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PubMed1997

Hippocampal N-acetylaspartate in neocortical epilepsy and mesial temporal lobe epilepsy.

Previous magnetic resonance spectroscopy (MRS) studies have shown that N-acetylaspartate (NAA) is reduced not only in the ipsilateral but also in the contralateral hippocampus of many patients with mesial temporal lobe epilepsy (mTLE). The reason for the contralateral damage is not clear. To test whether the hippocampus is also damaged if the focus is outside the hippocampus, we have measured patients with neocortical epilepsy (NE). Therefore, the goals of this study were to determine if hippocampal NAA is reduced in NE and if hippocampal NAA discriminates NE from mTLE. MRS imaging (MRSI) studies were performed on 10 NE patients and compared with MRSI results in 23 unilateral mTLE patients and 16 controls. The results show that, in contrast to mTLE, NAA was not reduced in the hippocampus of NE patients, neither ipsilateral nor contralateral to the seizure focus. These results suggest that repeated seizures do not cause secondary damage to the hippocampus. The absence of spectroscopic differences in NE may help to distinguish NE from mTLE.

Adult↗
PubMed2003

[Treatment termination in children with idiopathic generalized epilepsy and cryptogenic focal epilepsy].

The regression of epileptic attacks after a period for remission brings hope, particularly for a sick child's parents, for prompt treatment termination. This process, however, is related to the risk that the disease will recur. The paper is about the evolution of these changes in EEG and the assessment of disease recurrence in the course of treatment and upon therapy termination in patients taking valproic acid preparations (VPA) during idiopathic generalized epilepsy (IGE) and those taking carbamazepine (CBZ) preparations during cryptogenic focal epilepsy (CFE). A total of 121 patients in whom the attacks regressed as a result of monotherapy, were subjected to the assessment. The patients were classified into 2 groups: the first group of 52 patients treated with VPA (IGE), patients' age at the beginning of treatment--from 6 to 14, and the second group of 69 patients treated with CBZ (CFE), patients' age at the beginning of treatment--from 5 to 12. In patients suffering from IGE, an absence of attacks prevailed; while in those with CFE--the secondary generalized ones prevailed. The drug dosage reduction lasted from 3 to 24 months. The observation time at treatment termination was 2 years. 10 routine EEG tests were assessed in each patient. Attack regression was achieved mainly within 2 weeks, sporadically up to 6 months. In the initial EEG tests 4 (5.8%) of the patients with partial seizures had normal records, in the remaining 94.2%--the records were abnormal. Throughout the duration of the treatment, systematic improvement of EEG was noted in groups 1 and 2. At the commencement of the dosage reduction, the EEG irregularities were maintained in 9% of the patients with IGE and in 26% of those with CFE, increasing during reduction until the discontinuance of the medication. Frequency of attack recurrences was twice as large in the patients with IGE (mainly after the end of treatment) than in those with CFE, where they occurred during the reduction period and up to 3 months after the end of treatment. The recurrence of the disease in children of the first group was only preceded in one third by irregularities in EEG; on the contrary, it occurred in two thirds of the second group. The morphology of changes was non-homogeneous.

Adolescent↗
PubMed2004

Understanding the delay before epilepsy surgery: who develops intractable focal epilepsy and when?

Intractable temporal lobe epilepsy that is surgically treated in adulthood is a disorder whose onset frequently occurs during childhood and early adolescence. The average duration of epilepsy prior to surgery is on the order of 20 years. The long delay between onset and surgery has at least two components: time from onset to intractability and time from evidence of intractability to surgery. The first interval is prolonged >10 years especially if the onset is during childhood. This suggests a complex natural history that we have not fully appreciated as well as a potential window of opportunity for early secondary intervention. The second interval is also prolonged, especially if onset was during childhood. Reasons for this are not fully clear but may include a reluctance to consider surgery and perhaps difficulty deciding whether seizures are sufficiently intractable to warrant surgery especially after what may have been a relatively benign initial course. Factors involved in the second delay need to be better understood so that surgical interventions can be appropriately targeted early rather than late, thereby reducing serious social, psychological, and educational consequences associated with uncontrolled seizures.

Adolescent↗
PubMed2005

Discriminating patients with frontal-lobe epilepsy and temporal-lobe epilepsy: utility of a multilevel design fluency test.

Patients with frontal-lobe epilepsy (FLE) or temporal-lobe epilepsy (TLE) and matched control participants were given a design fluency test that assessed nonverbal fluency and switching ability. Patients with FLE generated fewer designs in the switching condition relative to the TLE patients and controls, whereas group differences did not emerge in the basic fluency conditions. When the side of the seizure focus and the presence or absence of a structural lesion were considered in patients with FLE, only those with left-lesional FLE generated fewer designs than controls did in the switching condition. Furthermore, patients with left-lesional and nonlesional FLE produced a greater proportion of set-loss errors than did controls. These results indicate that patients with FLE are impaired when they must simultaneously generate new designs and engage in cognitive switching; however, the pattern of impairment may depend on the side of the seizure focus and the presence of a structural lesion.

Adult↗
PubMed2004

Evidence for linkage between juvenile myoclonic epilepsy-related idiopathic generalized epilepsy and 6p11-12 in Dutch families.

PURPOSE: Previous linkage studies provided evidence for juvenile myoclonic epilepsy (JME) susceptibility loci at 6p11-12, HLA-6p21.3 region, 15q14, and 5q34. These results indicate locus heterogeneity or interpopulation differences, thus underlining the importance of replication studies. METHODS: We describe a replication linkage study of the 6p-q13 region in 18 families ascertained from JME probands of Dutch descent. In the presence of heterogeneity, the definition of the disease status may be crucial, and we therefore used two disease phenotypes: narrow [JME/idiopathic generalized epilepsy (IGE)-"only"] and broad (JME/IGE-plus-fast EEG background activity). RESULTS: We found evidence of linkage at 6p11-12 in multipoint analyses (p < 0.01 in a replication study) for both these disease definitions. Analysis of this region, assuming heterogeneity and autosomal dominant inheritance with a conservative 60% of penetrance, gave a maximum multipoint parametric lod score of 2.07 at D6S1573 for the narrow phenotype and peaked at 2.53 between D6S1623 and D6S1573 for the broad phenotype. The p value for nonparametric linkage reached 0.0013 for the narrow phenotype and 0.0010 for the broad. Significant exclusion (lod score <or=2) was found for the HLA region and for 10 to 30 cM telomeric to HLA. Our results provide evidence for a susceptibility locus for JME/IGE-plus-fast EEG background activity at 6p11-12. CONCLUSIONS: Additionally, by using a narrow definition, we were able to confirm previous evidence for a JME-IGE locus at this location.

Adolescent↗
PubMed1983

Primary generalized epilepsy and temporal lobe epilepsy: a psychological study using Rorschach tests.

Rorschach tests were performed on patients with primary generalized epilepsy (PGE) and on patients with temporal lobe epilepsy (TLE) combined with generalized tonic-clonic convulsions. Each group consisted of 20 cases and the two groups were matched as closely as possible. Patients with PGE were found to be characterized by variable responses, sensitivity, extroversion and undifferentiated tendencies. Patients with TLE are practical but inefficient and constricted in character. Cases not on medication displayed the same character types. On the basis of the above findings, speculation is made concerning the features of neurotic or psychotic PGE patients.

Adult↗
PubMed1997

Adolescent onset of idiopathic photosensitive occipital epilepsy after remission of benign rolandic epilepsy.

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

Adolescent↗
PubMed2001

Basic science and epilepsy: experimental epilepsy surgery.

Epilepsy surgery, as is employed for the management of intractable seizures, was performed in animals harboring a seizure focus induced by a local application of kainic acid (KA). Amygdalo-hippocampectomy failed to stop spontaneous seizures in the contralateral hippocampus. Callosotomy inhibited seizure propagation to the contralateral sensori-motor cortex. However, epileptic activity ipsilateral to the focus, including subcortical structures, persisted even after the callosotomy. Multiple subpial transection (MST) around the epileptic cortical focus suppressed the seizure activity of the cortex. However, seizure propagations in subcortical structures remained, even after MST. Nefiracetam (a new nootropic agent) was tested in these models, and its promising effect on the intractable extratemporal epilepsy is reported.

Amygdala↗
PubMed2003

Practice parameter: temporal lobe and localized neocortical resections for epilepsy: report of the Quality Standards Subcommittee of the American Academy of Neurology, in association with the American Epilepsy Society and the American Association of Neurological Surgeons.

OBJECTIVES/METHODS: To examine evidence for effectiveness of anteromesial temporal lobe and localized neocortical resections for disabling complex partial seizures by systematic review and analysis of the literature since 1990. RESULTS: One intention-to-treat Class I randomized, controlled trial of surgery for mesial temporal lobe epilepsy found that 58% of patients randomized to be evaluated for surgical therapy (64% of those who received surgery) were free of disabling seizures and 10 to 15% were unimproved at the end of 1 year, compared with 8% free of disabling seizures in the group randomized to continued medical therapy. There was a significant improvement in quantitative quality-of-life scores and a trend toward better social function at the end of 1 year for patients in the surgical group, no surgical mortality, and infrequent morbidity. Twenty-four Class IV series of temporal lobe resections yielded essentially identical results. There are similar Class IV results for localized neocortical resections; no Class I or II studies are available. CONCLUSIONS: A single Class I study and 24 Class IV studies indicate that the benefits of anteromesial temporal lobe resection for disabling complex partial seizures is greater than continued treatment with antiepileptic drugs, and the risks are at least comparable. For patients who are compromised by such seizures, referral to an epilepsy surgery center should be strongly considered. Further studies are needed to determine if neocortical seizures benefit from surgery, and whether early surgical intervention should be the treatment of choice for certain surgically remediable epileptic syndromes.

Anticonvulsants↗
PubMed2002

[Higher brain dysfunction in benign childhood epilepsy with centrotemporal spike and atypical benign partial epilepsy of childhood].

Although some recent studies have reported various cognitive impairments and behavioral disorders in children having benign childhood epilepsy with centrotemporal spike (BCECT), it is still commonly believed that BCECT does not cause any definite neuropsychological impairment. In addition, reported impairments range over various cognitive functions, and there is no general agreement on this issue. We performed detailed neuropsychological tests in 17 children with BCECT and analyzed the profiles of their subtests. Atypical benign partial epilepsy of childhood (ABPE) is a type of BCECT in which patients have minor generalized seizures and their EEGs show continuous spike-waves during sleep. We also performed the same tests in five patients with ABPE, and compared the results in the two groups. Neuropsychological tests performed are as follows: Kaufman assessment battery for children (K-ABC), Wechsler intelligence scales for children-revised (WISC-R), Illinois test of psycholinguistic abilities (ITPA), Benton visual retention test (BVRT), Token test, calculation, figure copying task, letter copying task, line bisection task, and line cancellation task. Mental processing composite of the K-ABC and FIQ of the WISC-R were within normal limits in all children with BCECT and ABPE, but were generally lower in ABPE than in BCECT. On the other hand, the profiles of subtests of ITPA in children with BCECT revealed the significant feature of the lower scores on verbal expression (p = 0.013) and auditory sequential memory (p = 0.035). Considering the normal scores in the elementary cognitive functions, such as visual and verbal functions and long-term memory, disturbance in the process of executive functions such as flexibility, fluency, and working memory could cause this characteristic profile. ABPE also showed the similar profile in the subtests of ITPA to that of BCECT. It is likely that both groups of children share the common cognitive dysfunction.

Attention Deficit Disorder with Hyperactivity↗
PubMed2003

Reflex myoclonic epilepsy in infancy: a benign age-dependent idiopathic startle epilepsy.

Reflex myoclonic epilepsy of infancy is an idiopathic epileptic disorder characterized by myoclonic attacks, with onset in the first 2 years of life precipitated exclusively by unexpected tactile or auditory stimuli. We report on a 9 month-old infant with myoclonic attacks, which consisted of frequent clusters of up to 10 symmetric jerks affecting mainly the arms and the head occurring as reflex responses to unexpected auditory stimuli. There was no family history of epilepsy or febrile convulsions. Ictal EEG demonstrated a typical 3 Hz spike-wave pattern, while there were no abnormalities, either in wakefulness or during sleep. The neurodevelopmental examination was unremarkable and MRI of the brain was normal. The attacks disappeared 3 weeks after initiating sodium valproate, and have not reappeared since then (follow-up 3 years and 3 months); today, at 4 years of age, the patient has normal psycho-motor development. RMEI should be considered as an age-dependent, idiopathic, generalized epileptic syndrome with an apparently good prognosis[Published with video sequences].

Acoustic Stimulation↗
PubMed1985

Visual evoked response abnormality in myoclonus epilepsy with large pupils. Occurrence in a family with acorpuscular myoclonus epilepsy.

Flash and pattern evoked responses were recorded from three siblings with myoclonus epilepsy who all had strikingly large pupils in daylight. Comparison with the visual evoked responses (VERs) of 15 normal and eight epileptic control subjects (including one with myoclonus epilepsy but normal pupils) disclosed a substantial reduction of the amplitude of the flash response as compared with the normal pattern response in these three patients. It is suggested that the VER constellation and the pupillary abnormality, together with the normal electroretinogram and diffusely distributed relative scotomas, were due to a ganglion cell loss in the retina.

Adult↗
PubMed2004

Fos induction and persistence, neurodegeneration, and interneuron activation in the hippocampus of epilepsy-resistant versus epilepsy-prone rats after pilocarpine-induced seizures.

Previous studies demonstrated that the spiny rat Proechimys guyannensis exhibits resistance to experimental epilepsy. Neural activation was studied in the Proechimys hippocampus, using Fos induction, within 24 h after pilocarpine-induced seizures; neurodegenerative events were investigated in parallel, using FluoroJade B histochemistry. These parameters were selected since pilocarpine-induced limbic epilepsy is known to elicit immediate early gene expression and cell loss in the hippocampus of seizure-prone laboratory rodents. At variance with matched experiments in Wistar rats, pilocarpine injection resulted in Proechimys in seizure episodes that, as previously reported, did not develop into status epilepticus. At 3 h and 8 h after seizure onset, Fos immunoreactivity filled the dentate gyrus of both rat species, and was quite marked in pyramidal cells of the Proechimys Ammon's horn. At 24 h, Fos immunoreactivity dropped in the Wistar hippocampus and persisted in Proechimys. At 8 h and 24 h, FluoroJade-stained neurons were very few in the Proechimys hippocampus, whereas they were abundant in that of Wistar rats. Double immunohistochemistry for Fos and parvalbumin, the protein expressed by fast-spiking hippocampal interneurons, indicated that Fos was induced up to 24 h in the vast majority of parvalbumin-containing cells of the Proechimys hippocampus, and in a minority of these cells in the Wistar hippocampus. The findings demonstrate that early postepileptic neurodegeneration is very limited in the Proechimys hippocampus, in which sustained Fos induction persists for several hours. The findings also indicate that Fos induction and persistence may not correlate with seizure intensity and may not be associated with neuronal death. Finally, the data implicate differential mechanisms of interneuron activity in anti-convulsant and pro-convulsant phenomena.

Animals↗
PubMed2005

Language in benign childhood epilepsy with centro-temporal spikes abbreviated form: rolandic epilepsy and language.

Although Benign Childhood Epilepsy with Centrotemporal Spikes (BECTS) has a good prognosis, a few studies have suggested the existence of language disorders relating to the interictal dysfunction of perisylvian language areas. In this study, we focused on language assessment in 16 children aged 6-15 currently affected by BECTS or in remission. An important proportion of children showed moderate or more severe language impairment. The most affected domains were expressive grammar and literacy skills. We found linguistic deficits during the course of epilepsy but also persistent deficits in children in remission, suggesting possible long-term effects. Our results support the hypothesis that BECTS may be associated with impairment to language and suggest the possibility of a direct link between epileptic activity and language development, and the existence of long-term consequences.

Adolescent↗
PubMed2004

Epilepsy surgery, resection volume and MSI localization in lesional frontal lobe epilepsy.

To verify whether interictal noninvasive information detected by magnetoencephalography (MEG) recordings can contribute to localize focal epileptic activity relevant for seizure generation in lesional frontal lobe epilepsy, magnetic source imaging (MSI) localizations of epileptic discharges were compared to the extent of neurosurgical resection and postoperative outcome. Preoperative MEG spike localizations were displayed in postoperative magnetic resonance imaging (MRI) scans to check whether dipole sites were located within the resection cavity. Moreover, MEG localizations were compared with results of prolonged video-EEG monitoring and, in three cases, with invasive EEG recordings. Our results in five cases with lesional frontal lobe epilepsy showed that good surgical outcome could be achieved in those patients where the majority of MEG spike localizations were located within the resected brain volume.

Adult↗
PubMed2003

Validation of the quality of life in childhood epilepsy questionnaire in American epilepsy patients.

The aim of this study was to adapt the Australian Quality of Life in Childhood Epilepsy Questionnaire (QOLCE) and determine its psychometric properties in a North American population. Participants were North American families with children diagnosed with epilepsy. Parents were asked to complete the American QOLCE (USQOLCE) and the Child Health Questionnaire (CHQ). Seventy-one families completed the USQOLCE. The internal consistency reliability of the subscales was good. USQOLCE subscales correlated highly with theoretically similar subscales contained in the CHQ. Theoretically dissimilar subscales on the two instruments did not correlate as well. USQOLCE correlated significantly with a parental rating of seizure severity and an independent measure of degree of postoperative seizure control. This study demonstrated that the USQOLCE is suitable for a North American population with evidence of its reliability and validity including its sensitivity to seizure burden.

Activities of Daily Living↗
PubMed2006

Self-induction behavior in patients with photosensitive and hot water epilepsy: a comparative study from a tertiary epilepsy center in Turkey.

PURPOSE: The goal of this study was to compare self-induction (SI) patterns in photosensitive epilepsy (PSE) and hot water epilepsy (HWE). METHODS: Among 7609 consecutive patients, 129 were diagnosed as having PSE, and 34, HWE. RESULTS: Thirteen patients with PSE (10 females, 3 males, age at onset: 8.6; 3 with mental retardation) and 9 patients with HWE (all males, age at onset: 13.1) reported experiencing SI. During the seizures, a pleasurable feeling was described by all patients with HWE, but only by 3 patients with PSE. Patients with PSE induced their seizures by going very close to the TV screen or by waving their hands and blinking in front of the light sources. In the patients with HWE, seizures were precipitated by increasing the temperature of the water poured over the head or body. CONCLUSIONS: Our results indicate that SI is more frequent in HWE, although this behavior was attributed mostly to PSE in previous studies.

Adolescent↗