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Congenital varicella syndrome: case with isolated brain damage.

We report a case of congenital varicella syndrome after maternal infection during the 10th week of pregnancy. None of the typical abnormalities were manifested at birth; however, the child experienced neonatal tonic convulsions that evolved to refractory focal epilepsy with adversive, complex partial, and secondarily generalized seizures. At the age of 5 years, after a prolonged generalized seizure following cranial trauma, cranial computed tomography (CT) was performed. It led to the misdiagnosis of post-traumatic intracerebral hemorrhage. Afterwards, because of persistent seizures and developmental delay with mild or slight intellectual deficit, the next CT and magnetic resonance imaging (MRI) investigation at 8 years of age disclosed massive malformation of the left cerebral hemisphere. The serologic evidence of varicella-zoster virus-specific IgG antibodies without a history of varicella disease after birth and maternal infection during early pregnancy were crucial to diagnosing the rare syndrome of congenital varicella consisting of isolated brain damage.

Brain↗

Reduction of rapid eye movement sleep by diurnal and nocturnal seizures in temporal lobe epilepsy.

BACKGROUND: Patients with brief, complex partial seizures frequently suffer from tiredness and decreased productivity that continue well beyond the postictal period. A possible explanation is that seizures, even when occurring during the day, disrupt sleep the following night. OBJECTIVE: To determine the effect of temporal lobe complex partial seizures on sleep structure and daytime drowsiness. METHODS: Patients with temporal lobe epilepsy were admitted for video-electroencephalography monitoring. All-night polysomnography was recorded under the following 3 conditions: seizure free, seizure during the day before the recording, and seizure during the recording. Percentage of time in each sleep stage, sleep efficiency, and time to first and second rapid eye movement (REM) period were compared for seizure vs control conditions. Daytime drowsiness was also measured, using a modified maintenance of wakefulness test and 2 subjective drowsiness tests. RESULTS: Daytime seizures reduced REM from 18%+/-1% to 12%+/-2% (P = .003). Night seizures reduced REM from 16%+/-1% to 6.8%+/-2% (P<.001). Night seizures also significantly reduced stages 2 and 4 while increasing stage 1 sleep. Night seizures, but not day seizures, significantly reduced sleep efficiency, increased time to first REM period, and increased drowsiness as measured by the maintenance of wakefulness test. CONCLUSIONS: Temporal lobe complex partial seizures decrease REM sleep, particularly when occurring during sleep but also when occurring on the previous day. This may, in part, be responsible for the prolonged impairment of functioning that some patients report following seizures.

Adult↗

Anticonvulsant and antiepileptogenic effect of L-deprenyl (selegiline) in the kindling model of epilepsy.

L-Deprenyl (selegiline) is an irreversible inhibitor of monoamine oxidase type B, but also exerts several effects on dopamine and noradrenaline systems independent of monoamine oxidase type B inhibition. Thanks to these properties, L-deprenyl has gained wide acceptance in the therapy of Parkinson's disease by using L-deprenyl both with levodopa and alone. Furthermore, L-deprenyl improves the performance of patients with Alzheimer's disease. Epilepsy, particularly temporal lobe epilepsy with complex-partial seizures, is often associated with disturbances of cognitive function and behavior, and it has been suggested that a drug combining cognition-enhancing and antiepileptic activity would be of benefit in the treatment of epileptic patients. This prompted us to study if L-deprenyl exerts anticonvulsant efficacy in amygdala-kindled rats, i.e., a useful model of complex-partial seizures in humans. In addition to anticonvulsant activity, i.e., effects on already developed seizures, we determined whether L-deprenyl exhibits antiepileptogenic properties, i.e., suppressive effects on development of kindling. In all experiments, behavioral alterations of the rats in response to L-deprenyl were monitored closely. In order to assess the role of active metabolites in the anticonvulsant and behavioral effects of L-deprenyl in the kindling model, the D-enantiomer of deprenyl, which is metabolized to more potent compounds (D-amphetamine and D-methamphetamine) than the L-enantiomer, was used for comparison. In fully kindled rats, L-deprenyl potently increased the threshold for focal afterdischarges. The most marked increase in afterdischarge threshold (up to 250% above control) was seen after a dose of 10 mg/kg, whereas the D-enantiomer was ineffective at this dosage.(ABSTRACT TRUNCATED AT 250 WORDS)

Animals↗

Mu-opiate receptors measured by positron emission tomography are increased in temporal lobe epilepsy.

Neurochemical studies in animal models of epilepsy have demonstrated the importance of multiple neurotransmitters and their receptors in mediating seizures. The role of opiate receptors and endogenous opioid peptides in seizure mechanisms is well developed and is the basis for measuring opiate receptors in patients with epilepsy. Patients with complex partial seizures due to unilateral temporal seizure foci were studied by positron emission tomography using 11C-carfentanil to measure mu-opiate receptors and 18F-fluoro-deoxy-D-glucose to measure glucose utilization. Opiate receptor binding is greater in the temporal neocortex on the side of the electrical focus than on the opposite side. Modeling studies indicate that the increase in binding is due to an increase in affinity or the number of unoccupied receptors. No significant asymmetry of 11C-carfentanil binding was detected in the amygdala or hippocampus. Glucose utilization correlated inversely with 11C-carfentanil binding in the temporal neocortex. Increased opiate receptors in the temporal neocortex may represent a tonic anticonvulsant system that limits the spread of electrical activity from other temporal lobe structures.

Adolescent↗

Hippocampal volumetrics differentiate patients with temporal lobe epilepsy and extratemporal lobe epilepsy.

OBJECTIVE: To determine whether the occurrence of hippocampal formation (HF) volumetric asymmetry can reliably discriminate between complex partial seizures (CPSs) of a temporal lobe origin and CPSs of an extra-temporal lobe origin in a prospective study of patients with intractable CPSs (approximately 70% of patients have electrographic foci in the temporal lobe [HF volumetric asymmetry on magnetic resonance imaging scans has been shown to lateralize such foci reliably)]. DESIGN: We examined HF volumetrics on magnetic resonance imaging scans that were acquired with a 1-T magnetic resonance imaging scanner (Siemens Magnetom, Siemens Medical Systems, Iselin, NJ) by using magnetization-prepared rapid gradient echo three-dimensional sequences (producing a gapless series of high-contrast 1.25-mm images). These data were compared with ictal, interictal, invasive, and noninvasive videoelectroencephalographic monitoring data, functional imaging data, and outcome data to define each patient's type of epilepsy. SETTING AND PATIENTS: Forty-one patients were recruited from a tertiary university comprehensive epilepsy program, and 22 control subjects were recruited from the neurologically normal university community. RESULTS: Among the control subjects, the difference in HF volumetrics (right-left HF volumetrics) was + 0.085 +/- 0.253 cm3. Of the 41 patients, 25 had temporal lobe epilepsy. When we set the upper limit of normal at the mean +/- 3 SDs, all patients beyond the upper limit had CPSs of a temporal lobe origin. Of the patients with temporal lobe epilepsy, only three fell within normal limits. No patient with CPSs of an extratemporal lobe origin fell beyond the upper limit. CONCLUSION: The presence of significant HF volumetric asymmetry makes it highly unlikely that a patient's CPSs are of an extratemporal lobe origin.

Adolescent↗

Effect of sleep on the electrographic manifestations of epilepsy.

Alterations in the level of arousal have profound predictable effects on the electrographic manifestations of epileptogenic abnormalities. The changes produced by sleep are dramatic in patients with generalized epilepsy as compared to patients with partial epilepsy. The epileptiform complexes associated with the generalized epilepsies show changes in spatial distribution, voltage, temporal sequencing, and waveform morphology. The changes are qualitatively similar in the different types of generalized seizure disorders but vary in the degree to which they are expressed in proportion to the "severity" of the seizure disorder. The presence of a generalized epileptogenic abnormality may result in alteration of K-complex waveform morphology. Multifocal spikes occurring in conjunction with bilateral synchronous discharges are frequently represented by a spatial field distribution consistent with a "horizontal dipole," during sleep especially in children.

Adult↗

[Stuporous state in an epileptic treated with sodium valproate. Diagnostic problems].

The authors report a case of stupor secondary to valproic acid, in a patient treated with this drug and phenobarbital for a complex partial form of epilepsy. The pathogeny of this rare complication is discussed. In this patient, a paradoxical epileptic reactivation is excluded, and the authors favor a metabolic mechanism: either hyperammoniemia or a perturbation in cerebral neuro-transmitter systems are proposed.

Adult↗

Temporal lobe epilepsy caused by dermoid cyst.

A 15-year-old boy presented with a dermoid cyst in the left temporal lobe manifesting as complex partial seizures. Magnetic resonance imaging demonstrated a tumor with mixed signal intensity in the left anterior temporal subdural area, but no evidence of rupture. Intraoperatively, the tumor was located mainly in the deep sylvian fissure, adjacent to the amygdala, and had compressed the hippocampus. Intraoperative electrocorticography (ECoG) showed sporadic interictal spikes in both the adjacent areas of the tumor and over the anterior segment of the hippocampus. Total removal of the tumor and gliotic area of the surrounding tissue including the amygdala was performed. The hippocampal epileptic region was treated by transection of the pyramidal layer to preserve verbal memory function. Histological examination showed the dermoid tumor was closely attached to the brain parenchyma. The complex partial seizures ceased completely after surgery. Intraoperative recording of ECoG from the hippocampus and other limbic structures was very important to determine the epileptogenic area even if the tumor did not directly invade the hippocampus.

Adolescent↗

Social competence in pediatric epilepsy: insights into underlying mechanisms.

This study compared parent-based Child Behavior Checklist (CBCL) social competence scores of 90 children with complex partial seizures (CPS) and 62 with absence epilepsy (CAE) of average intelligence with scores of 91 healthy children. It also examined the role of seizure-related, cognitive, behavioral, linguistic, social communication, and demographic variables on these measures. When differences in cognitive, linguistic, and demographic variables were controlled for, the CPS and CAE groups had significantly lower scores in the school, but not in the social interaction and activities domains compared with the healthy control group. Among the patients, lower Full Scale IQ externalizing behaviors, disruptive disorders, minority status, and impaired social communication, but not seizure variables, predicted lower social competence scores. These findings demonstrate the importance of controlling for cognitive, behavioral, and demographic variables in social competence studies of children with CPS and CAE and the need to assess cognition and behavior when parents report school and social problems in these children.

Achievement↗

Vigabatrin as add-on therapy for adult complex partial seizures: a double-blind, placebo-controlled multicentre study. The Canadian Vigabatrin Study Group.

Vigabatrin (VGB) is a novel antiepileptic drug effective as adjunctive therapy in patients with partial seizures. In this study, the efficacy and tolerability of VGB as adjunctive therapy were evaluated in patients with refractory epilepsy. Adult patients with a definite diagnosis of complex partial seizures and/or partial seizures secondarily generalized were recruited from 10 Canadian centres. Patients were randomized to receive either active medication or placebo in a double- blind fashion and entered a 36-week titration and maintenance phase with regularly scheduled visits. Both efficacy parameters and safety assessments were monitored. Clinical laboratory, evoked potential studies, MRI, and neuropsychological tests were also performed. Forty-eight percent of VGB-treated patients vs. 26 percent of placebo-treated patients had a 50 percent or greater reduction in the frequency of complex partial seizures and partial seizures secondarily generalized. Vigabatrin was well tolerated by the majority of patients. Minor neurological side effects were observed in a number of patients in both treatment groups. No serious systemic toxicity was observed. No changes in evoked potential studies or MRI findings were noted. Vigabatrin was found to be an effective and well-tolerated antiepileptic drug when used as adjunctive therapy in patients with difficult to control complex partial seizures and for partial seizures secondarily generalized. Vigabatrin is a selective irreversible inhibitor of the GABA- degradating enzyme GABA transaminase and has shown efficacy in a number of clinical trials in patients with difficult to control partial seizures. Vigabatrin has been found most effective against complex partial and secondarily generalized tonic-clonic seizures in both adults and children. Vigabatrin has also been shown to reduce infantile spasms secondary to various aetiologies and is most effective in spasms associated with tuberous sclerosis. The aim of this study was to further extend the clinical experience with VGB as adjunctive therapy in the treatment of adult patients with difficult to control complex partial seizures and/or partial seizures secondarily generalized. In addition to the assessments of efficacy and tolerability to VGB, neuropsychological evaluations were also carried out.

Adolescent↗

Microwave oven injuries in patients with complex partial seizures.

Microwave ovens are often recommended as a safe cooking alternative for persons with epilepsy. We report four patients who suffered serious burns to their hands while handling microwave-heated liquids during a complex partial seizure (CPS). Injuries were due to the contact of the skin with a very hot container. The fact that all patients held on to the hot containers despite being burned and that they did not remember experiencing any pain at the time of the accident indicates that neither high temperatures nor pain will prevent patients who are having a CPS from suffering this type of injury. Unfortunately, there is no foolproof way to prevent the individual from opening the oven and removing its contents during a CPS. The only solution for this problem is "prevention"-individuals with poorly controlled CPS should be cautioned about these risks. The use of microwave settings that permit the heating but not boiling of liquids and the use of gloves while heating food and liquids to scalding temperatures may minimize the risk of this type of injury.

Accidents, Home↗

Profile of epilepsy in a developing country: a study of 1,000 patients based on the international classification.

The present prospective study undertaken in a specialized neurological center of a developing country deals with 1,000 epileptic patients classified in accordance with the International Classification. Eighty-one percent of the patients could be classified, with a lower incidence in the younger age group. Partial epilepsy was found to be far more common than generalized epilepsy (80% versus 20%). Primary generalized epilepsy was seen in 15% and secondary generalized in 5%. Partial epilepsy with elementary symptomatology was seen in 58% and complex symptomatology in 7%. Secondarily generalized seizures were seen in the remaining 15%. Primary generalized epilepsy and partial epilepsy with complex symptomatology were more common in adults. Secondary generalized epilepsy and partial epilepsy with secondarily generalized seizures were more common in children. Partial epilepsy with elementary symptomatology, however, did not vary significantly with age. The higher incidence of partial epilepsy in our patients, compared to the West, could be due to greater frequency of CNS infections and birth injuries, which are common childhood hazards in the developing countries.

Adult↗

Epilepsy in my life.

The author presents a personal account of his battle with the problem of complex partial seizures (temporal lobe epilepsy). This exposure and knowledge obtained from the tales of colleagues in a support group convinced him that the emotional side of epilepsy is not properly understood.

Epilepsy, Temporal Lobe↗

Conversational repair in pediatric epilepsy.

This study examined if children with complex partial seizures disorder (CPS) and primary generalized epilepsy with absence (PGE) were impaired in the use of self-initiated repair during a conversation compared to normal children. Transcriptions of speech samples of 92 CPS, 51 PGE, and 65 normal children, ages 5-16 years, were coded for self-initiated repair according to Evans (1985). The WISC-R, a structured psychiatric interview, and seizure-related information were obtained for each child. We found impaired use of repair in both the CPS and PGE groups compared to the normal subjects. The CPS patients, particularly those with a temporal lobe focus, overused self-initiated corrections of referents and syntax compared to the PGE and normal subjects. The CPS and PGE patients with frontal lobe involvement underused fillers compared to the normal children. These findings provide additional evidence that both CPS and PGE impact the ongoing development of children's communication skills.

Adolescent↗

Interictal temporal hypoperfusion is related to early-onset temporal lobe epilepsy.

Previous studies of interictal regional cerebral blood flow (rCBF) in temporal lobe epilepsy have shown variable correlations with clinical measures. We used high spatial resolution hexamethyl propyleneamine oxime single photon emission computed tomography (HMPAO SPECT) in 80 consecutive patients with complex partial seizures (CPS), comparing results with those from a large series of normal subjects. Visual image analysis detected abnormalities of rCBF in 41 of 80 (51%; numeric analysis detected abnormalities in 38 of 80). Age at epilepsy onset was significantly younger in patients with temporal hypoperfusion (p = 0.002), and the frequency distribution of hypoperfusion versus age at epilepsy onset was reverse exponential. The results of numerical image analysis showed that degree of hypoperfusion did not vary with age at epilepsy onset. These data suggest a single insult operating early in life as a cause of temporal hypoperfusion, as has been shown for mesial temporal sclerosis (MTS). We could not demonstrate relationships with other clinical variables, including time since last seizure.

Adolescent↗

Initial experience with SPECT imaging of the brain using I-123 p-iodoamphetamine in focal epilepsy.

Nineteen patients with complex partial seizures refractory to medical treatment were examined with routine electroencephalography (EEG), video EEG monitoring, computed tomography or magnetic resonance imaging, neuropsychological tests and interictal single photon emission computed tomography (SPECT) with I-123 iodoamphetamine (INT). In 18 patients, SPECT identified areas of focal reduction in tracer uptake that correlated with the epileptogenic focus identified on the EEG. In addition, SPECT disclosed other areas of neurologic dysfunction as elicited on neuropsychological tests. Thus, IMP SPECT is a useful tool for localizing epileptogenic foci and their associated dynamic deficits.

Adolescent↗

A relationship between particular reproductive endocrine disorders and the laterality of epileptiform discharges in women with epilepsy.

Cerebral limbic structures modulate the endocrine function of the hypothalamopituitary axis in both animals and humans. In an investigation of 30 women who had complex partial seizures with unilateral temporal lobe epileptiform discharges and reproductive endocrine disorders, there was a significant difference between the EEG laterality distributions associated with polycystic ovarian syndrome (PCO) and those associated with hypogonadotropic hypogonadism (HH). There was a strong predominance of left-sided discharges (15 versus one) with PCO, and right-sided discharges (12 versus two) with HH. Each distribution differed significantly from that of 30 women with epilepsy who had no reproductive endocrine disorder (left/right: 17/13). Moreover, among women with PCO who had unilateral non-temporal lobe foci, six of seven had right-sided epileptiform discharges. This represents a significant difference from the EEG laterality distribution among women with PCO who had temporal foci. These relationships between altered patterns of reproductive hormonal secretion and the predominant laterality of EEG epileptiform discharges in women with epilepsy are consistent with a lateralized asymmetry in cerebral influences on reproductive endocrine function.

Adult↗

Ictal EEG in benign partial epilepsy in infancy.

The aim of this study is to further clarify ictal electroencephalographic findings of patients with benign partial epilepsy in infancy in order to better understand its neurophysiologic features. The study group consisted of 13 infants with definite benign partial epilepsy in infancy, in whom ictal electroencephalograms were recorded and its benignity was confirmed at 8 years or more. The seizure manifestation was reviewed on the basis of video findings in eight patients in whom simultaneous video-electroencephalography recording was available. In the other five patients, the seizure manifestations were determined according to the observations of physicians, nurses, or technicians. Thirteen seizures from eight patients were complex partial, and six seizures from six patients were secondarily generalized ones. Ictal discharges at the onset of a seizure were focal in all seizures. The site of the origin of seizures was in the temporal area in 10 of 13 complex partial seizures, whereas it was in the parietal or occipital area in all 6 secondarily generalized seizures. Among 13 complex partial seizures, paroxysmal discharges remained focal throughout the seizures in 6 seizures, whereas they spread to one hemisphere in the other 7 seizures. Motion arrest or decreased responsiveness was uniformly observed. Lateral eye deviation was commonly recognized in complex partial seizures, whereas head rotation was observed only in seizures in which hemispheric propagation of ictal discharges was observed. Ictal electroencephalographic findings of patients with benign partial epilepsy in infancy were relatively uniform, suggesting the homogeneity of patients with benign partial epilepsy in infancy.

Aged↗