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[Benign epilepsy in children and adolescents].

THE CONCEPT OF BENIGN EPILEPSY: The benign nature of epilepsy is difficult to establish. The diagnosis of epilepsy often has an effect disproportionate to the severity. In everyday practice, most cases of epilepsy follow a benign course. A benign evolution is the rule in certain syndromes which are usually diagnosed early. In other cases however, the benign prognosis cannot be determined except after long-term follow-up. BENIGN EPILEPSY: There are 3 large categories of benign epilepsy: partial idiopathic epilepsy, generalized idiopathic epilepsy, occasional seizures. They are particularly frequent in children and adolescents, but can be observed in the neonatal period and in older patients. DIAGNOSIS OF BENIGN EPILEPSY: A syndromic approach to the problem is required. The patient's history, clinical signs, electroencephalographic data, and in certain cases neuroimaging findings are required for diagnosis. Confirmation of the benign nature of the epilepsy is reassuring for the patient and the family and can avoid unnecessary explorations. The appropriate therapeutic attitude is usually abstention from long-term antiepileptic treatment. It is importance for all clinicians to be familiar with "benign" epilepsy.

Adolescent↗

Exceptionally long absence status: multifactorial etiology, drug interactions and complications.

To our knowledge, petit mal status lasting longer than 2-3 days has been documented only once [1]. We report a 66 year-old man with well-documented, idiopathic generalized epilepsy who developed petit mal status lasting 6 weeks. Valproate levels remained low because of interactions with phenytoin. When phenytoin was discontinued, valproate levels increased, and he progressively improved. Chronic ischemic changes in the white matter may have been an additional factor in the causation and, most likely, in the duration of his status. Exceptionally long status and stupor increase the risk of medical complications. Valproate remains the medication of choice for the treatment of petit mal status. Despite the extraordinary course in this patient, complete recovery took place, confirming the benign nature of even such a prolonged episode.

Aged↗

[Staged total callosotomy for medically intractable seizures].

For the treatment of intractable generalized epilepsies, two-staged total callosotomy was performed in five patients. In all patients, preceded anterior callosotomy failed to obtain satisfactory seizure control. All patients showed mental retardation with various degrees. Mean age at the first operation was 10.2 years and 4 patients were operated in their childhood. All patients showed various types of seizures; drop attack (DA) in 3 patients, tonic seizure (TS) in 2, myoclonus (MY) in 2, complex partial seizure (CPS) in 2, atypical absence (AA) in 1, and head drop (HD) in 2. After anterior callosotomy, complete cessation of CPS and 50-80% reduction of DA was obtained in one, respectively. However, only less than 50% reduction of seizures was obtained in other types of seizures. Two years after anterior callosotomy, posterior portion of the corpus callosum was divided. After staged total callosotomy, complete cessation of DA was obtained in all patients and 80-100% reduction of AA was obtained in one patient. One adult patient showed the disconnection syndrome which did not affect activities of his daily life. Our study revealed the efficacy of posterior callosotomy in DA patients with unsatisfactory results after anterior callosotomy. This strategy should be considered especially in childhood cases, since obvious complication was not observed in such cases.

Adolescent↗

[Giant evoked potentials].

INTRODUCTION: There are many, diverse nosological entities with the common factor of the genesis of cortical evoked potentials of great amplitude, commonly known as giant evoked potentials. In most cases they are conditions with the common clinical condition of myoclonic of cortical origin, such as progressive myoclonic epilepsy, generalized idiopathic epilepsy, myoclonias of toxic, infectious or postanoxic origin. Giant potentials have been shown both in studies of focal hemisphere lesions and in some cases of patients with corticobasal degeneration. OBJECTIVE: The aim of this paper was to show, by presenting interesting cases, some of the conditions mentioned and to review some concepts concerning the mechanisms which may be involved in the production of these electrophysiological responses. PATIENTS AND METHODS: We studied 6 patients aged between 2 and 22 years, in whom multimodal evoked potentials, electroencephalograms and imaging studies had been done. RESULTS: Giant somatosensory potentials were shown in the patients with obvious myoclonia. Visual potentials of great amplitude were common to the other patients presented, with or without myoclonia. CONCLUSION: Giant evoked potentials respond to a state of cortical hyperexcitability which may have various causes.

Adolescent↗

[Symmetrical hemispheric porencephalic cysts due to twin embolization syndrome: a case report].

Twin embolization syndrome (TES) is a complication of monozygotic twining following in utero demise of the co-twin. Passage of thromboplastic materials into the circulation of the surviving fetus results in ischemic structural defects of various systemic organs including the central nervous system (CNS). In our case of monozygotic twining prenatal sonography at the 18th week of gestation revealed intracranial abnormality and demise of the co-twin. Postnatal MRI demonstrates localized cerebral parenchymal defects (porencephalic cysts) in the bilateral hemispheres, which probably were the sequelae to the occlusion of the peripheral branches of the bilateral middle cerebral arteries. She developed physio-mental retardation and subsequently West syndrome. At the 6th months, right porencephalic cyst-peritoneal shunt was performed for progressive enlargement of the head. While the enlargement of the head was well controlled, no changes in her epileptic symptoms were noted. The development of generalized epilepsy in our case may indicate that the involvement of the CNS with TES in our case is not restricted to the porencephalic cysts and their surrounding areas.

Brain↗

[The trigger role of the left cerebral hemisphere in generalizing epileptic activity].

Overall 593 epileptic patients with left hemispheral lateralization of the focus and 46 patients with primary generalized epilepsy were examined. EEG and computerized mapping of cortical biopotentials were used. It has been shown that in right-handed patients, the left hemisphere triggers epileptogenesis whatever the site of the source of epileptic activity. The mechanism by which epileptogenesis is triggered includes the posterotemporal and central cortical areas of the left hemisphere.

Adolescent↗

[Non-convulsive status epilepticus].

DEVELOPMENT: Non-convulsive status epilepticus (NCSE) is an epileptic condition lasting more than 30 minutes in which continuous or recurrent seizure activity on the electroencephalogram (EEG) is responsible for diverse clinical symptoms including alteration of mental state, abnormal behaviour, perception disturbances or consciousness impairment. Most authors recognize two types of NCSE depending on the ictal electroencephalographic changes: generalized (absence status epilepticus) and complex partial status epilepticus (CPSE). In addition, there is also a simple form of partial status which is likely underdiagnosed because of its frequent progression to CPSE, subtle and varied symptomatology and high proportion of normal electroencephalograms (EEG). Absence status epilepticus (ASE) may occur in different clinical situations including idiopathic generalized epilepsy (typical AS), symptomatic generalized epilepsy (atypical AS), and elderly subjects without antecedents of epilepsy on chronic treatment with psychotropic drugs (de novo AS of late onset). Furthermore, CPSE is more frequent than previously mentioned. Although initially was equated with temporal lobe status epilepticus, extratemporal cases of CPSE have been well-documented, and the electroclinical characteristics of two types (I and II) of frontal CPSE have been recently described. NCSE may occur in patients with diverse clinical diagnosis such as hypoxic-anoxic encephalopathy, cancer, drugs, autoimmune disorders, Creutzfeldt-Jakob disease, chromosomal alterations, peritoneal dialysis, infections, cerebral hamartomas or head trauma. CONCLUSION: The identification of NCSE may be particularly arduous and, therefore, a high level of suspicion is essential to obtain an early diagnosis. An urgent EEG with administration of intravenous benzodiazepines is considered as the method of choice in the diagnostic evaluation of NCSE.

Anticonvulsants↗

[International classification of epileptic seizures, epilepsies, and epileptic syndromes].

Epilepsy is one of the common diseases in neurology. Its correct diagnosis and classification are important for choosing the most appropriate treatment. Currently, Classification of Epileptic Seizures in 1981 and Classification of Epilepsies and Epileptic Syndromes in 1989, both were proposed by International League Against Epilepsy (ILAE), are available. As for Classification of Epilepsies and Epileptic Syndromes, two divisions continue to be widely used to shape the major classes: The first separates epilepsies with generalized seizures (generalized epilepsy) from epilepsies with partial or focal seizures (localization-related, partial or focal epilepsies). The other separates epilepsies of known etiology (symptomatic or secondary epilepsies) from those that are idiopathic (primary) and those that are cryptogenic. In 2001, ILAE Task Force proposed a new classification. However, its daily use has yet been limited. Simple and useful classification will lead to better understanding and more appropriate management of epilepsy.

Epilepsy↗

[Lissencephaly. Clinical, electroencephalographic and radiological aspects].

We report 2 cases of lissencephaly and review the cardinal symptoms: microcephalia, mental retardation, pyramidal syndrome and generalized epilepsy. CT-Scan and MRI show the anatomical abnormalities of this malformation: microcephalia, smooth cortex reducing the surface of white matter and lack of sulci and gyri. The fact that the myelin is normal on MRI suggests that this major disorder of neuronal migration does not come from radial glial fibers.

Cerebral Cortex↗

[Results of monotherapy in mild and drug-resistant forms of epilepsy].

Monotherapy with one drug chosen out of basic anti-epileptic agents was carried out in 312 patients, including 2/3 of cases resistant to the drugs. Group A consisted of 110 patients treated with a single drug since the onset of the disease whereas group B consisted of 202 patients in whom polytherapy was reduced to monotherapy. The best results were achieved in patients of group A with short and medium duration of the disease. The results noted in patients of group B were worse and independent of the duration of epilepsy. The most favourable effects were noted in children with mild epilepsy and generalized epilepsy of unknown post-traumatic etiology. The results of monotherapy in focal seizures and epilepsy with organic lesions were markedly worse. The most effective drugs in this population were: barbiturates, valproic acid derivatives, and carbamazepine. The authors stress that all forms of epilepsy, especially newly diagnosed and previously untreated cases, should be treated with single agent.

Adolescent↗

[The clinical picture and electroencephalographic changes in patients with multifocal epilepsy depending on the location of the epileptic foci].

As many 116 patients were examined for the clinical course and electroencephalographic changes as well as for the x-ray, CT and NMR-tomography data, on the basis of which different forms of multifocal epilepsy were distinguished: with unilateral epileptic foci (cortical and cortico-subcortical form), bitemporal and with multiple bilateral epileptic foci. The data provided make it possible to differentiate between multifocal epilepsy and generalized epilepsy and to perform surgical treatment using the optimal surgical intervention.

Adolescent↗

The role of vigabatrin in the management of infantile epileptic syndromes.

More than 360 children with intractable epilepsy have been treated with vigabatrin in single-blind or open, add-on studies. Approximately 50% or more of patients with West syndrome and partial seizures have shown a 50% or greater reduction in seizure frequency with the use of vigabatrin. A less consistent response has been found between studies evaluating vigabatrin in children with Lennox-Gastaut syndrome, although, overall, approximately 50% of these patients have also shown a greater than 50% decrease in seizures. The use of vigabatrin in idiopathic localization-related epilepsy, idiopathic generalized epilepsy, and the Landau Kleffner syndrome have not been reported, but its evaluation in these conditions may be warranted based on the relatively excellent safety profile of vigabatrin. Vigabatrin has been shown to aggravate "nonprogressive myoclonic epilepsies." Vigabatrin has been well tolerated in children, with mild drowsiness and agitation being the most commonly reported side effects.

Aminocaproates↗

[Two cases whose epileptic attacks disappeared at the time of infection].

Two cases with secondary generalized epilepsy whose epileptic attacks disappeared abruptly were reported. Permanent improvement was observed in case 1 with residual state of West syndrome, aged 4 years, after viral pneumonia, both epileptic attacks and epileptic discharges on EEG disappeared more than 3 years. Transient improvement was observed in case 2 with Lennox-Gastaut syndrome, aged 6 years, after an episode with insomnia, poor appetite and confusion for three days. After this episode epileptic attacks disappeared for 9 months and epileptic discharges on EEG were also markedly decreased. However, epileptic attacks relapsed following the reduction of phenytoin after 9 months. An effect resembling ketogenic diet therapy and a change of pharmacokinetics of antiepileptic drugs may be involved in the transient improvement of case 2. However, any effects involving an immunological effect and secretions of adreno-cortico-hormone following after stress may be not involved in the permanent improvement of case 1.

Anticonvulsants↗

[Early infantile epileptic encephalopathy and glycine encephalopathy].

INTRODUCTION: Early infantile epileptic encephalopathy (EIEE) with suppression burst activity in EEG (Ohtahara syndrome) is a rare type of epileptic encephalopathy in infancy and represents the earliest type of age-related symptomatic generalized epilepsy. The main etiologic factors associated to EIEE are cerebral dysgenesia and metabolopathies, principally nonketotic hyperglycinemia. CLINICAL CASE: We report a neonate with EIEE secondary to glycine encephalopathy, diagnosed by increased of LCR/plasma glycine index.

Brain Diseases↗

[Epilepsies during the first year of life].

OBJECTIVE: An important number of epileptic patients who began with epileptic seizures during the first year of life have not just been well classified. The objective is to identify the different types of epilepsies and epileptic seizures which begin during the first year of life, according to the last classification of epilepsy and epileptic syndromes of 1989. MATERIAL AND METHODS: We have studied 471 patients who consulted to our service in the last 5 years, with epileptic seizures during the first year of life. We excluded neonatal seizures and febrile convulsions. RESULTS: 1. Partial epilepsy: a) idiopathic: 12 (2.5%); b) symptomatic: 130 (28%); c) cryptogenic: 25 (5%). 2. Generalized epilepsy: a) idiopathic: benign myoclonic epilepsy in infancy, 6 (1%); b) cryptogenic-symptomatic: symptomatic West syndrome, 155 (33%); cryptogenic West syndrome, 65 (14%); cryptogenic myoclonic epilepsy, 6 (1%); early infantile epileptic encephalopathy, 8 (1.6%); early myoclonic encephalopathy, 4 (0.8%); other symptomatic generalized epilepsies without specific aetiology, 14 (3%). 3. Epilepsies and syndromes undetermined as to whether they are focal or generalized: a) severe myoclonic epilepsy in infancy, 15 (3%); symptomatic epilepsy with multifocal and independent spikes, 3 (0.6%); other undetermined epilepsies not defined above, 3 (0.6%). 4. Special syndromes: occasional convulsions, 15 (3%). CONCLUSIONS: West syndrome is the commonest type of epilepsy during the first year of life, particularly symptomatic West syndrome. Symptomatic partial epilepsies are in the second place in frequency. We identified 12 patients with idiopathic partial epilepsy, who full-filled clinical and EEG characteristics of a recently described epilepsy: benign infantile familial convulsions. The study showed the predominance of symptomatic epilepsy, generalized (represented by West syndrome) and partials. Finally 6% of the patients have a generalized epilepsy, cryptogenic or symptomatic, not defined in the classification.

Epilepsy↗

GABA(A) receptor epilepsy mutations.

Idiopathic generalized epilepsy (IGE) syndromes are diseases that are characterized by absence, myoclonic, and/or primary generalized tonic-clonic seizures in the absence of structural brain abnormalities. Although it was long hypothesized that IGE had a genetic basis, only recently have causative genes been identified. Here we review mutations in the GABA(A) receptor alpha1, gamma2, and delta subunits that have been associated with different IGE syndromes. These mutations affect GABA(A) receptor gating, expression, and/or trafficking of the receptor to the cell surface, all pathophysiological mechanisms that result in neuronal disinhibition and thus predispose affected patients to seizures.

Epilepsy↗

Electroclinical and magnetoencephalographic analysis of epilepsy in patients with congenital bilateral perisylvian syndrome.

PURPOSE: Epilepsies in four patients with congenital bilateral perisylvian syndrome (CBPS) were studied electroclinically and by magnetoencephalography (MEG) to determine whether cortical dysplasia associated with this syndrome is epileptogenic. METHODS: We studied three women and one man (mean +/- SD age 30.8 +/- 3.4 years) with a diagnosis of CBPS based on magnetic resonance imaging (MRI). All had drug-resistant epilepsy. In addition to electroclinical analysis, the distribution of equivalent-current dipoles (ECDs) analyzed from MEG signals was examined. RESULTS: Patient 1 had left-sided parietal lobe epilepsy and ECDs clustered in the left temporoparietal lobe. Patient 2 had bilateral, independent temporoparietal lobe epilepsy, and ECDs were found in both hemispheres, with clustering in the right temporoparietal lobe but not in the left hemisphere. Patient 3 presumably had parietal lobe epilepsy with unidentified focus side and ECDs clustered in the left parietal and right temporal lobes. Patient 4 had symptomatic generalized epilepsy, and ECDs were observed in both hemispheres without clustering. In the three patients who had partial epilepsy (patients 1-3), clusters of ECDs overlaid the perisylvian dysplasia and were not found elsewhere. CONCLUSIONS: Patients with CBPS may have either symptomatic partial epilepsy or symptomatic generalized epilepsy, and those with partial epilepsy are likely to have temporoparietal foci. In addition, cortical dysplasia is closely related to the generation of partial seizures and may be epileptogenic per se. However, this study did not elucidate the relationship between cortical dysplasia and CBPS-generated generalized epilepsy.

Adult↗