity may evolve from typical absence seizures. This seizure type should be included in the International Classification of Seizures. Its recognition and distinction from complex partial seizures with secondary generalization are important for appropriate therapy.     

Electroencephalographic findings in children with cerebral palsy: a study of 151 patients

EEG abnormalities were studied in 151 patients (79 boys, 72 girls age range 0.4-13 years) with cerebral palsy (CP). They all had standardised EEG recordings, which were read by the same electroencephalographer. Eighty-one children had seizures and 70 were seizure-free. The EEG abnormalities in the seizure group included slow waves in 36 patients (generalised asynchronous in 33 and generalised synchronous in 3); amplitude abnormalities in 2 (focal in 1, generalised 1); epileptiform activity (including isolated sharp waves, isolated spikes, and spike-wave and polyspike-wave complexes) was seen in 66 (focal in 12; generalised in 48 and multifocal in 6). Hypsarrythmia was found in 4 and burst suppression in 1. Only 6 recordings were normal giving an overall percentage of abnormality of 92.6%. Of the CP patients without seizures, 28 (40%) showed generalised asynchronous slow waves; epileptiform activity was found in 27 (focal in 2, generalised in 23 and multifocal in 2); 3 subjects showed hypsarrythmia and 24 recordings were normal. The overall percentage of abnormality in this group was 76%. Cerebral palsy in children, regardless of its cause may be associated with generalised focal EEG abnormalities. This may reflect heterogeneity of the neural-generator in the underlying disease process.     

Clinical,EEG, and positron emission tomography features of childhood-onset epilepsy with localized cortical dysplasia detected by magnetic resonance imaging

We studied clinical, EEG, and positron emission tomography (PET) findings in 18 patients with childhood-onset epilepsy with localized cortical dysplasia detected by magnetic resonance imaging. The age at onset of epilepsy was prior to 6 months of age in about half of the patients; the oldest patient was 7 years. Unilateral dysplastic lesions were more frequently associated with partial epilepsy, whereas bilateral dysplasia was associated more with generalized epilepsy. Patients with partial epilepsy had secondarily generalized seizures more often at the onset. Two patients with partial epilepsy presented generalized seizures transiently: undetermined epilepsy with infantile spasms triggered by partial seizures in one and epilepsy with continuous spike waves during slow-wave sleep in the other. The size of the lesion was not correlated with seizure outcome but was significantly correlated with mental outcome. The PET abnormality of glucose metabolism usually corresponded to the areas of cortical dysplasia and EEG focus, but the correspondence was better in partial epilepsy than generalized epilepsy.     

Nonconvulsive Status Epilepticus in Adults: Thirty-Two Consecutive Patients from a General Hospital Population

We studied all adult patients who between 1984 and 1989 were initially diagnosed at our hospital as having nonconvulsive status epilepticus. Thirty-two patients fulfilled the criteria, which included ictal EEG recordings. The annual incidence was 1.5 in 100,000 inhabitants. The median age at onset of status was 51 years. Ten patients had status as their first epileptic manifestation, but most patients had a previous history of epilepsy. Median duration of epilepsy at onset of status was 4 years. Fourteen patients had focal ictal seizure activity on EEG and thus met the criteria for complex partial status. Eighteen patients had generalized seizure activity on EEG, but only 6 of these had a history of absence epilepsy or juvenile myoclonic epilepsy. None had Lennox-Gastaut syndrome. The clinical features of status in the remaining 12 patients were in some respects similar to those of the patients with complex partial status. We hypothesize that the EEG seizure activity in these patients may have been generalized from an initial focus.     

Ganglioglioma and Intractable Epilepsy: Clinical and Neurophysiologic Features and Predictors of Outcome After Surgery

Summary: Purpose: To review the clinical, neurophysiologic, and radiological data of patients with ganglioglioma who had undergone evaluation and surgery in our Epilepsy Program.
Methods: The medical and neurophysiologic records of 38 patients with intractable epilepsy and ganglioglioma were re- viewed. Data underwent statistical analysis.
Results: There were 28 temporal and 10 extratemporal resections, with a mean age at seizure onset of 10.5 years and mean age at surgery of 22 years. Five tumor resections performed earlier were recorded. Twenty-nine patients had auras and 20 had secondarily generalized seizures. All 28 patients with temporal tumor had complex partial seizures. Preoperative MRI demonstrated the tumor in 36 of 36 patients: 17 of 29 demonstrated gadolinium enhancement, and 17 of 36 had mass effect. Scalp interictal sharp waves were present in 32 patients, and in 15 they were multiregional. In two patients, scalp EEG seizure onset was from the hemisphere contralateral to the tumor. Postoperatively, 79% of patients (30 of 38) were seizure-free (Engel's class I) at 6 months, 72% at 1 year (26 of 36), and 63% at 2 years (20 of 32). Excellent outcome was associated with a lower age at operation (p = 0.008), shorter duration of epilepsy (p = <0.01), absence of generalized seizures (p = <0.01), and no epileptiform discharges on a postoperative EEG (p = 0.01).
Conclusions: Good surgical outcome is expected in patients with ganglioglioma despite years of medically resistant seizures. Good outcome may be achieved despite EEG findings that may conflict with tumor location, and is more likely when surgery is performed relatively soon after epilepsy onset.     

Partial epilepsy with pericentral spikes: a new familial epilepsy syndrome with evidence for linkage to chromosome 4p15

The genetic analysis of simple Mendelian epilepsies remains a key strategy in advancing our understanding of epilepsy. In this article, we describe a new family epilepsy syndrome, partial epilepsy with pericentral spikes, which we map to chromosome 4p15. We distinguish it clinically, electrophysiologically, and genetically from previously described Mendelian epilepsies. The family described is a large Brazilian kindred of Portuguese extraction in which affected family members manifest a variety of seizure types, including hemiclonic, hemitonic, generalized tonic-clonic, simple partial (stereotyped episodes of epigastric pain), and complex partial seizures consistent with temporal lobe epilepsy. The syndrome is benign, either requiring no treatment or responding to a single antiepileptic medication. Seizure onset is in the first or second decades of life, with seizures in individuals up to the age of 71 years and documented encephalogram changes up to the age of 30 years. A key feature of partial epilepsy with pericentral spikes is a characteristic encephalogram abnormality of spikes or sharp waves in the pericentral region (centroparietal, centrofrontal, or centrotemporal). This distinctive encephalogram abnormality of pericentral spikes unites these several seizure types into a discrete family epilepsy syndrome. As with other familial epilepsies, the inherited nature of this new syndrome may be overlooked because of the variability in penetrance and seizure types among affected family members.     

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.     

A case of parietal lobe epilepsy with ictal laughter

We report here about an 8-year-old boy with parietal lobe epilepsy (PLE) and ictal laughter. At the age of 6, he began to experience drop seizures, followed by sensory fits. Interictal EEG showed frequent spikes at C3, C4, P3 and Cz. Despite treatment with antiepileptic drugs, he often fell down in seizures after feeling abnormal sensations in the right shoulder. On ictal video EEG at the age of 7 years, (1) he became motionless and complained of fear and pain in the right hand, (2) he had clonic seizures of the right upper limb and fell down to his left, (3) he laughed though he did not feel funny. Ictal EEG showed spikes which originated in Pz and then were generalized. In many of the previously reported cases, ictal laughter is associated with hypothalamic hamartomas, infantile spasms,. complex partial seizures of frontal, temporal, or parietal origin. We diagnosed the present case as having PLE. However, other localization could not be roled out because the spikes were generalized quickly. To date, there are two reported cases of ictal laughter with PLE, but ictal EEG is lacking in these patients. Ictal laughter is rare in non-lesional cryptogenic PLE, but it may imply PLE's pathogenesis.     

Myoclonic encephalopathy in the CDKL5 gene mutation.

OBJECTIVE: Epilepsy with mutation of the CDKL5 gene causes early seizures and is a variant of Rett syndrome (MIM (312750), which is reported typically as infantile spasms. The purpose of this study was to analyze the epileptic histories and EEGs of patients with the CDKL5 mutation. METHODS: We reviewed the epilepsy histories and electroclinical analyses of three girls aged 9.5, 7.4, and 9.4 years, each with a mutation of the CDKL5 gene. RESULTS: We revealed the presence of an encephalopathy that started by 1.5 months of age. At first, seizures involved tonic spasms or complex partial seizures, and were complicated by the later appearance of complex partial, tonic, and unexpectedly, myoclonic seizures. This form of epilepsy was drug resistant. Routine and prolonged video EEGs both displayed a homogeneous electroclinical pattern consisting of (a) unique background with diffuse high voltage sharp waves of 6-7 Hz, and absence of the typical rhythmic frontal-central theta activity present in Rett syndrome; (b) unique awake and sleep background, with diffuse, high voltage, continuous sharp waves with multifocal and diffuse spikes; (c) rhythmic, diffuse, 15 Hz activity accompanied clinically by tonic seizures; (d) intercritical pattern with pseudoperiodic, diffuse, sharp waves or pseudoperiodic, diffuse spike and polyspike or wave discharges; and (e) diffuse, spike, polyspike and wave discharges accompanied by massive or focal myoclonias or both. CONCLUSIONS: Patients with the CDKL5 mutation have an early onset, epileptic encephalopathy in infancy that evolves into myoclonic seizures in childhood with a unique EEG pattern. SIGNIFICANCE: Recognizing this type of encephalopathy could be useful in prompting clinicians to proceed further with their diagnostic work in patients not fitting the criteria of classical Rett syndrome.     

Children with Focal Sharp Waves: Clinical and Genetic Aspects

Summary: Purpose: To investigate the spectrum of clinical manifestations in children with benign focal sharp waves in the EEG to gain further insight into the genetic background of clinical and EEG symptomatology in a family study. Methods: All 147 children (134 with seizures, 13 without) met the following inclusion criteria: (a) at least one EEG with focal sharp waves characteristic of benign partial epilepsies, and (b) at least 1 sibling investigated by EEG. The families were questioned orally or in writing regarding the occurrence of seizures. Patients’ records were evaluated by a standardized scheme. Results: The following types of seizures occurred: febrile convulsions (FC), afebrile generalized tonic-clonic seizures (GTCS), simple and (rarely) complex partial seizures; and rolandic seizures in the strict sense. Neonatal seizures were overrepresented (6%); there were no indications of lesional causes. FC occurred in 38 children (26%). As compared with unselected cases of FC, complex symptoms were overrepresented. Family data suggested a maternal preponderance in the transmission of FC liability. Affected relatives of FC probands manifested FC more often than did relatives of probands without FC. Families of 32 patients with typical rolandic seizures (24% of the 134 probands with seizures) showed no aggregation of rolandic epilepsy, but did show variable seizure types. In the entire sample, EEG investigations showed focal sharp waves in 11% of siblings aged 2–10 years. No relation existed between clinical symptomatology and sharp wave findings in siblings. In 66% of probands, the EEG disclosed generalized genetic patterns. Siblings with generalized spike-waves (sw) and/or theta rhythm had focal sharp waves more often than those without sw andor theta rhythm. Conclusions: The phenotypic expression of the genetic anomaly underlying focal sharp waves shows considerable variability. The clinical and EEG findings are in agreement with a multifactorial pathogenesis of epilepsies with “benign” focal epileptiform sharp waves.     

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.     

Atypical "benign" partial epilepsy of childhood or pseudo-lennox syndrome. Part II: family study

Atypical benign partial epilepsy of childhood (ABPE = Pseudo-Lennox syndrome) shows semiologic parallels to Lennox-Gastaut syndrome, however--besides the lack of tonic seizures--it has an entirely different etiology and prognosis. Recently Hahn et al [17] investigated the long-term evolution of 43 cases with ABPE. Symptomatology, EEG findings, and course were found to overlap with Rolandic epilepsy, Landau-Kleffner syndrome and ESES. The incidence of seizures in relatives was determined in the whole series investigated by Hahn et al [17]. Five of 56 siblings suffered from seizures (3 Rolandic seizures; one febrile convulsions; one unclassified). Three fathers reported grand mal. In 29 families of the series of Hahn et al EEG recordings were performed: 22 brothers, 19 sisters and 16 pairs of parents. In 29% of the siblings a sharp wave focus was demonstrable. The rate rose to 40% when only siblings investigated at the age of maximum expression (3 to 10 years) were considered. Sharp wave foci were mostly multifocal and indistinguishable from those observed in siblings of children with Rolandic epilepsy. Photoparoxysmal response and generalized spikes and waves during rest and hyperventilation were also found to be significantly elevated (26% and 13% respectively). We conclude that ABPE is a subgroup of idiopathic partial epilepsy of childhood (representing a less benign part of a spectrum) that has to be ranked in a continuum with Rolandic epilepsy. The different clinical phenotype might be caused by a higher expressivity of the identical genetic trait, possibly facilitated by other genetic or acquired factors. Genetic heterogeneity represents another possibility.