Intractable Epilepsy and Disturbed Visuomotor Performance

Abstract: The factors relevant to intractability, types of epilepsy and impairment of dexterity in patients with intractable epilepsy were studied independently in different groups of patients. The factors relevant to intractable epilepsy that were disclosed in 202 patients, who required hospitalization more than twice, were as follows: strong seizure propensity, neuropsychiatric disorders including mental deterioration of various degrees, ataxia, personality changes and psychotic episodes, intolerance to antiepileptic drugs due to acute or chronic side effects, idiosyncrasy and internal disorders, self-induced seizure, misdiagnosis and mistreatment, and breakdown of family care of patients. The types of epilepsy in 224 patients with intractable epilepsy whose seizures were not adequately controlled and recurred on a monthly basis in spite of hospitalization were classified as follows: 101 patients with localization-related epilepsies or syndromes, 106 with generalized epilepsies or syndromes, 16 with undetermined epilepsies or syndromes and one with specific syndrome. In regard to partial epilepsies, frontal lobe epilepsy and partial epilepsy with multiple foci were at least partially intractable as temporal lobe epilepsy. With respect to intractable generalized epilepsies, miscellaneous symptomatic generalized epilepsies like intractable grand mal epilepsy with progressive mental retardation in childhood were as important as Lennox-Gastaut or West syndrome though it defies classification into any established syndromes. The proposed International Classification of Epileptic Syndromes and Epilepsies¹ was found adequate for analysis of intractable epilepsy. The disturbance of fine motor performance found in 84 patients who participated in occupational therapy was investigated by test programs comprising nine subbatteries. Disturbance of manual dexterity was found in about 80% of the patients and that of the visuomotor performance in about 90%. These impairments seemed to be responsible for the patients failing to be properly employed. It is necessary that in addition to persisting seizures, neuropsychological impairment must be identified for better social care for patients with intractable epilepsy.     

Epilepsies of neonatal onset: seizure type and evolution

Most neonatal seizures are occasional seizures and not true epilepsy. This study investigates seizure types of true neonatal epilepsies and their evolution with development. Seventy-five children with epilepsies of onset within 1 month of life, who were examined between 1970 and 1995, and whose seizure types could be confirmed with ictal EEG recordings, were studied. The patients were followed up for a minimum of 3 years and the evolution of epileptic syndromes was investigated. Sixty-three (84%) of 75 patients had partial seizures, while nine had generalized seizures, and only three had both generalized and partial seizures. Twenty-three of 24 neonates with benign familial or non-familial neonatal convulsions presented with partial seizures; these syndromes should not necessarily be categorized into generalized epilepsy as they are in the present International Classification. Age-dependent changes were a common feature of symptomatic neonatal epilepsies. Eighteen (41%) of 44 patients with symptomatic epilepsies of neonatal onset developed West syndrome in infancy. Fifteen (83%) of these 18 patients presented with symptomatic localization-related epilepsy in the neonatal period. In seven of these 15 patients, West syndrome was followed by localization-related epilepsy. Symptomatic localization-related epilepsy with transient West syndrome in infancy is another type of age-dependent epileptic syndrome.     

Recent advance in epileptology]

Nowadays epilepsy is classified according to the international classification of epilepsies, epileptic syndromes and related disorders proposed by International League Against Epilepsy (ILAE, 1989). Among these epilepsies and epileptic syndromes, I detailed the clinical symptomatology and the clinical significance of benign familial neonatal convulsions, juvenile myoclonic epilepsy, epilepsy with myoclonic absence, mesial temporal lobe epilepsy and chronic progressive epilepsia partialis continua of childhood. Of 218 patients with temporal lobe resection who were followed for over 2 years, 172 patients or 79% were seizure free according to Engel's criteria of seizure outcome after surgical treatment. Of 66 patients with extra-temporal lobe epilepsy, 43 patients or 65% were seizure free. From the postsurgical outcome I talked about the need of strict presurgical evaluation to have the good postsurgical outcome. The neuropathology of surgically resected 278 specimens of temporal lobe epilepsy was mesial temporal sclerosis in 184, neuroepithelial tumors in 66, cortical malformation in 11, vascular anomaly in 13, and others in 4. Thirty-nine of resected 66 specimens of extra-temporal lobe epilepsies were cortical malformation. I presented the recent progress of genetic study in epilepsies and stressed the importance of correct diagnosis of epilepsy for genetic study.     

Pediatric Epilepsy Syndromes: An Update and Critical Review

Summary: Epilepsy syndromes occupy an important position in the current nosology of the epilepsies, describing and classifying seizure disorders with shared clinical and EEG features. Increasingly, this schema is being refined as new information becomes available and our understanding of etiology and presentation of each syndrome widens. Advances in neuroimaging and neurogenetics have been particularly important and are likely to fundamentally change our concepts of syndrome classification. At present, the International League Against Epilepsy classification of epilepsy syndromes according to presumed localization (partial, generalized, undetermined) and etiology (idiopathic, cryptogenic, symptomatic). In clinical practice, it is often useful to conceptualize epilepsy syndromes according to their usual age at presentation, which greatly facilitates syndrome identification in new patients and recognizes the age-related expression of many childhood epilepsies. Definitional problems exist for many pediatric epilepsy syndromes, particularly the epileptic encephalopathies of early infancy, the benign epilepsies of infancy and childhood, the myoclonic epilepsies of infancy and early childhood, and the idiopathic generalized epilepsies of childhood and adolescence. It is likely that further input from the fields of molecular genetics and neuroimaging will enable the classification of epilepsies to become more etiologically oriented and disease specific.     

Cerebral and cerebellar volume reduction in children with intractable epilepsy

PURPOSE: Adult epilepsy studies have demonstrated cerebral and cerebellar volume reduction beyond the epileptogenic zone, correlating this with an inferior surgical outcome. We determined whether brain volumes were reduced in childhood epilepsy and the significance of this. METHODS: Cerebral, cerebellar, and hippocampal volumes were measured by quantitative magnetic resonance imaging on 112 children (ages 4-18) with epilepsy syndrome determined by video-EEG telemetry. Eighty-seven had partial epilepsy and 25 had generalized epilepsy or indeterminate syndrome. Normative volumes were obtained from 44 child controls from the community. RESULTS: A significant reduction in cerebral (12.6%) and cerebellar (7.9%) volume was present in the epilepsy group compared with controls. Analysis of subgroups revealed that cerebral volume was significantly decreased in frontal lobe and nonlocalized partial epilepsies. The mean hippocampal ratio of 0.73 for mesial temporal lobe epilepsy was significantly less than for all other syndromes and controls. There was no difference in the rate of hippocampal volume reduction between syndromes. There was a significant correlation between IQ and cerebral and cerebellar volume, but not duration or age of onset of epilepsy. CONCLUSIONS: Cerebral and cerebellar volume reduction is common in intractable epilepsy syndromes of childhood. These cross-sectional data suggest that brain volume reduction is present at epilepsy onset and is not a result of intractable seizures. Hippocampal asymmetry is more sensitive than volume reduction as a marker for mesial temporal lobe epilepsy, but neither measure is specific.     

癫癎及癫癎综合征国际分类(1989年)的临床应用分析

目的探讨各类癫和癫综合征在就诊人群中的分布,以利于癫患者的诊治。方法按国际抗癫联盟(ILAE)1989年推荐的癫和癫综合征的分类方案对门诊患者进行调查。结果1191例患者中,部分性癫和综合征766例(64·3%),全面性癫和综合征240例(20·2%),不能确定为部分性或全面性的癫及综合征79例(6·7%),特殊综合征16例(1·3%),其他非癫疾患90例(7·6%)。在部分性癫和综合征中,症状性者占了绝大多数,而全面性癫和综合征中,特发性者占绝大多数。结论正确地进行癫分类对临床有重要的指导意义,各种癫综合征的分布是有规律可循的。     

Classification of epilepsies and epileptic syndromes using the 1989 International League against epilepsy classification: A hospital-based study of 1,250 patients in a developing country

The 1989 International Classification of Epilepsies and Epileptic Syndromes (ICE) of the International League Against Epilepsy was used to study the distribution of epilepsies and epileptic sundromes in 1,250 patients attending an Epilepsy Clinic in Sri Lanka. Of this largely adult population, 917 (73.4%) were classified as having localization-related epilepsy, 228 (18.2%) as having generalized epilepsy, and 104 (8.3%) as having epilepsy undetermined as to whether focal or generalized. Only one case was termed special syndromes because the definition of epilepsy excluded situation-related seizures. Of the localization-related epilepsies, the majority (82.9%) were cryptogenic and 14.5% were symptomatic. Of them, approximately one third were temporal lobe epilepsies; about half the cases could not be localized to a specific lobe of the brain. The generalized category consisted of 214 (93.9%) idiopathic and 14 (6.1%) symptomatic epilepsies. Juvenile myoclonic epilepsy (JME) was the most common idiopathic generalized epilepsy (115 cases, 50.4%). Epilepsies with specific modes of seizure precipitation accounted for 6.8% (85 cases) of the total series. All 85 cases were localization-related epilepsies; 76 had eating epilepsy (EE), and 9 had self-induced epilepsy (SIE). Although some overlap occurred between certain subcategories and specific localization of localization-related epilepsies was difficult, the 1989 ICE was relevant and applicable in a clinical setting with limited investigatory facilities.     

Sleep and epilepsy

This review considers the effect of sleep on seizures and interictal electroencephalogram (EEG) paroxysmal activities (PAs), as classified by the International League Against Epilepsy criteria. No type of seizure is, per se, specifically linked with non-rapid eye movement (NREM) or rapid eye movement (REM) sleep. However, in some syndromes, seizures are more frequent in slow wave sleep (SWS) [partial motor or generalized seizure in benign epilepsy with centro-temporal spikes (BECTS), frontal seizures in idiopathic familial or not familial frontal lobe epilepsy and generalized tonic seizure in secondary generalized epilepsy are increased by SWS]. Conversely myoclonia and grand mal seizures are associated with awakening in some forms of generalized idiopathic epilepsy. There is a mean increase in PAs during SWS in generalized and in partial epilepsies on the whole. However, precise analysis shows that in partial cryptogenic or symptomatic epilepsy and, most likely, in the majority of generalized idiopathic epileptic syndromes about 20% of patients have an increase in PA density during SWS, 20% experience an increase in waking, 50% have very few PAs and in 10% there is no significant difference between sleep and waking. BECTS, however, exhibits a definite increase in sleep PA increase and in juvenile myoclonic epilepsy an increase in PAs during the intra-night awakening is reported. There are at least three syndromes, which cause a huge increase in PAs during sleep: the Landau-Kleffner syndrome and the syndromes of continuous focal or generalized spike-waves during SWS. Their physiopathology and neuropsychological consequences are discussed. Neurophysiological animal data are also reported highlighting the relationships between slow sleep oscillations and the generation of spike waves. A biochemical review is also presented.     

Idiopathic Generalized Epilepsies Imitating Focal Epilepsies

Summary:  Classification of epileptic seizures and epilepsy syndromes as either focal or generalized is a fundamental and early part in the diagnostic process and is generally fairly easily accomplished. However, in patients with idiopathic generalized epilepsies, seizure and EEG features may suggest, particularly to the unwary, the occurrence of focal rather than generalized seizures. Misinterpretation of typical absence seizures as focal seizures, especially as temporal lobe seizures and of myoclonic seizures as focal clonic seizures, is a relatively common error and focal features during generalized tonic–clonic seizures may also be quite common. Sequences of seizures in idiopathic generalized epilepsies (such as absences or jerks followed by generalized tonic–clonic seizures) may also cause confusion. Versive and circling seizures are seizure types whose ictal semiology is clearly focal; nevertheless such seizures are described in idiopathic generalized epilepsies accompanied by generalized EEG discharges. The occurrence of focal EEG abnormalities in certain idiopathic generalized epilepsy syndromes is common. This is best known in juvenile myoclonic epilepsy.     

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.     

Prevalence, Classification, and Severity of Epilepsy and Epileptic Syndromes in Children

Summary: Purpose: To determine the point prevalence of active childhood epilepsy in a defined area and evaluate the usefulness of ILAE classification of seizures, and epilepsies/syndromes with special interest in severe epilepsies. Methods: By using the latest ILAE International Classification of Epileptic Seizures (ICES, 1981) and Epilepsies and Epileptic Syndromes (ICE, 1989), we determined the age- and sex-specific prevalence rates of epilepsy, type of seizures, epilepsies, and recognizable epileptic syndromes, as well as the proportion of severe cases in each seizure/epilepsy/syndrome category in all children 0–15 years of age from a geographically defined area in Finland. All medical records, neurophysiological recordings and available clinical data were reviewed retrospectively. Results: Point prevalence of active epilepsy on December 12, 1992 was 3.94 per 1,000. According to ICESDCE, we were able to classify 96% of seizures and 90% of epilepsies and syndromes. Generalized seizure and epilepsy/syndrome types were more prevalent in children 0–6 years of age and partial/ localization-related in children 6–15 years of age. Epilepsy was intractable in 17% of all cases and correlated significantly with symptomatic etiology and early onset of epilepsy, as well as with additional neuroimpairments. Conclusions: A considerable number of cases fell into the nonspecific categories of ICE, which limits the value of present epilepsy/syndrome classification in terms of prognosis, prediction, and indication for special investigations in individual cases. A number of intractable cases was relatively low, indicating good prognosis in many childhood epilepsies, especially when additional neuroimpairments are absent.     

Factors that preclude the successful use of monopharmacy in the medical treatment of patients with epilepsy

A neurologist conducted research efforts for more than 12 years as a step toward the establishment of monotherapy for epilepsy. Of 406 patients with epilepsy, seizures could be controlled for more than one year in 72% and for more than 3 years in 54%. Monotherapy was given to 57% of all the patients with a success rate of 54%. Factors that were found likely to interfere with a reduction in antiepileptic drug therapy to one drug modality included: symptomatic etiology, prolonged duration of illness, low age at onset and secondary generalized epilepsy with a large number of seizures in combination, for generalized epilepsies; symptomatic etiology, prolonged duration of illness, low age at onset, other than occipital lobe origin, complex partial seizure with secondary generalization, temporal lobe epilepsy with associated automatisms, elementary sensorimotor seizure and high frequency of seizures, for partial epilepsies. In addition to these factors relevant to the nature of epilepsies, other factors apparently unrelated to the disease process, e.g., liability of polypharmacy to produce side effects precluding dosage elevation, patient's rejection to reduce drug and the inability of a physician to treat the patient properly for want of information, were also recognized to exist.     

Symptomatic generalized epilepsy associated with an inverted duplication of chromosome 15.

An inverted duplication of chromosome 15 (inv dup[15] chromosome) is the most common supernumerary marker chromosome in humans. Inv dup(15) chromosomes are commonly associated with mental retardation, epilepsy, behavioral problems and structural malformations. Though epilepsies associated with inv dup(15) chromosomes are often intractable, there have been very few reports regarding the seizure manifestations or types. We report a patient with severe mental retardation and intractable epilepsy, associated with an inv dup(15) chromosome. The seizures recorded with EEG-VTR monitoring were axial and generalized tonic seizures, and our case was diagnosed as symptomatic generalized epilepsy. Molecular and cytogenetic analysis showed an inv dup(15) chromosome containing the Prader-Willi syndrome/Angelman syndrome region mapped within bands 15q 11-q13.     

Factors That Preclude the Successful Use of Monopharmacy in the Medical Treatment of Patients with Epilepsy

Abstract: A neurologist conducted research efforts for more than 12 years as a step toward the establishment of monotherapy for epilepsy. Of 406 patients with epilepsy, seizures could be controlled for more than one year in 72% and for more than 3 years in 54%. Monotherapy was given to 57% of all the patients with a success rate of 54%. Factors that were found likely to interfere with a reduction in antiepileptic drug therapy to one drug modality included: symptomatic etiology, prolonged duration of illness, low age at onset and secondary generalized epilepsy with a large number of seizures in combination, for generalized epilepsies; symptomatic etiology, prolonged duration of illness, low age at onset, other than occipital lobe origin, complex partial seizure with secondary generalization, temporal lobe epilepsy with associated automatisms, elementary sensorimotor seizure and high frequency of seizures, for partial epilepsies. In addition to these factors relevant to the nature of epilepsies, other factors apparently unrelated to the disease process, e.g., liability of polypharmacy to produce side effects precluding dosage elevation, patient's rejection to reduce drug and the inability of a physician to treat the patient properly for want of information, were also recognized to exist.     

Classification criteria of epileptic seizures and syndromes

Care must be exercised not to intermingle with classification of seizures and classification of epilepsies in an inconsistent fashion. Criteria for each class must be defined as clearly as possible, and these criteria must be those which are necessary for classifying any given case. The international classification first proposed by the ILAE in 1970 was an attempt to distinguish seizures from epilepsies; the seizure types defined in the 2001 diagnostic scheme are conceptually akin to the syndromes in the 1989 classification in the sense that they imply etiological, therapeutic, and prognostic significance. However, there exists no room in the new diagnostic scheme to accommodate electro-clinical seizure types, which have been used for more than three decades. The concept of epileptic syndrome in the 1989 classifications seems to have been changed to epileptic seizure type in the 2001 diagnostic scheme, and seizure type of the latter virtually becomes synonymous with epileptic syndrome of the former. In the 2001 diagnostic scheme, seizure type can be used to supplement syndrome, and can stand alone when syndrome diagnosis cannot be made. In other words, seizure types may replace syndromes, or vice versa. We should not return to an era prior to 1970 where no distinction exists between epilepsies and seizures. In a cohort of patients with active epilepsy, to what extent is syndrome classification applicable? In 300 consecutive patients hospitalized in a tertiary center, syndromic diagnosis was applicable to only 61%. Similarly, another 100 consecutive patients, classification of epilepsy was possible but not defined as a syndrome in 32% patients, according to the 1989 classification. The 1989 syndrome classification assigned in each category "other epilepsies not defined as a syndrome." These epilepsies are diagnosed only dichotomously; idiopathic focal or generalized, symptomatic focal or generalized, or undetermined whether focal or generalized. In other words, even if we could complete a list to include all the new syndromes that may exist, it is very unlikely that it would cover all epilepsies.     

Epilepsy syndromes associated with hypothalamic hamartomas.

PURPOSE: Hypothalamic hamartoma (HH) related epilepsy presents with gelastic seizures (GS), other seizure types and cognitive deterioration. Although seizure origin in GS has been well established, non-GS are poorly characterized. Their relationship with the HH and cognitive deterioration remains poorly understood. We analyzed seizure type, spread pattern in non-GS and their relationship with the epileptic syndrome in HH. METHODS: We documented all current seizure types in six adult patients with HH-epilepsy with video-EEG monitoring, characterized clinical-electrographic features of gelastic and non-gelastic seizures and correlated these findings with cognitive profile, as well as MRI and ictal SPECT data. RESULTS: Only four seizure types were seen: GS, complex partial (CPS), tonic seizures (TS) and secondarily generalized tonic-clonic seizures (sGTC). An individual patient presented either CPS or TS, but not both. GS progressed to CPS or TS, but not both. Ictal patterns in GS/TS and in GS/CPS overlapped, suggesting ictal spread from the HH to other cortical regions. Ictal SPECT patterns also showed GS/TS overlap. Patients with GS-CPS presented a more benign profile with preserved cognition and clinical-EEG features of temporal lobe epilepsy. Patients with GS-TS had clinical-EEG features of symptomatic generalized epilepsy, including mental deterioration. CONCLUSIONS: Video-EEG and ictal SPECT findings suggest that all seizures in HH-related epilepsy originate in the HH, with two clinical epilepsy syndromes: one resembling temporal lobe epilepsy and a more catastrophic syndrome, with features of a symptomatic generalized epilepsy. The epilepsy syndrome may be determined by HH size or by seizure spread pattern.     

Hyperventilation revisited: physiological effects and efficacy on focal seizure activation in the era of video-EEG monitoring

PURPOSE: Hyperventilation is an activation method that provokes physiological slowing of brain rhythms, interictal discharges, and seizures, especially in generalized idiopathic epilepsies. In this study we assessed its effectiveness in inducing focal seizures during video-EEG monitoring. METHODS: We analyzed the effects of hyperventilation (HV) during video-EEG monitoring (video-EEG) of patients with medically intractable focal epilepsies. We excluded children younger than 10 years, mentally retarded patients, and individuals with frequent seizures. RESULTS: We analyzed 97 patients; 24 had positive seizure activation (PSA), and 73 had negative seizure activation (NSA). No differences were found between groups regarding sex, age, age at epilepsy onset, duration of epilepsy, frequency of seizures, and etiology. Temporal lobe epilepsies were significantly more activated than frontal lobe epilepsies. Spontaneous and activated seizures did not differ in terms of their clinical characteristics, and the activation did not affect the performance of ictal single-photon emission computed tomography (SPECT). CONCLUSIONS: HV is a safe and effective method of seizure activation during monitoring. It does not modify any of the characteristics of the seizures and allows the obtaining of valuable ictal SPECTs. This observation is clinically relevant and suggests the effectiveness and the potential of HV in shortening the presurgical evaluation, especially of temporal lobe epilepsy patients, consequently reducing its costs and increasing the number of candidates for epilepsy surgery.     

Long-Term Follow-Up of Childhood Epilepsy Associated with Tuberous Sclerosis

Summary: Purpose: To study the clinical and electroencephalographic (EEG) characteristics of patients whose epilepsy is associated with tuberous sclerosis, with special reference to their clinical course.
Methods: We investigated the electroclinical and radiologic features of 38 patients with epilepsy associated with tuberous sclerosis.
Results: Eleven patients showed only generalized epilepsy, and 10 showed only localization-related epilepsy throughout their clinical course. Among the other 17 cases, the nature of the epilepsy changed between generalized and localization-related epilepsies during the clinical course. A shift from generalized to localization-related epilepsies was more common than the reverse. Seventeen had West syndrome (WS), three had Lennox-Gastaut syndrome (LGS), and eight had epilepsies that evolved from WS to LGS. Tonic spasms, mostly in series, were seen in all 28 patients with generalized epilepsy. Eleven of the 28 patients had partial seizures and tonic spasms in the same period. Six of them showed "simultaneous seizures," consisting of tonic spasms in series and a partial seizure. Partial seizures were the main seizure type in 27 patients with localization-related epilepsy, but three of them also showed tonic spasms that included "simultaneous seizures." Ictal EEGs revealed multiple active foci in the same period that could shift during the clinical course. Neither the location nor number of tubers was related to the clinical course. As for seizure outcome, 12 (32%) of 38 patients were free from seizures at follow-up.
Conclusions: In epilepsies associated with tuberous sclerosis, there may be an interrelation between generalized and localization-related epilepsies, as well as one between generalized and partial seizures.     

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.