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PubMed1999

Photosensitive epilepsies and pathophysiologic mechanisms of the photoparoxysmal response.

OBJECTIVE: To identify the pathophysiologic mechanisms of the photoparoxysmal response (PPR) in various photosensitive epilepsy syndromes, and to discuss the relation between these pathophysiologic mechanisms and the classification of epilepsies and epileptic syndromes. BACKGROUND: The authors found two types of pathophysiologic mechanisms of PPRs (wavelength-dependent PPRs and quantity-of-light-dependent PPRs) in patients with idiopathic generalized epilepsy and hereditary dentatorubral-pallidoluysian atrophy. METHODS: Intermittent photic stimulation with wavelength-specific optical filters was performed in photosensitive epileptic patients: six patients had severe myoclonic epilepsy in infancy (SMEI), eight had localization-related epilepsy (LRE), and seven had symptomatic generalized epilepsy (SGE). RESULTS: Four of the six photosensitive SMEI patients had quantity-of-light-dependent PPRs. Five of the eight photosensitive LRE patients had wavelength-dependent PPRs. Four of the seven photosensitive SGE patients had wavelength-dependent PPRs, and two had quantity-of-light-dependent PPRs. CONCLUSIONS: The type of pathophysiologic mechanism for eliciting PPRs by low-luminance photic stimulation was closely related to the classification of the epilepsy syndrome.

Adolescent↗
PubMedDate not supplied

Abortive generalized spike and waves in children: a review.

Full-blown generalized spike and wave patterns in electroencephalogram lasting more than 3 seconds are diagnostically significant, suggesting one of the generalized epileptic syndromes. However, brief (less than 3 seconds) bursts of generalized spike waves activity are often difficult to evaluate the clinical correlations when such bursts are the only remarkable finding in a record which is otherwise within broad limits of normality. After reviewing the literature about the classification and genetic aspects of generalized spike and waves, we concluded that one or more of the following conditions will be the most possible to correlate with abortive generalized spike and waves: 1. as a interictal pattern of generalized tonic-clonic seizures. 2. simple or complex partial seizures. 3. generalized epilepsy under effective anticonvulsant treatment. 4. withdrawal from anticonvulsant therapy. 5. natural evolution of generalized spike waves. 6. drug induced. 7. a genetic trait of relatives of generalized epilepsy. 8. normal pattern.

Electroencephalography↗
PubMed1996

Cryptogenic partial epilepsies with anti-GM1 antibodies: a new form of immune-mediated epilepsy?

PURPOSE: We wished to study immune system dysfunction which has been proposed as a potential cause of epilepsy. Epileptogenic action of antibodies directed against GM1 gangliosides was demonstrated in rats, but the potential role of anti-GM1 antibodies in human epilepsy has not yet been studied. METHODS: We investigated the presence of IgG or IgM anti-GM1 antibodies in the sera of 64 patients with various types of epileptic syndromes: idiopathic generalized epilepsy (IGE) (n = 6), symptomatic or cryptogenic generalized epilepsy (SCGE) (n = 7), symptomatic partial epilepsy (SPE) (n = 26), and cryptogenic partial epilepsy (CPE) (n = 25). RESULTS: Two patients had elevated titers of IgM anti-GM1 antibodies, one patient had elevated titers of both IgM and IgG anti-GM1 antibodies, and 1 patient had elevated titers of IgG anti-GM1 antibodies. All 4 patients had complex partial seizures (CPS) secondarily generalized, drug resistance, psychiatric disorders, and normal brain imaging. Anti-GM1 antibodies were never associated with IGE, SCGE, or SPE. We compared the reactivity of sera from these patients with the sera from 5 patients with motor neuropathies with conduction block (MNCB) against different gangliosides and concluded that epilepsy sera did not react with the Gal(beta 1-3)GalNAc epitope. Two anti-GM1-positive patients were treated successfully with high-dose intravenous immunoglobulins (IgIV). CONCLUSIONS: Our findings suggest that detection of anti-GM1 antibodies could allow identification of a subgroup of patients with partial epilepsy associated with an autoimmune response. If anti-GM1 antibodies prove pathogenic, they could be an important prognostic factor for drug resistance and worsening of seizures.

Adult↗
PubMed2001

Incidence of epileptic discharge in various epileptic syndromes.

Three hundred eight patients with childhood and adolescent epilepsy were examined to clarify the incidence of epileptic discharges on initial and follow-up electroencephalograms. Epileptic discharges were found in 75.6% patients on the initial electroencephalogram, which is higher than figures previously reported for adults. The cumulative incidence of epileptic discharges was 92.3% by the third electroencephalogram recording. However, in 17.1% patients with nonspecific idiopathic generalized epilepsy, no epileptic discharges were found even after three electroencephalogram recordings. The incidence of epileptic discharges in patients with generalized epilepsy (84.3%) was significantly higher than in patients with localization-related epilepsy (71.6%). The incidence of epileptic discharges in patients with partial seizures was lower than those in patients with generalized seizures. The incidence of epileptic discharges was low in the 0- to 3-year-old and 15- to 20-year-old groups, and high in the 3- to 12-year-old groups. In the positive epileptic discharge patients, 38.8% of electroencephalograms were abnormal only during the waking or sleeping portion of the recordings. Knowing the incidence of epileptic discharges for each type of epilepsy will be useful in planning further electroencephalogram research and performing electroencephalograms in the clinical setting.

Adolescent↗
PubMed1985

Prevalence of epilepsy in Okayama Prefecture: a neuroepidemiologic study.

On the prevalence day, 2,378 epileptic children were identified. Therefore, the prevalence rate for epilepsy was 8.2 per 1,000. The lowest prevalence rate was 1.2 in children under one year of age, and the highest was 11.0 at five years of age. The rate was higher for males than females. The annual incidence rate for epilepsy was estimated at 145.0 per 100,000 for 1975. The onset of seizures was high in the first three years, totaling 1,795 cases (77.7%), and this decreased after four years of age. Primary generalized epilepsy was found in 577 cases (31.7%), secondary generalized epilepsy in 167 (9.2%), partial epilepsy with elementary symptomatology in 205 (11.3%), with complex symptomatology in 61 (3.3%) and partial seizures secondarily generalized in 801 (44.5%). The study on such a number of cases had hitherto never been reported in the world.

Age Factors↗
PubMed2000

Frontal nonconvulsive status epilepticus associated with high-dose tiagabine therapy in a child with familial bilateral perisylvian polymicrogyria.

PURPOSE: Antiepileptic drugs are known to exacerbate absence and myoclonic seizures, especially in patients with idiopathic generalized epilepsies. Exacerbation of nonconvulsive generalized seizures in patients with partial epilepsy is less common. Recently, however, a number of cases of putative generalized nonconvulsive status epilepticus (NCSE) or NCSE without further specification have been reported in patients with chronic partial epilepsy treated with the gamma-aminobutyric acid reuptake inhibitor tiagabine. Although complex partial status epilepticus during tiagabine therapy has also been reported, possible precipitation of NCSE specifically associated with frontal lobe discharges does not appear to have been recognized. In this communication, we describe the case of a boy with familial bilateral perisylvian polymicrogyria who developed frontal NCSE after being stabilized on high-dose tiagabine METHODS: A 12-year-old boy with familial bilateral perisylvian polymicrogyria, mental retardation, and refractory partial seizures was administered tiagabine in addition to sodium valproate. The tiagabine dosage was increased gradually up to 10 mg t.i.d. (1 mg/kg per day), resulting in complete seizure control. RESULTS: After 1 week on maintenance treatment, seizures were completely controlled, but the child developed hypoactivity, decreased reactivity, and affective detachment. An EEG recording revealed subcontinuous sharp-wave discharges with irregular runs of atypical spike-wave complexes over the anterior regions of both hemispheres, consistent with a diagnosis of frontal NCSE. A reduction in tiagabine dosage to 15 mg/day led to complete regression of the behavioral and affective changes and to disappearance of the subcontinuous EEG discharges. CONCLUSIONS: Although tiagabine-induced NCSE has been described previously, particularly in patients with preexisting spike-wave abnormalities, this is the first report that identifies its potential role in the precipitation of frontal NCSE.

Anticonvulsants↗
PubMed2004

Effects of zonisamide monotherapy in children with epilepsy.

This study examined the efficacy and safety of zonisamide as monotherapy in pediatric patients with epilepsy. Seventy-seven children with epilepsy (ages 8 months-15 years) were treated with zonisamide. Nine patients were withdrawn early because of side effects; these patients were included in side effect but not efficacy analyses. Zonisamide dosages were initiated at approximately 2 mg/kg per day and adjusted for each patient individually to a maximum of 12 mg/kg per day. Among 44 patients with cryptogenic/symptomatic partial epilepsy, 36 (82%) became seizure free; 4 (9%) had a > or =50% reduction in seizure frequency; and 4 (9%) had no change in seizures with zonisamide treatment. Of 11 patients with cryptogenic/symptomatic generalized epilepsy, 10 (91%) became seizure free, and 1 experienced no change with zonisamide treatment. Similarly, 4 patients (100%) with idiopathic partial epilepsy, and 8 of 9 patients (89%) with idiopathic generalized epilepsy became seizure free with zonisamide treatment; in the last group, 1 experienced no change. Thirty patients (39%) reported side effects, including somnolence (11.7%), decreased spontaneity (7.8%), anorexia (6.5%), and rash (6.5%). Thus, zonisamide is effective for partial seizures with or without secondarily generalized seizures in children and should be considered a broad-spectrum antiepilepsy agent.

Adolescent↗
PubMed1981

[Longitudinal study of epilepsies occurring during adolescence (author's transl)].

A total of 216 cases of epilepsies occurring during adolescence (11-18 years) were studied for a period of 10 years mean. The frequency of primary generalized epilepsies (PGE) was very high: 3/4 of the cases. Adolescence epilepsies were, in a decreasing order of frequency, PGE with grand mal seizures, partial epilepsies (PE) with complex semiology seizures, PE with elementary semiology seizures, PGE with petit mal seizures and secondary generalized epilepsies. Family antecedents could be found in PGE. Organic lesions were often observed in PE with complex semiology seizures, but never in PE with elementary semiology seizures. The seizures evolved as follows: disappearance for most of the PGE; sometimes good evolution in PE with elementary semiology seizures; more rarely good evolution in PE with complex semiology seizures. The epilepsies with a good evolution represent 80% of the epilepsies occurring during adolescence.

Adolescent↗
PubMed2004

Genetics of the epilepsies.

PURPOSE OF REVIEW: This article reviews the most significant advances in the field of genetics of the epilepsies during the past year, with emphasis on newly identified genes and functional studies leading to new insights into the pathophysiology of epilepsy. RECENT FINDINGS: Mutations in the chloride channel gene CLCN2 have been associated with the most common forms of idiopathic generalized epilepsies. A mutation in the ATP1A2 sodium potassium ATPase pump gene has been described in a family in which familial hemiplegic migraine and benign familial infantile convulsions partly co-segregate. The leucine-rich, glioma-inactivated 1 gene (LGI1) (also known as epitempin) was found to be responsible for autosomal-dominant lateral temporal lobe epilepsy in additional families. The serine-threonine kinase 9 gene (STK9) was identified as the second gene associated with X-linked infantile spasms. Mutations in the Aristaless-related homeobox gene (ARX) have been recognized as a cause of X-linked infantile spasms and sporadic cryptogenic infantile spasms. A second gene underlying progressive myoclonus epilepsy of Lafora, NHLRC1, was shown to code for a putative E3 ubiquitin ligase. SUMMARY: Genes associated with idiopathic generalized epilepsies remain within the ion channel family. Mutations in non-ion channel genes are responsible for autosomal-dominant lateral temporal lobe epilepsy, a form of idiopathic focal epilepsy, malformations of cortical development, and syndromes that combine X-linked mental retardation and epilepsy. Most genetic epilepsies have a complex mode of inheritance, and genes identified so far account only for a minority of families and sporadic cases. Functional studies are leading to a better understanding of the mechanisms underlying hyperexcitability and seizures.

Carrier Proteins↗
PubMed1987

Effect of generalized spike-and-wave discharge on glucose metabolism measured by positron emission tomography.

Positron emission tomography was used to study the cerebral metabolic rate for glucose (CMRGlc) in 7 adult patients with generalized spike-and-wave activity in the electroencephalogram. No consistent changes were seen in the CMRGlc. There was a slight trend toward an increased CMRGlc in 2 patients with primary generalized epilepsy, while in the 5 other patients with minor deviations from this condition or with secondary generalized epilepsy, the CMRGlc was unaffected by spike-and-wave activity or was below the normal range. Neither the amount of spike-and-wave activity in the EEG nor the presence of clinically evident absence seizures appeared to influence the CMRGlc. Therefore, we conclude that neuronal activity underlying spike-and-wave discharge does not seem to require increased glucose utilization.

Adolescent↗
PubMed1988

Comparison of monotherapy with valproate and other antiepileptic drugs in the treatment of seizure disorders.

Comparative data strongly suggest that valproate is the drug of choice for patients with primary and secondarily generalized epilepsies and that it is as effective as other antiepileptic drugs in the treatment of less clearly defined or partial epilepsies beginning in adult life. In simple absence epilepsy, several studies found no significant difference in efficacy between valproate and ethosuximide. In all primary generalized epilepsies, valproate has in many studies produced an excellent response, with one comparative study showing that valproate was as effective as phenytoin. Infantile spasms responded as well to valproate as to adrenocorticotropic hormone in several studies, while valproate produced fewer side effects. More long-term studies designed to compare the efficacy of monotherapy with valproate with that of other antiepileptic drugs are needed, and several are underway in both Europe and the United States.

Anticonvulsants↗
PubMed1990

Myoclonic status epilepticus: a clinical and electroencephalographic study.

We reviewed the clinical features and ictal EEGs in 23 adults with myoclonic status epilepticus (MSE). Anoxic encephalopathy was the most common cause of MSE, occurring in 15 patients; 8 developed MSE within 14 hours following the anoxic insult. Metabolic encephalopathies were present in 4 patients, while 2 had degenerative CNS disorders. In 2 patients with generalized epilepsy, MSE developed during a medication change. Five types of EEG patterns were associated with MSE. Generalized periodic complexes (usually spikes, polyspikes, or sharp waves), often with attenuation of background activity between complexes (11 patients) or a burst-suppression pattern (4 patients), were the most common types. Outcome was poor: 20 patients died without regaining consciousness, while 1 remains in a vegetative state. The 2 patients with generalized epilepsy, both of whom were conscious during MSE, survived without sequelae.

Adult↗
PubMed1980

Visual perception disabilities in epileptic children at the beginning of schooling.

Even among normally intelligent epileptic children learning difficulties occur with relative frequency. It seemed plausible to assume that visual perception problems play an important role. We decided, therefore, to analyse the quantity and quality of such disabilities. 42 epileptic children between 5 and 8 years of age, all capable of attending school, were tested with M. Frostig's Developmental Test of Visual Perception. The results can be summarized as follows: 1. The average perceptual quotient fell below the normal range. 2. There were significant differences in perceptual ability between children with primary generalized epilepsy and those with generalized secondary or partial epilepsy, to the disadvantage of the latter group. 3. In particular the Scaled Score of Subtest II measuring figure ground perception proved to be a sensitive criterion. The Subtest is therefore recommended as a screening method to uncover visual perception deficiencies in epileptic children prior to the beginning of schooling.

Child↗
PubMed2004

Efficacy and tolerability of the new antiepileptic drugs I: treatment of new onset epilepsy [RETIRED]: report of the Therapeutics and Technology Assessment Subcommittee and Quality Standards Subcommittee of the American Academy of Neurology and the American Epilepsy Society.

OBJECTIVE: To assess the evidence demonstrating efficacy, tolerability, and safety of seven new antiepileptic drugs (AEDs) (gabapentin, lamotrigine, topiramate, tiagabine, oxcarbazepine, levetiracetam, and zonisamide-reviewed in the order in which these agents received approval by the US Food and Drug Administration) in the treatment of children and adults with newly diagnosed partial and generalized epilepsies. METHODS: A 23-member committee, including general neurologists, pediatric neurologists, epileptologists, and doctors in pharmacy, evaluated the available evidence based on a structured literature review including MEDLINE, Current Contents, and Cochrane library for relevant articles from 1987 until September 2002, with selected manual searches up until 2003. RESULTS: There is evidence either from comparative or dose-controlled trials that gabapentin, lamotrigine, topiramate, and oxcarbazepine have efficacy as monotherapy in newly diagnosed adolescents and adults with either partial or mixed seizure disorders. There is also evidence that lamotrigine is effective for newly diagnosed absence seizures in children. Evidence for effectiveness of the new AEDs in newly diagnosed patients with other generalized epilepsy syndromes is lacking. CONCLUSIONS: The results of this evidence-based assessment provide guidelines for the prescription of AEDs for patients with newly diagnosed epilepsy and identify those seizure types and syndromes where more evidence is necessary.

Acetates↗
PubMed1993

The Lennox-Gastaut syndrome.

One of the most challenging areas in nosology is in the field of severe generalized epilepsy of early childhood. This is certainly true in the case of Lennox-Gastaut syndrome (LGS), an age-related epileptogenic encephalopathy which comprises several types of generalized seizures including tonic seizures, atypical absence seizures and frequent status epilepticus. EEG shows generalized slow spike waves, and as the disease progresses, cognitive functions deteriorate. LGS is listed in the 1989 classification of the International League Against Epilepsy alongside epilepsy with myoclonic astatic seizures and West's syndrome. A number of variants or atypical forms have been proposed. As a result, differential diagnosis presents a major challenge and includes specific generalized epilepsies, i.e., metabolic or inflammatory; secondarily generalized epilepsies, i.e., those arising from the frontal lobe; and severe forms of idiopathic generalized epilepsy, i.e., Doose syndrome. Antiepileptic drug (AED) treatment of LGS has been disappointing. Results obtained from anterior callosotomy have been promising, but only a small number of patients have been evaluated. Although the syndrome is rare, the severe nature and intractability of LGS emphasizes the need for the development of specific AEDs which would completely modify the quality of life for these patients.

Anticonvulsants↗
PubMed1992

Plasma urea and ammonia in epileptic patients and their relatives.

The plasma levels of urea and ammonia were examined in patients with primary generalized epilepsy, patients with partial epilepsy and in the first-degree relatives of these subjects. The results show a significant decrease in plasma urea in both groups of patients and their first-degree relatives as compared to the non-epileptic controls. The plasma ammonia concentrations were significantly higher in both groups of patients and in the relatives of generalized epilepsy patients as compared to the controls. The observed changes in plasma urea and ammonia were found not to be due to the effect of anticonvulsant drugs. The data suggest that a metabolic defect in urea synthesis may constitute one of the genetic components in the multifactorial etiologies of primary generalized and partial epilepsies.

Adolescent↗
PubMed2006

Temporal lobe epilepsy and GEFS+ phenotypes associated with SCN1B mutations.

SCN1B, the gene encoding the sodium channel beta 1 subunit, was the first gene identified for generalized epilepsy with febrile seizures plus (GEFS+). Only three families have been published with SCN1B mutations. Here, we present four new families with SCN1B mutations and characterize the associated phenotypes. Analysis of SCN1B was performed on 402 individuals with various epilepsy syndromes. Four probands with missense mutations were identified. Detailed electroclinical phenotyping was performed on all available affected family members including quantitative MR imaging in those with temporal lobe epilepsy (TLE). Two new families with the original C121W SCN1B mutation were identified; novel mutations R85C and R85H were each found in one family. The following phenotypes occurred in the six families with SCN1B missense mutations: 22 febrile seizures, 20 febrile seizures plus, five TLE, three other GEFS+ phenotypes, two unclassified and ten unaffected individuals. All individuals with confirmed TLE had the C121W mutation; two underwent temporal lobectomy (one with hippocampal sclerosis and one without) and both are seizure free. We confirm the role of SCN1B in GEFS+ and show that the GEFS+ spectrum may include TLE alone. TLE with an SCN1B mutation is not a contraindication to epilepsy surgery.

Adolescent↗