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1.
One of the nosological problems of childhood epilepsies is the evolutionary change of foci with age. Benign childhood epilepsy with centro-temporal spike may evolve to show EEG features of childhood epilepsy with occipital paroxysms and vice versa. West syndrome is generally accepted as generalized epilepsy but is often preceded or followed by partial seizures and may have both partial seizures and tonic spasms in a single attack. Among epilepsies with both generalized and focal seizures, severe myoclonic epilepsy in infancy is more linked to generalized epilepsy whereas epilepsy with continuous spike-waves during slow wave sleep is more linked to localization-related epilepsy. Any type of partial epilepsy may develop nonconvulsive generalized seizures transiently with generalization of paroxysmal discharges during the clinical course. Moreover, nonconvulsive generalized seizures may occur even in localization-related epilepsy with spike-wave index during NREM sleep in less than 85%.  相似文献   

2.
Abstract: One of the nosological problems of childhood epilepsies is the evolutionary change of foci with age. Benign childhood epilepsy with centro-temporal spike may evolve to show EEG features of childhood epilepsy with occipital paroxysms and vice versa. West syndrome is generally accepted as generalized epilepsy but is often preceded or followed by partial seizures and may have both partial seizures and tonic spasms in a single attack. Among epilepsies with both generalized and focal seizures, severe myoclonic epilepsy in infancy is more linked to generalized epilepsy whereas epilepsy with continuous spike-waves during slow wave sleep is more linked to localization-related epilepsy. Any type of partial epilepsy may develop nonconvulsive generalized seizures transiently with generalization of paroxysmal discharges during the clinical course. Moreover, nonconvulsive generalized seizures may occur even in localization-related epilepsy with spike-wave index during NREM sleep in less than 85%.  相似文献   

3.
Summary: Purpose: To determine the distribution of various epilepsies and epileptic syndromes in the epileptic population treated in a university hospital in a developing country. Methods: Data concerning 2,531 patients with epilepsy seen between January 1989 and June 1994 were analyzed using the international League Against Epilepsy (ILAE) classification. Results: Of 2,531 cases, 48% fell into ILAE categories 1.3, 3.2, or 4.1 (cryptogenic, without unequivocal generalized or focal seizures; or situation-related seizures, respectively). Localization-related epilepsies (LREs) and epileptic syndromes (1.1, 1.2, 1.3) were found in 1,591 (62.9%) patients; of these patients, symptomatic localization-related epilepsies totaled 62.7%, and idiopathic localization-related epilepsies accounted for only 0.7%. Juvenile myoclonic epilepsy was the most common type of idiopathic generalized epilepsy (IGE), comprising 4.9% of the total study population and 7.7% of patients registered in the epilepsy clinic. A combination of childhood and juvenile absence epilepsies were found in only 0.4% of the total study population. Single computed tomography (CT) enhancing lesion (SCTEL) and focal cerebral calcification (FCC) accounted for 22% of the etiologic factors for localization-related epilepsies. Neurologic deficits were found in 9.5% of patients with SCTEL; none were found with FCC. None of the patients with these lesions had any history of antecedent events that suggested CNS involvement. In patients with localization-related epilepsies with unremarkable clinical data, the proportion of CT scans showing SCTELs was 39 (95% confidence interval [CI], 0.35–0.43) and 0.18 (95% CI, 15–0.21) for FCCs. The proportion for both lesions together was 0.57 (95% CI, 0.53–0.61). Seizures did not recur once the lesion resolved in patients with SCTELs. In patients with FCCs, seizure remission was 71.5% (95% CI, 53.7–85.4) at 3 years. Conclusions: This study illustrates the rarity in one patient population of some of the syndromes and categories described in the ILAE classification. Childhood and juvenile absence epilepsies together formed a small proportion. SCTEL and FCC were important etiologic factors for localization related epilepsies. The epilepsy associated with SCTEL was a form of benign epilepsy; epilepsy associated with FCC had remission rates similar to other remote symptomatic epilepsies. Without neuroimaging evidence, these 2 lesions would have been missed and the patients might have been grouped under cryptogenic localization related epilepsy. For this reason, we emphasize the need for neuroimaging in patients with localization related epilepsies with unremarkable clinical findings, before classification into the cryptogenic category. In the absence of neuroimaging, such patients should be classified as “probably cryptogenic.”  相似文献   

4.
Colin D. Ferrie 《Epilepsia》2005,46(S9):91-95
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.  相似文献   

5.
Children with Focal Sharp Waves: Clinical and Genetic Aspects   总被引:11,自引:9,他引:2  
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.  相似文献   

6.
In this prospective, population-based study of 594 cases of newly diagnosed epilepsy, proportions in categories as defined by the International League Against Epilepsy (ILAE) were as follows: (1) localization-related epilepsies: 1.1* idiopathic, 1.2%; 1.2* symptomatic, 16.2%; and 1.3 cryptogenic, 24.6%; (2) generalized epilepsies: 2.1* idiopathic (idiopathic generalized epilepsy) with 3-Hz spike and wave: absence epilepsy, 2.2%; juvenile myoclonic epilepsy, 1.5%; and nonspecific idiopathic generalized epilepsy, 5.6%; 2.3.1* symptomatic generalized epilepsies, 1.5%; 2.3.2* specific syndromes with generalized epilepsy, 0.3%; 3.2 seizures without unequivocal focal or generalized features, 32%; 4.1 situation-related syndromes, isolated seizures, 9.9%; seizures due to acute toxic or metabolic cause,* 4.5%. Only 33.6% were in diagnostic ILAE categories (asterisks) and many rare syndromes were not represented. The remainder (66.4%) were in various nonspecific categories. Only 24% of localization-related epilepsies could be clinically localized to a single ILAE-proposed site of origin and of these best localized cases, 14% had strongly discordant imaging or electroencephalograms. These major problems in applying the ILAE classification to epilepsy in the general population and its underemphasis of modern imaging techniques are discussed.  相似文献   

7.
Summary: A population-based survey of childhood epilepsy was made in 1975 on the total population of children aged >10 years living in Okayama Prefecture (n = 2,378 patients). Using the data obtained, we attempted to re-classify the various types of epilepsy according to the international classification (ILAE, 1989). Reclassification was possible in 1,872 (78.7%) of the 2,378 cases. The 1,872 cases consisted of 1,045 (55.8%) with localization-related epilepsies, 824 (44.0%) with generalized epilepsies, and 3 (0.2%) with epilepsies undetermined whether focal or generalized. Classification of the epilepsies in a population-based survey using the international classification involves difficulties, because both clinical and EEG findings are essential. However, if an appropriate area is selected, classification of epilepsies and epileptic syndromes in a population-based survey is possible by referring to all medical records stored at every hospital and practitioner's clinic that administers treatment to patients with epilepsy in the area.  相似文献   

8.
Long-Term Follow-Up of Childhood Epilepsy Associated with Tuberous Sclerosis   总被引:10,自引:4,他引:6  
Yoko Ohtsuka  Iori Ohmori  Eiji Oka 《Epilepsia》1998,39(11):1158-1163
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.  相似文献   

9.
West Syndrome and its Related Epileptic Syndromes   总被引:1,自引:1,他引:0  
Yoko Ohtsuka 《Epilepsia》1998,39(S5):30-37
Summary: The purpose of this project was to study the relationship between West syndrome (WS) and its related epileptic syndromes, and reconsider the nosological limits of WS. The electroclinical features of 45 patients who experienced spasms in series were investigated, as well as some features not common in patients with WS. All patients were mentally retarded. The patients were divided into three groups: Group 1 consisted of 12 patients with refractory epilepsies with onset in early infancy, group 2 consisted of 5 patients with symptomatic localization-related epilepsies associated with spasms in series, and group 3 consisted of 28 patients with generalized epilepsies who had spasms in series after age 2 years. Partial seizures were the dominant symptom throughout the clinical course and spasms in series associated with atypical hypsarrhythmia appeared transiently during infancy in a significant number of the patients in group 1. In group 2, complex partial seizures (CPS) were the main seizure type and hypsarrhythmia was not observed during the clinical course. The EEGs in group 3 patients showed diffuse slow spike-waves or multifocal epileptic discharges in all but 1 patient. The EEG of the remaining patient still showed hypsarrhythmia at age 8 years. Therefore, group 1 patients should be classified as having WS although cortical mechanisms play a critical role in the occurrence of their seizures. Group 2 patients should be considered as having a type of localization-related epilepsy even though they share a similar pathophysiological mechanism with group 1. In group 3, 1 patient whose EEG still showed hypsarrhythmia was classified as having WS. The other patients should be classified as having generalized epilepsies other than WS.  相似文献   

10.
Mayer TA  Schroeder F  May TW  Wolf PT 《Epilepsia》2006,47(6):1059-1067
PURPOSE: Perioral reflex myoclonias (PORM) are obvious, frequent, but often unobserved focal seizures in different epileptic syndromes and the leading seizure type in reading epilepsy. PORMs remain often undiagnosed because the patients are not aware that these are epileptic seizures and fail to report them. Their semiology is not fundamentally different in various epileptic syndromes. METHODS: We studied the frequency of PORM in patients with juvenile myoclonic epilepsy (JME) compared with patients with focal epilepsies. Twenty-five patients with JME were investigated with a standardized neuropsychological test program and compared with 25 matched patients with focal epilepsies. Statistical significance was calculated by using Fisher's exact test. RESULTS: We found significant differences between the groups regarding both frequency of PORM and activation of epileptic discharges. These observations seem to indicate that PORM, like praxis-induced seizures, are typical traits in JME. CONCLUSIONS: PORM are more frequent in JME compared with focal epilepsies. The distinction between focal and generalized epileptic ictogenesis may be less clear than is traditionally believed.  相似文献   

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