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PubMed2002

Genetic abnormalities underlying familial epilepsy syndromes.

Genetic defects have been recently identified in certain inherited epilepsy syndromes in which the phenotypes are similar to common idiopathic epilepsies. Mutations in the neuronal nicotinic acetylcholine receptor 4 and 2 subunit genes have been detected in families with autosomal dominant nocturnal frontal lobe epilepsy. Both receptors are components of neuronal acetylcholine receptor, a ligand-gated ion channel in the brain. Furthermore, mutations of two K+-channel genes were also identified as the underlying genetic abnormalities of benign familial neonatal convulsions. Mutations in the voltage-gated Na+-channel 1, 2 and 1 and the gamma aminobutyric acid (GABAA) receptor 2 subunit genes were found as a cause of generalized epilepsy with febrile seizures plus, a clinical subset of febrile convulsions. Na+-channels, GABAA receptor and their auxiliaries may be involved in the pathogenesis of this subtype and even in simple febrile convulsions. Mutation of a voltage-gated K+-channel gene can cause partial seizures associated with periodic ataxia type 1 and some forms of juvenile myoclonic epilepsy and idiopathic generalized epilepsy can result from mutations of a Ca2+-channel. This line of evidence suggests the involvement of channels expressed in the brain in the pathogenesis of certain types of epilepsy. Our working hypothesis is to view certain idiopathic epilepsies as disorders of ion channels, i.e. 'channelopathies'. Such hypothesis should provide a new insight to our understanding of the genetic background of epilepsy.

Epilepsy, Generalized↗
PubMed2002

Ictal EEG patterns in band heterotopia.

UNLABELLED: Band heterotopia (BH) or "double cortex" syndrome is a neuronal migration disorder resulting in a diffuse band of subcortical grey matter and variable abnormality of the overlying cortex. Patients with BH have a spectrum of psychomotor delay and seizures. Associated epileptic syndromes and interictal EEG findings have been described, but ictal EEG patterns are lacking. METHODS: We describe the clinical, interictal, and ictal EEG findings in two girls with BH and intractable seizures. RESULTS: Ictal EEG patterns correlated well with clinical seizure types, and did not have features unique to BH. Similarly, seizure behaviors and interictal EEG findings were typical of those seen in symptomatic generalized epilepsies. CONCLUSIONS: Despite evidence implicating the ectopic grey matter in seizure discharges, we conclude that seizure semiology and associated ictal EEG patterns in BH are no different from those seen in other causes of symptomatic generalized epilepsies.

Brain↗
PubMedDate not supplied

[Idiopathic generalized epileptic syndromes of children].

INTRODUCTION: The idiopathic generalized epilepsies of children form a neurobiological continuum which starts during the first years of life, until adolescence. In this group, infantile absence epilepsy may be considered to be the maximum expression of the idiopathic generalized epilepsies in this age group. Infantile absences have seen studied in animal models, in humans and genetically. DEVELOPMENT: We analyze the clinical and encephalographic characteristics of infantile absence epilepsies together with their prognosis and epidemiology. We also consider the palpebral myoclonias with absences and absences with perioral myoclonias. The generalized tonic-clonic seizures of childhood form an ill-defined epileptic syndrome, with very heterogeneous clinical and electroencephalographic characteristics. CONCLUSION: It seem important to be able to establish the precise diagnosis in these syndromes, since correct treatment depends on it.

Adolescent↗
PubMed2005

Topiramate monotherapy as broad-spectrum antiepileptic drug in a naturalistic clinical setting.

Topiramate was assessed in an open-label trial as broad-spectrum antiepileptic monotherapy, independently from the epilepsy type or syndrome. Adults and children aged 2 years and older, who were diagnosed with epilepsy within the last 5 years, treatment-naive or failing prior treatment with one antiepileptic drug (AED), received individually adjusted doses of topiramate, after escalation to 100mg/day over 4 weeks (maximum 400mg/day) or 3mg/kg/day over 6 weeks (maximum 9 mg/kg/day), respectively. Patients were followed for >or=7 months and optionally up to a maximum of 13 months. Data were analysed for all patients (n=692), as well as for focal (n=421) and generalized epilepsies (n=148). The median topiramate dose used was 125 mg/day in adults and 3.3mg/kg/day in children ( or=50% reduction in mean monthly seizure frequency. Patients with focal and generalized epilepsies alike responded to treatment (73.9 and 83.8% with at least 50% seizure reduction): 39.4% of patients with focal epilepsy and 61.5% of those with generalized epilepsy were seizure-free. The mean monthly seizure frequency was significantly reduced versus baseline at all visits (p<0.001). Similar response rates were obtained from the 237 patients completing the 1-year observation period. During the mandatory 7-month period of study, 8.8% of patients reported insufficient tolerability as a reason for dropout. The most frequent adverse event was paraesthesia. Our results support findings that emerge from controlled studies that topiramate is effective and well tolerated when used as initial or second monotherapy. They also suggest that in a naturalistic setting, overall good retention on treatment and seizure freedom are observed at low doses in a broad spectrum of epilepsies.

Adolescent↗
PubMed1983

Enhanced response of cortical neurons to thalamic stimuli precedes the appearance of spike and wave discharges in feline generalized penicillin epilepsy.

Peristimulus time histograms of extracellularly recorded action potential discharges of cortical neurons in response to single shock and/or repetitive stimulation of 'specific' and 'non-specific' nuclei of the thalamus were studied after i.m. penicillin injection during a period corresponding to that of the development of spike and wave (SW) discharges of feline generalized penicillin epilepsy (FGPE). After i.m. penicillin cortical neurons displayed an enhancement of both the excitatory and 'inhibitory' phases of their responses to single shock stimulation of n. centralis medialis (NCM). This increase was even more pronounced for responses induced by repetitive stimulation of NCM at the frequencies inducing typical recruiting responses. These changes always preceded the appearance of SW discharges. Changes of the responses of cortical neurons to single shock and repetitive stimulation of 'specific' thalamic nuclei after penicillin were weak and inconsistent, although when observed were characterized by an enhancement of both excitatory and 'inhibitory' phases. The latter appeared not to decrease after i.m. penicillin. These data suggest that the appearance of SW discharges of FGPE is closely related to an increased responsiveness of cortical neurons to thalamocortical volleys arising from the so-called 'non-specific' nuclei. This facilitation of the recruiting process is accompanied by an increase of both excitatory and 'inhibitory' phases of the cortical neuronal responses induced by the volleys.

Animals↗
PubMedDate not supplied

Classification of the epilepsies: an investigation of 945 patients in a developing country.

The present study classified epilepsies in Nigerians and compared them with the profile of epilepsies as found in other countries: India, France, and Denmark. Partial epilepsy formed the largest group (76.6%) in this study and in that in India (80%), but these incidences were higher than that found in France (62%), owing to a higher frequency of birth injury, CNS infections, and childhood febrile convulsions in developing countries. In contrast to our study and that in France, where partial epilepsy with complex symptomatology formed the largest subgroup, partial epilepsy with elementary symptomatology formed the largest subgroup in India. The reason for this is not totally clear, though etiological factors and criteria for categorization are contributory. The incidence of partial epilepsy was lower in children than in adults owing to a relatively lower incidence of partial epilepsy with elementary symptomatology in children in the present series and a lower incidence of complex symptomatology in children in France and Denmark. Nigerian children seem more vulnerable to complex symptomatology owing to a high incidence of febrile illness (e.g., from malaria) and febrile convulsions. The incidence of generalized epilepsies in children was higher than in adults. Grand mal formed the largest subgroup of generalized epilepsies in children in this series and in Denmark, whereas petit mal formed the largest subgroup in France and India. Petit mal was relatively rare in children in our series (2.5%) compared with children in the French study (17.5%). Secondary generalized epilepsy was peculiar to children in all the series.(ABSTRACT TRUNCATED AT 250 WORDS)

Adolescent↗
PubMed2006

MRI volumetry shows increased anterior thalamic volumes in patients with absence seizures.

The interaction between thalamus and cortex appears to be critical to the pathophysiology of idiopathic generalized epilepsies (IGEs). The objective of this study was to investigate thalamic volumes of a group of patients with IGEs using high-resolution MRI. Thalamic segmentation was performed by the same rater, who was unaware of the diagnosis. Thalamic volumes were divided into anterior half and posterior half. One hundred forty-seven patients were scanned (71 with juvenile myoclonic epilepsy, 49 with generalized tonic-clonic seizures only, and 27 with absence epilepsy). Subgroup analyses with corrections for multiple comparisons showed that, when compared with those of controls, anterior thalamic volumes were increased in patients with absence epilepsy and juvenile myoclonic epilepsy with absence seizures, but not in patients with generalized tonic-clonic seizures only and juvenile myoclonic epilepsy without absence seizures. Our results demonstrated that the anterior thalamus is structurally different in patients with IGEs and absence seizures as compared with patients with IGEs without absence seizures.

Adult↗
PubMed2005

Epilepsy increases vulnerability of long-term face recognition to proactive interference.

Proactive interference (PI) decreases short- and long-term memory in healthy subjects. Neurological patients exhibit a heightened PI effect on short-term memory. It is, however, not known if PI affects long-term memory in neurological patients. We analyzed whether epilepsy heightens the negative effect of PI on long-term face memory. PI was induced by a list of 20 faces learned 24 hours prior to a target list of 20 faces. We tested immediate and 24-hour recognition for both lists. Twelve healthy controls and 42 patients with generalized epilepsy or temporal lobe epilepsy (TLE) were studied. PI led to a decrease in 24-hour recognition in patients with generalized epilepsy and TLE but not in controls. Thus, PI may cause long-term memory disturbances in epilepsy patients. PI was also associated with decreased short-term memory, but only in right TLE. This confirms the dominant role of the right temporal lobe in short-term face memory.

Adolescent↗
PubMed1999

Callosotomy for epilepsy after West syndrome.

PURPOSE: To analyze the results of callosotomy in 17 children with symptomatic generalized epilepsy after West syndrome, according to the different seizure types and surgical procedures, to define selection criteria for candidates to callosotomy. METHODS: Callosotomy was performed in two successive stages. Partial callosotomy, anterior in 13 and posterior in three, was followed by completion in 14 cases in all but four patients (complete callosotomy in one stage in one, no completion in three). All patients had clinical, video-EEG, and neuropsychological evaluation before and after each stage of callosotomy, with a mean final follow-up of 4 years. RESULTS: Seizure frequency improved in only two of 13 patients after anterior callosotomy, in none of three after posterior callosotomy, but in nine of 14 after complete callosotomy. After complete callosotomy, spasms disappeared in 80% of the cases, and drop attacks, the most severe ictal event, completely stopped or were dramatically reduced in 90% of the children. One patient no longer had episodes of status epilepticus, and another one acquired the ability to walk after complete callosotomy. From the cognitive viewpoint, nine patients with improved seizure frequency after complete callosotomy also had improved behavior and cognitive functions, but two others experienced speech deterioration after posterior callosotomy at age 11 years and completion of callosotomy at age 16 years. CONCLUSIONS: As in other severe generalized epilepsies in childhood, drop attacks provide the best indication for complete callosotomy in patients with previous West syndrome. Because drop attacks can be identifiable by falls only, the previous acquisition of walking should be considered as a key feature for any benefit to be obtained.

Child↗
PubMed2000

Opposite effects of T- and L-type Ca(2+) channels blockers in generalized absence epilepsy.

The role of the T-type Ca(2+) channel blocker, ethosuximide, the L-type Ca(2+) channel blocker, nimodipine and L-type Ca(2+) channel opener, BAY K8644 (1,4 Dihydro-2, 6-dimethyl-5-nitro-4-[trifluoromethyl)-phenyl]-3-pyridine carboxylic acid methyl ester), was investigated on spike-wave discharges in WAG/Rij rats. This strain is considered as a genetic model for generalized absence epilepsy. A dose-dependent decrease in the number of spike-wave discharges was found after i.c.v. ethosuximide, an increase after i.p. nimodipine and a decrease after i.c.v. BAY K8644. BAY K8644 was also able to antagonise the effects of nimodipine. Preliminary data were obtained with two conotoxins, MVIIC and GVIA, which block P/Q-type and N-type Ca(2+) channels, respectively. Only after i.c.v. administration of omega-conotoxin GVIA were the number and duration of spike-wave discharges reduced, but animals showed knock-out lying. The latter suggests behavioural or toxic effects and that the decrease in spike-wave activity cannot unequivocally be attributed to blockade of N-type Ca(2+) channels. It can be concluded that T- and L-type Ca(2+) channel blockers show opposite effects on spike-wave discharges. Furthermore, these effects are difficult to explain in terms of a model for spindle burst activity in thalamic relay cells proposed by McCormick and Bal [Sleep and arousal: thalamocortical mechanisms.

3-Pyridinecarboxylic acid, 1,4-dihydro-2,6-dimethy↗
PubMed2000

A psychosocial approach to epileptic patients.

A psychoeducational approach was taken with 174 epileptic patients. Using this approach, no family problems were recognized among patients with idiopathic generalized epilepsy (IGE) or among those with symptomatic generalized epilepsy (SGE). However, 11 patients with temporal lobe epilepsy (TLE) and I patient with non-temporal lobe epilepsy (non-TLE) did exhibit family problems indicating that such problems involving IGE or SGE cases can be prevented through educational programs using a psychoeducational approach. This fails, however, to prevent such problems for TLE or non-TLE cases. Furthermore, small group psychotherapy was given to 10 patients with intractable TLE. They were directed to make self-evaluations regarding therapeutic factors originally introduced by Yalom but specially modified for these particular patients. Relatively high evaluations were given on every factor when compared with the results of individual psychotherapy. These results point out the importance of providing such psychotherapeutic approaches as group psychotherapy and self-help groups in addition to educational programs in order to enhance the quality of life (QOL) of epileptic patients and their families.

Epilepsy↗
PubMed1998

Epilepsy diagnosis and localization in patients with antecedent childhood febrile convulsions.

PURPOSE: A history of febrile convulsions (FC) is often obtained in patients presenting for surgical treatment of temporal lobe epilepsy (TLE), but it is not clear that preferential temporal localization of epilepsy is associated with antecedent FC. METHODS: We prospectively inquired about FC and their characteristics in all patients presenting to an epilepsy clinic through a patient questionnaire and interview. We studied the incidence of antecedent childhood febrile convulsions in relation to epilepsy diagnosis. RESULTS: FC were reported by 133 of 1005 study patients (13.2%). TLE was more likely to be preceded by FC (78/310, 25.2%) than extratemporal epilepsy (ETE) (12/216, 5.6%) (p < 0.000001) or generalized epilepsy (GE) (16/146, 11.0%) (p < 0.001). Patients with GE were more likely than patients with TLE to have had simple FC (p < 0.00005). Prolonged duration was the most common FC complex feature in TLE patients. CONCLUSIONS: We demonstrated a preferential association of FC with temporal lobe foci and a weaker association between FC and GE. FC does not appear to be a clear risk factor for ETE.

Adolescent↗
PubMed1992

Drug treatment of uncontrolled seizures.

The success of AED therapy for epileptic seizures varies according to epilepsy and seizure type. The outcome is excellent in the idiopathic generalized epilepsies, poor in the secondary generalized epilepsies and intermediate for the partial epilepsies. For this latter group, a table of outcome is described in Table I. Overall, approximately 70% of patients can be reasonably managed using one AED, although several may need to be tried before considering monotherapy to be a failure. Of the 30% unsatisfactorily managed by monotherapy, approximately another one-third can be adequately managed given a combination of two drugs. Combinations of more than two drugs provide little if any additional benefit. Evidence that such combinations have efficacy is inferred from the observation of seizure exacerbation when one drug is removed purposely to elicit seizures during evaluation for possible epilepsy surgery. The remaining patients are often considered for trial of an investigational drug although prospects for important improvement are very small even with use of agents shown to be effective and introduced for treatment of epilepsy. Thus, 15% of patients may conservatively be considered for surgical therapy. This would not suggest that all such patients are appropriate candidates from other aspects, but at least they can be considered to have failed all reasonable drug therapy.

Anticonvulsants↗
PubMed1995

Significance of spontaneous epileptiform abnormalities associated with a photoparoxysmal response.

Spontaneous epileptiform abnormalities (SEAs) are reported to occur in up to 65% of patients with a photoparoxysmal response (PPR). Although the PPR is usually assumed to indicate primary generalized epilepsy, the clinical significance of associated SEAs is not known. We conducted the first study designed to correlate seizure classification with type of SEA in photosensitive patients. We examined seizure classifications and SEAs in 115 consecutive patients who had a PPR. A PPR was the only epileptiform abnormality in 47 patients (41%). Twenty-seven patients (24%) had focal SEAs and 41 (36%) had only generalized SEAs. Seventeen patients (15%) had partial seizures and 40 (35%) had only generalized seizures. Seizure classification was strongly associated with type of SEA (p < 0.0001). Patients with focal SEAs tended to have partial seizures, while patients with generalized SEAs tended to have only generalized tonic-clonic or absence seizures. Also, the presence of SEAs was significantly associated with a history of seizures (p < 0.0001), compared with patients who had a PPR but no SEAs. Although the PPR is often presumed to signify primary generalized epilepsy, most patients with a PPR and focal SEAs have partial seizures.

Adolescent↗
PubMed2005

Post-ictal circulating levels of allopregnanolone in children with partial or generalized seizures.

INTRODUCTION: Allopregnanolone (3alpha-hydroxy-5alpha-pregnan-20-one) is a neurosteroid with a potent modulating activity on the gamma-aminobutyric acid (GABA)(a) receptor complex. It plays a key role in the epileptogenesis of partial seizures. Serum allopregnanolone concentrations significantly increase in the postcritical phase. In the present study we investigated the post-ictal serum allopregnanolone levels in children with partial seizures and generalized seizures, respectively. PATIENTS AND METHODS: Three groups of subjects were included in the study. Group 1 consisted of 18 children affected by complex partial seizures. Group 2 consisted of 11 children presenting with generalized epilepsy. Group 3 consisted of 20 healthy age-matched subjects. Serum allopregnanolone levels were assayed in the inter-ictal phase and within 30 min after an epileptic event. RESULTS: The data we obtained suggest that circulating allopregnanolone level significantly increases in the post-ictal phase. However, we found no significant differences in the post-ictal serum allopregnanolone concentrations between patients with partial seizures and those with generalized seizures. CONCLUSIONS: Further studies are needed to establish if allopregnanolone is a reliable circulating marker of epileptic seizures. However, our observations seem to indicate that post-ictal circulating allopregnanolone level is not useful in differentiating focal and generalized epilepsy events.

Anesthetics↗
PubMed1978

Treatment of childhood epilepsy with dipropylacetic acid (DPA).

Dipropylacetate (DPA) was used in the treatment of different types of epilepsy in 112 children aged 1--20 years, with a mean age of 9.2 years, for a period of 19.8 months, ranging from 1 to 49 months. Of this group, 64 children were therapy-resistant to other antiepileptic medications prior to the introduction of DPA; 31 were treated for the first time with an antiepileptic drug, which was DPA; 44 were treated with DPA alone; and 68 had one or more additional antiepileptic medication. The following results were found while DPA was administered in a relatively high dosage with a mean of 48 mg/kg body weight/day and ranging from 7 to 125 mg/kg/day. 1. Statistically, the results are significantly better in primary generalized epilepsy than in partial or in secondary generalized epilepsy. 2. Ninety-two percent of 51 patients who had absences were treated successfully. The same applies to 87% of 30 patients with primary generalized grand mal with spike wave, to all four patients who had impulsive petit mal, and to 47% of the 15 patients who had centrencephalic myoclonic-astatic petit mal. 3. Positive effect of DPA in partial epilepsy and secondary generalized epilepsy was seen only if the EEG pattern was 'centrencephalic' besides focal changes. During therapy with DPA, five patients with pure focal EEG showed an increase in seizure frequency, which demonstrated complete therapeutic failure. 4. Centrencephalic seizure activity (irregular spike wave, 3/s spike wave, and more than 3.5/s spike wave) were treated successfully (P less than 0.001). Focal changes or focal sharp wave with tendency to spread or generalization were treated unsucessfully.

Adolescent↗
PubMed2001

Spasms in clusters in epilepsies other than typical West syndrome.

Although spasms in clusters are one of the major characteristics of West syndrome (WS), there are a significant number of patients who show spasms in clusters but do not fit the standard pattern of WS. It is possible to divide these atypical cases into the following three groups. Group 1: refractory epilepsies beginning in early infancy, associated with atypical electroencephalographic (EEG) features; Group 2: generalized epilepsies with spasms in clusters at ages of 2-3 years or above; and Group 3: localization-related epilepsies with spasms in clusters. Ictal clinical and EEG findings of spasms in clusters in these atypical patients and also those in WS are similar. Patients in Group 1 often suffer from Aicardi syndrome, cortical malformations, early myoclonic encephalopathy and Ohtahara syndrome. Most patients in Group 2 suffer from Lennox-Gastaut syndrome and other generalized epilepsies such as severe epilepsy with multiple independent spike foci. A significant number of them had a history of WS. Small number of patients in Group 2 can be diagnosed as having late-onset WS or long-lasting WS. In Groups 1 and 3 patients, cortical mechanisms play a critical role in their pathophysiology. The presence of older patients with spasms in clusters might indicate not only developing process of the brain but also some selective dysfunction of the brain plays an important role in the occurrence of spasms in clusters. Investigations on these atypical patients can help the understanding of pathophysiological mechanisms of WS and its related epileptic syndromes.

Brain↗