[Identical twin cases of benign infantile epilepsy with complex partial seizure].
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Diseases in Twins↗
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Results for “Epilepsy, Complex Partial”
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Abnormal cortical activation during planning of voluntary movement in patients with epilepsy with focal motor seizures: event-related desynchronization study of electroencephalographic mu rhythm.
PURPOSE: The spatiotemporal distribution of EEG mu rhythm desynchronization was analyzed in patients with partial epilepsy to determine whether frequent focal motor seizures could induce a change of cortical activation during the planning of a voluntary movement. METHODS: The event-related desynchronization (ERD) of the mu rhythm was quantified during a self-paced voluntary movement of the thumb. The results were compared between two groups of patients with epilepsy: in one group (n = 12), the patients had frontal lobe epilepsy with frequent focal motor seizures (FMS); in the second group (n = 12), they had temporal lobe epilepsy (TLE) with complex partial seizures but no ictal movement disorder. The results were also compared with those of control subjects of same age (n = 10). RESULTS: In the control group, desynchronization of mu rhythm began over the contralateral central region 2,000 ms before the movement onset. In the FMS group, the desynchronization of mu rhythm was delayed, appearing only 500 ms before the movement onset, and the amplitude of ERD was increased over the frontocentral region. In the TLE group, the spatiotemporal pattern of ERD was the same as in normal subjects, but the amplitude of ERD was increased. CONCLUSIONS: These results indicate that there is a change of reactivity of mu rhythm in patients with partial epilepsy. The change in spatiotemporal pattern of ERD in patients with frequent focal motor seizures suggests that there is an abnormal cortical activation during the planning of a voluntary movement.
Adult↗
Febrile Seizures.
Febrile seizures should not be treated with continuous or intermittent antiepileptic medication. Parents should be given a comprehensive explanation as to the nature of this benign disorder and potential strategies to terminate prolonged seizures or clusters of seizures. Patients with complex febrile seizures (ie, those that are prolonged, focal, recur within the same day or those which affect children with pre-existing neurologic abnormalities) should be encouraged to use rectal diazepam at the time of recurrent seizures. Chronic daily prophylaxis should be considered only in highly selected cases. There is no definitive way to distinguish the small subset of children with febrile seizures that are at an increased risk for the development of complex partial epilepsy with mesial temporal sclerosis. Complex febrile seizures, particularly focal and prolonged seizures beyond 15 minutes duration are at somewhat higher risk as demonstrated by family studies, magnetic resonance imaging (MRI) studies, and retrospective analysis of intractable complex partial epilepsy.
Journal Article↗
Double-blind placebo-controlled evaluation of flunarizine as adjunct therapy in epilepsy with complex partial seizures.
Flunarizine was compared to placebo in a double-blind cross-over trial of 2 16-week treatment periods separated by a 4-week wash-out period. The patients had epilepsy with complex partial seizures with or without secondary generalised seizures. Twenty-nine patients entered the trial, but 7 dropped out. Of the 22 patients completing the trial, 13 were women; the median was 39 years (range 15-58) and the median duration of epilepsy 23 years (range 4-55). There was no statistically significant difference between flunarizine 15 mg daily and placebo as adjunct therapy in total seizure frequency, neuropsychological tests, and patient's preferences. No interactions with concomitant antiepileptic drugs and no laboratory abnormalities were registered.
Adolescent↗
A study of ring 20 chromosome karyotype with epilepsy.
We reported a 24-year-old woman with moderate mental retardation and partial epilepsy. She developed complex partial seizures at 3 years of age and generalized tonic convulsions at 9 years. Chromosome analysis revealed that she had mosaicism (87%) of 46, XX, and r(20) (p13,q13.3). Her electroencephalogram showed bilateral 2-3 Hz sharp and wave complex over the bilateral frontopolar, and centro-parieto-occipital areas. Computed tomographic and magnetic resonance image examinations were normal. Twenty-five cases of ring 20 chromosome karyotypes (including this case) have been reported in the literature; 19 showed epilepsy, and 18 showed moderate mental retardation. Many of the patients showed growth retardation and minor malformations. The ring 20 syndrome is associated with a high incidence of epilepsy, particularly partial epilepsy. Our findings indicate that the main features of the ring 20 syndrome are partial epilepsy and mental retardation.
Abnormalities, Multiple↗
Homocarnosine and seizure control in juvenile myoclonic epilepsy and complex partial seizures.
OBJECTIVE: To assess the relationship between seizure control and gamma-aminobutyric acid (GABA), homocarnosine, and pyrrolidinone levels in the visual cortex of patients with epilepsy taking valproate or lamotrigine. Previous studies suggested that poor seizure control was associated with low GABA and homocarnosine levels. METHODS: In vivo measurements of GABA, homocarnosine, and pyrrolidinone were made in a 14-cm(3) volume of the occipital cortex using (1)H spectroscopy with a 2.1-Tesla MR spectrometer and an 8-cm surface coil. Twenty-six adults (eight men) taking valproate or lamotrigine were recruited; 12 had complex partial seizures (CPS) and 14 had juvenile myoclonic epilepsy (JME). RESULTS: Median homocarnosine levels were normal for patients with JME and below normal for patients with CPS. Better seizure control was associated with higher homocarnosine levels for both groups. Median GABA was below normal for patients with JME, lower than for patients with CPS. Brain GABA was lowest in patients with JME even when seizure control was excellent. Pyrrolidinone levels were above normal in almost all patients with JME. CONCLUSIONS: Low GABA levels are associated with poor seizure control in patients with CPS, but not in JME. Higher homocarnosine levels are associated with better seizure control in both types of epilepsy.
Adult↗
Temporal intermittent rhythmic delta activity in electroencephalograms.
Temporal intermittent rhythmic delta activity (TIRDA) has been reported to be highly specific for diagnosing complex partial epilepsy. Of 12,198 electroencephalographic (EEG) recordings performed at the Mayo Clinic between May 1, 1990 and May 1, 1991, 33 records from 27 patients (18 women and nine men; mean age, 41.5 years; range, 13-82 years) showed TIRDA. Clinical seizures were diagnosed in all patients, and complex partial epilepsy was well documented in 23. In a control group of 100 patients without TIRDA and matched for age and sex, generalized seizures were diagnosed in 25 and partial seizures in 15. Differences between TIRDA and control groups were highly statistically significant. Focal temporal sharp waves or spikes occurred in 23 patients with EEG recordings that contained TIRDA; three of these patients also exhibited generalized atypical spike-and-wave discharges. Four patients had TIRDA but no other epileptiform activity, although earlier EEGs of three of these patients contained spikes or sharp waves. These findings confirm earlier work, and we conclude that TIRDA represents an important epileptogenic abnormality.
Adolescent↗
Blink rate in pediatric complex partial seizure disorder.
This study examined spontaneous blink rate, a putative measure of dopamine function, in 30 children with complex partial epilepsy and 61 normal children. The children with epilepsy had significantly lower blink rates than the normal children in a conversation and a verbal recall task, particularly if they had a schizophrenia-like psychosis, EEG evidence for left focal epileptic activity, illogical thinking, discourse deficits, and distractibility. They modulated their blink rates across a listening, a conversation, and a verbal recall task like the normal children. Given previously reported low blink rates in schizophrenic children, these findings suggest that children with complex partial epilepsy or schizophrenia might have similar biological features.
Analysis of Variance↗
The epidemiology of drug resistant epilepsy and adverse effects of antiepileptic drugs.
Patients with partial, particularly complex partial, epilepsy, especially where this is related to underlying cerebral disease or damage, tend to respond poorly to existing antiepileptic drug therapy. The epidemiology of such patients is reviewed, together with the adverse effects of antiepileptic drug therapy which may be acute (dose-related and idiosyncratic) or chronic. Such chronic toxicity may cause nervous system, skin, hepatic, haematological, endocrine, and connective tissue problems, and also disorders of pregnancy. As complex partial epilepsy often responds poorly to drug therapy, the possible benefit of surgical treatment should be considered at a relatively early stage.
Abnormalities, Drug-Induced↗
Visual field constriction in children with epilepsy on vigabatrin treatment.
Vigabatrin is considered the drug of choice for infantile spasms and simple and complex partial epilepsy in childhood. Its mechanism of action relies on the irreversible inhibition of gamma-aminobutyric acid (GABA) transaminase. Since June 1997 several articles have been published reporting visual field constriction in adult patients on vigabatrin therapy. Recently, 7 pediatric patients, 1 on vigabatrin monotherapy and 6 on add-on therapy with visual field constriction have been described. We have observed 30 pediatric patients with epilepsy (14 boys and 16 girls), ages ranging from 4 to 20 years (mean: 11 years and 2 months) treated with vigabatrin for infantile spasms, simple and complex partial epilepsy, who had never complained of ophthalmologic disturbances. Twenty-one patients underwent complete routine ophthalmologic examination (fundus oculi, visual acuity, intraocular pressure, and visual field tests); 9 children (<6 years old) underwent only fundus examination, because collaboration was lacking. We report on 4 children showing constriction of visual field, prevailing in nasal hemifield. In 1 child, visual abnormalities were stable even 10 months after vigabatrin discontinuation, while in another a greater improvement was observed 5 months after discontinuation. The possible mechanisms have been discussed and the cone dysfunction, connected with GABA augmentation in the outer retina, has been outlined. We suggest a possible protocol to control visual abnormalities in epileptic children.
Adolescent↗
Chronic epilepsy with complex partial seizures is not always medically intractable--a long-term observational study.
OBJECTIVE: To study the prognosis of patients with complex partial seizures (CPS) with or without simple partial (SPS) and secondarily generalized tonic-clonic seizures (GTCS) and to analyze the factors related to the degree of medical responsiveness. MATERIAL AND METHODS: A total of 266 adult patients with CPS were included in a hospital based observational survey with a follow-up of 2 to 25 years. Clinical characteristics, seizure frequency, electroencephalography (EEG), cerebral computed tomography (CCT) and magnetic resonance imaging (MRI) findings were analyzed. Patients were categorized according to their degree of medical responsiveness into one of three groups: seizure free, improved control (>50% seizure reduction) and poor control. RESULTS: Mean age at follow-up was 44.7 years (SD 14.7, range 19-93). Mean age at seizure onset was 18.1 years (SD 14.7, median 15, range 1-79). Complete seizure control was achieved in 40%, improved seizure control in 36% and poor seizure control in 24%. Patients entered remission after a mean period of 15.7 years (SD 12.6, median 13, range 1-54) of active epilepsy. A third of all seizure-free patients were still in remission 6.1 years (SD 5.3, median 3.5, range 1-18) after discontinuation of antiepileptic drugs (AED). Patients with poor seizure control had a significantly younger age at onset (P<0.01), a higher initial seizure frequency (more than 3 per month) (P<0.01), abnormal neurological examination (P<0.01), and were more often mentally handicapped (P<0.01). Multiple logistic regression analysis revealed a high initial seizure frequency, mental handicap and an abnormal neurological examination as independent risk factors for poor seizure control. A positive family history, a history of febrile convulsions and/or psychosis, an abnormal EEG or MRI was not predictive of poor outcome. CONCLUSIONS: Not all patients with CPS were medically intractable. Seizure remission can be achieved after a long time of active epilepsy. Poor seizure control was associated with a high initial seizure frequency, mental handicap and abnormal neurological examination.
Adult↗
Role of I-123-iomazenil SPECT imaging in drug resistant epilepsy with complex partial seizures.
Fifteen patients with therapy resistant partial complex seizures with no structural lesions were examined interictally with 123-I-IOMAZENIL SPECT for measurement of benzodiazepine receptor distribution and with 99m-Tc-HMPAO SPECT for measurement of cerebral blood flow distribution. Regional abnormalities were correlated with the seizure onset patterns in EEG later recorded with implanted subdural strips. SPECT scans were made immediately after and at 1 and 2 h after intravenous injection of 123-I-Iomazenil. During that time there was a continuous change from an immediate flow-related distribution toward a more specific receptor distribution. The decay of radioactivity of I-123 in the brain was linear over time. Two patients on benzodiazepine treatment showed much faster elimination and showed no focal abnormalities. Eight patients with clear-cut unifocal seizure onset showed concordant focal benzodiazepine defects. These patients showed a progressive focus/homotopic non-focus enhancement over time much larger than the HMPAO scans in the same patients. Also the estimated focal area of abnormality was more restricted in the Iomazenil scans than in HMPAO scans. Five patients had more complex seizure onset patterns. In these patients a mismatch between the locations of abnormalities in Iomazenil and HMPAO scans were often found but benzodiazepine receptor abnormalities were more circumscribed also in these patients.(ABSTRACT TRUNCATED AT 250 WORDS)
Adolescent↗
Middle childhood onset of interictal psychosis.
The authors describe three children (mean age = 7.8 years) with complex partial epilepsy, left temporal lobe involvement, and interictal schizophrenia-like psychosis. As described in adults with complex partial epilepsy, these children met DSM-III criteria for schizophrenia, their affect was intact, and they demonstrated no negative signs of schizophrenia. Unlike adult epileptic patients, these children demonstrated psychotic symptomatology despite inadequate seizure control and after a short latency period. The possible role of early onset seizures, temporal lobe lesions, and kindling on the developing brain are discussed.
Child↗
Autosomal dominant cortical myoclonus and epilepsy (ADCME) with complex partial and generalized seizures: A newly recognized epilepsy syndrome with linkage to chromosome 2p11.1-q12.2.
We describe a pedigree in which eight individuals presented with a non-progressive disorder with onset between the ages of 12 and 50 years. It was characterized by predominantly distal, semi-continuous rhythmic myoclonus (all patients), generalized tonic-clonic seizures (all patients) and complex partial seizures (three patients). Most individuals had rarely suffered seizures and had a normal cognitive level, but three individuals with intractable seizures had mild mental retardation. The pattern of inheritance was autosomal dominant with high penetrance. We defined this disorder as autosomal dominant cortical myoclonus and epilepsy (ADCME). All patients had frontotemporal as well as generalized interictal EEG abnormalities. A neurophysiological study of the myoclonus suggested a cortical origin. Back-averaging of the data generated a series of waves with a frequency that mirrored the frequency of EMG bursts. Frequency analysis identified significant peaks with coherence between EMG and EEG, which were recorded over the contralateral rolandic area in five patients. The frequency of coherence was 8-25 Hz and phase spectra confirmed that EEG activity preceded EMG activity by 8-15 ms. In two individuals there was also significant coherence between the ipsilateral EEG and EMG, consistent with the transcallosal spread of myoclonic activity. The C-reflex at rest was enhanced and somatosensory and visual evoked potentials were of high amplitude. The resting motor threshold intensity to transcranial magnetic stimulation was significantly reduced (38%; SD +/- 7; P = 0.01) and the post-motor evoked potential silent period (101 ms; SEM +/- 10) was significantly shortened compared with the controls (137 ms; SEM +/- 18). These clinical and neuro- physiological characteristics suggest diffuse cortical hyperexcitability and high propensity for intra-hemispheric and inter-hemispheric cortical spread, as well as rhythmic myoclonic activity. Genome-wide linkage analysis identified a critical region spanning 12.4 cM between markers D2S2161 and D2S1897 in 2p11.1-q12.2, with a maximum two-point LOD score of 3.46 at Theta 0.0 for marker D2S2175. Multipoint LOD score values, reaching 3.74 around D2S2175, localize the ADCME gene to the centromeric region of chromosome 2. The exclusion of the locus for familial adult myoclonic epilepsy on chromosome 8q23.3-q24 from linkage to our family and the new localization of the responsible gene to chromosome 2cen, together with the different phenotype, define a new epilepsy syndrome. We hypothesize that the responsible gene causes cortical hyperexcitability that is widespread but particularly involves the frontotemporal circuits.
Adult↗
Ifenprodil and arcaine alter amygdala-kindling development.
The NMDA receptor complex is thought to be altered in kindling, an animal model for complex partial epilepsy. This receptor complex has several modulatory sites including those for glutamate, glycine and polyamines with activation resulting in altered cation channel opening. Two NMDA receptor effectors, ifenprodil and arcaine, were evaluated for effects on the acquisition of electrical kindling of the amygdala. Rats were administered 0, 3.2, 10, 32 and 100 microg of ifenprodil or 0, 32 or 100 microg of arcaine, intracerebroventricularly, 10 min before a daily kindling stimulus. Ifenprodil, at low doses, enhanced kindling acquisition, while the highest dose, 100 microg, inhibited kindling. Arcaine increased the number of trials required to reach fully generalized (stage 5) seizures at the 100 microg dose. Since these agents had mixed actions on kindling development, it is unclear whether these or similar NMDA effectors would be useful in the modulation of complex partial seizures.
Amygdala↗
Complex partial seizures. Behavioral epilepsy.
One of the most frustrating problems for veterinarians is the animal with a recurring behavioral disorder that is apparently a seizure disorder. Similar human disorders have been shown to be caused, in most cases, by organic disease in the cerebrum. There are reports in the veterinary literature that appear to support the same theory; however, there is no well-designed study with adequate animal numbers that proves the syndromes to be the same as in human medicine. It is clear that much research needs to be done. Diagnostic work up should be done meticulously, with emphasis on looking for intracranial disease. Treatment with phenobarbital is recommended to control the seizures, although the results will be variable.
Animals↗
Neocortical propagation in temporal lobe spike foci on magnetoencephalography and electroencephalography.
Propagation of the neuronal population of the interictal epileptic spike was quantified in 5 patients with complex partial epilepsy arising from temporal lobe using electroencephalography and magnetoencephalography. During the spike complex in each patient there was a spike at the deep sphenoidal electrode and a spike at the superficial scalp electrode on spontaneous electroencephalography. In each patient the sphenoidal spike had a different peak latency than the scalp spike, consistent with spike propagation. Electroencephalography was used to trigger two magnetoencephalographic averages of stereotyped spikes during the sphenoidal peak and the scalp peak. Magnetoencephalography discriminated the centers of two cortical spike populations at different latencies, showing deeper localization with sphenoidal trigger and more superficial localization with scalp trigger in each patient (p less than 0.05). Latency differences and propagation distances of spikes were consistent with the conduction velocity of corticocortical fibers. Noninvasive estimates of the cortical surface area of the spikes agreed with estimates obtained by electrocorticography over temporal neocortex. These findings indicate propagation of neuronal populations active during human interictal spikes between deep and superficial cortex of temporal lobe, likely by monosynaptic or oligosynaptic pathways. This interictal system appears to be partly independent of the hippocampal interictal system in complex partial epilepsy.
Cerebral Cortex↗
Nonepileptic seizures and childhood sexual and physical abuse.
Nonepileptic seizures (NES) must be distinguished from epilepsy to avoid the adverse effects of unnecessary antiepileptic drugs and to initiate appropriate psychiatric treatment. A higher frequency of prior sexual abuse has been suspected in NES, although no prospective controlled study has compared patients with NES and epilepsy. A series of patients with conversion disorder presenting as epilepsy and 140 patients with complex partial epilepsy (CPE) without evidence of conversion were selected from a series of consecutive admissions to a comprehensive epilepsy center. The groups did not differ with respect to age, years of education, race, or marital status, but the percentage of women was greater in the conversion NES group (73.2%) than in the CPE control group (50.7%; p < 0.002). The frequency of a history of sexual or physical abuse was greater in the NES group (32.4%) than in the CPE controls (8.6%; p < 0.000). Severity of sexual but not physical abuse was significantly greater in the NES group relative to controls (p < 0.05). There was a trend for a closer relationship of the perpetrator of sexual abuse to the victim among the NES patients compared with CPE controls (p < 0.1). These results support the impression that childhood abuse is more common among patients with conversion NES than with epilepsy, and suggests that in some cases childhood abuse may be a contributory pathogenetic factor.
Adult↗