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1.
Summary: Five patients with chronic incapacitating seizures averaging 15–5,000/month were selected for study. All patients had more than one seizure type and had received maximal doses of antiepileptic drugs (AEDs). The centromedian thalamic nucleus (CM) was stimulated electrically through bilateral multicontact platinum electrodes stereotaxically placed in CM and connected to internalized pulse generators. Electrophysiologic confirmation of electrode position included thalamically elicited recruiting responses and EEG desynchronization recorded at the scalp. Stimulation parameters were adjusted individually in the range of 450–800-μA intensity, 65 pps, 0.09 ms, in 1-min trains, alternating right and left side stimulation and with 4-min intervals delivered for 2 h/day. Quantitative evaluation included frequency of seizures/month, number of maximal interictal paroxysmal discharges, and frequency of background activities counted in selected scalp EEG samples, taken throughout the observation period (7–33 months). Significance of changes was evaluated by parametric Student's t test. Generalized tonic-clonic seizures (GTC) decreased dramatically, almost disappearing in all cases (p < 0.001), with a significant reduction in interictal paroxysmal discharges (p < 0.01) and a tendency toward an increase in EEG back-ground frequency. Other generalized seizures (atypical absences) decreased significantly, but there was no change in the number of complex partial seizures (CPS). CM stimulation is useful in control of GTC, but its beneficial effect on other seizure types has not been established.  相似文献   

2.
In Dravet syndrome, interictal and ictal electroencephalography (EEG) recording may remain misleading, and are not specifically altered. Moreover, there is a great polymorphism of clinical and EEG seizure types. Some can be observed in other epileptic syndromes, but others are more specific--particularly the peculiar unilateral seizures, the falsely generalized seizures, probably with a focal onset, and the unstable seizures. In some cases, the ictal manifestations are characterized by the persistent predominant recurrence of convulsive seizures, often induced by body temperature increase, eventually associated with partial complex seizures. The myoclonic events, absences with myoclonic component, obtundation status, and photosensitivity and/or pattern sensitivity are absent or appear relatively late and recur transiently for short periods. In these cases interictal EEG is characterized by the persistent paucity of paroxysmal discharges. In other cases, on a background of convulsive seizures and body temperature sensitivity, one may find a variable association of (1) myoclonic seizures of different types, (2) a strong sensitivity to light and pattern stimulations, with early onset and persistent in time. In these cases, interictal paroxysms and spontaneous and induced (intermittent photic stimulation, patterns, and eye closure) stimulation tend to appear early and to be frequent and persistent during the evolution. According to these electroclinical patterns it is possible to divide the population into two subsets, both sharing common genetic mechanisms but with a different clinical outcome.  相似文献   

3.
结节性硬化致癫痫发作的临床特点及治疗观察   总被引:4,自引:1,他引:3  
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  相似文献   

4.
MECP2 duplication syndrome (MECP2 DS) is an X-linked disorder characterized by early-onset hypotonia, poor speech development, recurrent respiratory infections, epilepsy and progressive spasticity. Epilepsy occurs in more than 50% of the affected patients. Generalized tonic-clonic seizures (GTCS) are the most common seizure-type described but atonic seizures, absences and myoclonic seizures have also been reported. Electroencephalographic (EEG) and seizure types occurring in MECP2 DS have been poorly investigated. Here we report on two male siblings carrying a maternally-inherited MECP2 duplication. Patients underwent several EEG recordings and long-lasting video-EEG monitoring. The most represented seizure types were myoclonic and atonic seizures. GTCS were rarely observed. In patients, we found a slowing of the background activity with multifocal paroxysmal activity, prominent on the frontal areas. In conclusion, our observations seem to suggest that MECP2 syndrome seem to have a peculiar epileptic pattern mainly characterized by the occurrence of myoclonic seizures, the recognition of which is important in order to undertake an appropriate treatment.  相似文献   

5.
Characterization and classification of neonatal seizures   总被引:26,自引:0,他引:26  
E M Mizrahi  P Kellaway 《Neurology》1987,37(12):1837-1844
To characterize and classify neonatal seizures, we studied 349 neonates, using a portable, cribside EEG/polygraphic/video monitoring system. We recorded 415 clinical seizures in 71 infants; 11 other infants had electrical seizure activity without clinical accompaniments. Each seizure was analyzed in terms of its clinical character and its relationship to the presence of EEG seizure activity. Focal clonic seizures, some forms of myoclonic seizures, and focal tonic seizures were consistently associated with electrical seizure activity. Most "subtle" seizures, all generalized tonic seizures, and some forms of myoclonic seizures were either not associated with EEG seizure activity or had an inconsistent relationship with such activity. Seizures that were consistently and coherently related to focal EEG seizure activity had a high correlation with focal brain lesions and a favorable short-term outcome. Seizures with no relationship or an inconsistent relationship to EEG seizure activity were correlated with diffuse processes such as hypoxic-ischemic encephalopathy and a poor short-term outcome. The clinical and background EEG features of infants whose seizures were not accompanied by EEG seizure activity suggest that these seizures may not be epileptic in character.  相似文献   

6.
The onset of Dravet syndrome typically occurs within the first year, with prolonged, generalized, or unilateral clonic seizures triggered by fever. In the early stages, other types of refractory seizures usually present that include myoclonic seizures, atypical absences, and partial seizures. Electroencephalography (EEG) findings are not pathognomonic, and signs of cognitive arrest or deterioration progressively appear. In contrast, in adults, myoclonic seizures, atypical absences, and focal seizures tend to disappear, and short tonic–clonic seizures, often associating a focal component, persist particularly during sleep. The sensitivity to fever persists into adulthood, and although mental deterioration occurs in infancy, usually leaving patients with severe mental impairment, further deterioration does not occur. The identification of genes associated with Dravet syndrome and related syndromes hints at the complexity of the etiology of such epilepsies. Identifying SCN1A mutations has become useful as a means to support an early diagnosis of Dravet syndrome, to benefit counseling, and to avoid use of antiepileptic drugs (AEDs) that may have adverse effects. However, the defining characteristics of seizure type and EEG patterns initially identified by Dravet remain fundamental to diagnosis.  相似文献   

7.
When antiepileptic drugs aggravate epilepsy   总被引:18,自引:0,他引:18  
Paradoxically, an antiepileptic drug (AED) may aggravate epilepsy. The number of AEDs is steadily increasing, and the occurrence of paradoxical aggravation will probably become a frequent problem. The overall status of the patient treated for epilepsy can be altered due to maladjustment to the diagnosis of epilepsy, to unwanted side-effects, to overdosage and to the occurrence of tolerance. However, the main mechanism of aggravation is the occurrence of an inverse pharmacodynamic effect. The specific effect of the AED is such that it controls epilepsy in most cases and increases seizures in other cases. Idiopathic generalised epilepsies (IGE) are particularly prone to pharmacodynamic aggravation: typical absences are constantly increased by carbamazepine (CBZ), vigabatrin, tiagabine, gabapentin, while phenytoin (PHT) is less aggravating. Juvenile myoclonic epilepsy is often aggravated by CBZ, less constantly by PHT and other AEDs. Generalised tonic-clonic seizures found in IGEs may respond to AEDs that aggravate the other seizure types. In symptomatic generalised epilepsies, patients have often several seizure types that respond differently to AEDs: myoclonias are generally aggravated by the same drugs that aggravated IGEs; tonic seizures in the Lennox-Gastaut syndrome respond to CBZ, which may however aggravate atypical absences. In severe myoclonic epilepsy of infancy, there is a nearly constant aggravating effect of lamotrigine. In some patients with benign rolandic epilepsy, a clear aggravation may be produced by CBZ, with occurrence of negative myoclonias, atypical absences, drop attacks, and at the maximum evolution into a state of electrical status epilepticus during sleep. It is much more difficult to pinpoint specific pharmacological sensitivity in other focal epilepsies, but aggravation clearly occurs. When treating epilepsy, the clinician should act according to seizure type, or, better, to epilepsy type. Patients are usually aware of aggravation before their doctors: we should listen carefully whenever they express a 'dislike' for an AED.  相似文献   

8.
Eleven neonates with seizures had continuous EEGs recorded before and up to several hours after administration of anticonvulsant therapy. Six of seven babies given phenobarbitone responded clinically, but in four of these the EEG showed that seizure discharges either persisted or recurred within two hours. Chloral hydrate had no immediate effect in one case and only a doubtful influence on EEG seizure activity in another. Three babies were given benzodiazepines: after an apparently rapidly effective bolus dose of diazepam, recurrence of seizure discharges was almost immediate. Infusions of diazepam or clonazepam eventually were associated with persistent control of clinical and EEG seizures. In the treatment of neonatal seizures, conventional anticonvulsants are rarely completely effective: EEG monitoring is essential for optimal therapeutic intervention.  相似文献   

9.
Reactive Occipital Epileptiform Activity: Is It Benign?   总被引:6,自引:6,他引:0  
Occipital epileptiform activity that is almost continuous and reactive to eye opening has been associated with a childhood epilepsy syndrome and basilar migraine with seizures. An association of these syndromes with a benign course had been disputed. In this study, a retrospective investigation of reactive occipital epileptiform activity (ROEA) was performed to determine the prognostic value of this distinctive EGG pattern. The EEG and hospital record of patients with ROEA were reviewed with an observation period of 6 months to 8 years. The patients were divided into good and poor outcome groups based on response to treatment. Of 33 patients, 12 (36.4%) had complete seizure control; 21 (63.6%) continued to have poorly controlled seizures. Only 3 (9.1%) patients were able to discontinue antiepileptic drugs (AEDs) without seizure recurrence. Analysis of clinical and EEG variables showed that a history of perinatal difficulties, abnormal neurologic findings, and abnormal EEG background activities occur significantly more frequently in the poor outcome group. This study suggests that ROEA is not uniformly associated with a benign course and that other factors are involved in determining prognosis of the epilepsy.  相似文献   

10.
Fylan F  Edson AS  Harding GF 《Epilepsia》1999,40(3):370-372
PURPOSE: The continued presence of EEG abnormalities in patients with a history of photosensitive seizures is used to signify the persistence of photosensitive epilepsy. The extent to which this approach places patients at risk of seizures is unclear, however. We describe those EEG abnormalities that may be tolerated with low risk of further seizures, and those that are indicative of poor seizure control. METHODS: Fifty patients with EEG evidence of persistent photosensitive epilepsy underwent photosensitivity testing with diffuse and patterned light; 58% of patients continued to experience seizures, and 42% were seizure free. The incidence of EEG abnormalities to diffuse and patterned intermittent photic stimulation (IPS) was analysed as a function of recent seizures. RESULTS: All patients showed EEG abnormalities to patterned IPS; there was no significant association between patterned IPS and poor seizure control. EEG abnormalities to diffuse IPS occurred in 58% of patients, and 76% of these patients had experienced a seizure within the previous year. These patients were more than twice as likely to be poorly controlled than those who showed abnormalities only to patterned IPS. These results were consistent for both medicated and unmedicated patients. CONCLUSIONS: EEG abnormalities to patterned IPS can be used to signify the persistence of photosensitive epilepsy, but abnormalities to diffuse IPS are more likely to indicate the patient is poorly controlled and at risk of further seizures.  相似文献   

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