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The epileptiform significance of intermittent rhythmic delta activity in childhood.

Intermittent rhythmic delta activity is reported in various disorders and is classified as a nonspecific abnormal electroencephalographic pattern. We have investigated its clinical and electroencephalographic features in childhood. Intermittent rhythmic delta activity was identified in 54 children over a period of 48 months. Epilepsy was present in 81%, 4% had only a single generalized tonic-clonic seizure, and 15% had no seizures. Generalized seizures were more common than partial seizures (83% versus 13%; 4% were mixed). The largest group of patients had idiopathic epilepsy. Epileptiform features were present in 70%. No patient identified prospectively has had a space-occupying lesion. Intermittent rhythmic delta activity should be considered an epileptiform pattern in children, most commonly occurring as an interictal pattern in primary generalized epilepsy.

Adolescent↗

Temporal lobe syncope: clinical variants.

Temporal lobe syncope (TLS) is a term coined by Landolt. Characteristically, the patient has psychomotor and drop attacks, and the interictal electroencephalogram (EEG) shows temporal lobe epileptic abnormalities. TLS is synonymous with type III complex partial seizures (CPSs) in the Delgado Escueta classification. Several variants of TLS can be recognized including atonic akinetic, simple akinetic, atonic, atonic-tonic complex (automatisms), sexual seizures, stress-induced convulsions, and gelastic atonic seizures. TLS must be distinguished from drop attacks of vertebrobasilar insufficiency and associated EEG abnormalities, and from hereditary tachyarrhythmias mimicking stress-induced convulsions. Epileptic falls and drop attacks are discarded by ictal EEG recordings. Recognition of TLS variants is important in the prospective evaluation of the surgical treatment of epilepsy given the past conflicting reports on the differential outcome of surgically treated CPSs. TLS is an attractive clinical term, easy to remember, and with pathophysiologic relevance to the clinician confronting the patient with a history of syncope and whose EEG discloses temporal lobe paroxysmal activity. The detailed ictal electrophysiology of TLS is unknown.

Adult↗

Multifocal independent Spike syndrome: relationship to hypsarrhythmia and the slow spike-wave (Lennox-Gastaut) syndrome.

During a 3 year period EEGs were performed in 64 children with multifocal independent spikes (MIS), 17 with slow spike-wave complexes (SSWC), 22 with MIS and SSWC and 15 with hypsarrhythmia. Only EEG records containing adequate wakefulness and sleep were analyzed in 40 children with two or more serial EEGs at least 5 months apart. Transitions from one pattern to another occurred in 25/40 patients, consistent in all cases with the following sequence: Hypsarrythmia-->MIS-->MIS and generalized discharges-->SSWC. Patients with and without transitions did not differ in their age at presentation or duration of follow-up. Eleven of 12 patients whose initial EEG showed hypsarrhythmia or multifocal independent spikes underwent transitions, compared to 0/8 patients with SSWC (p < 0.001), indicating that SSWC is a stable pattern in children. Over a 6 month period, we also prospectively analyzed EEGs of 20 patients with multifocal spikes, hypsarrhythmia, and slow spike-wave complexes. Sleep activated additional spike foci, increased the frequency of generalized spike discharges and produced synchronization of bitemporal and bifrontal spike-wave discharges at 1.5-2.5 Hz the same as SSWC.

Adolescent↗

Frontal lobe epilepsy with secondarily generalized 3 Hz spike-waves: a case report.

We report a case of frontal lobe epilepsy with bursts of 3 Hz spike-wave, often dominant in the left frontal lobe, that sometimes developed into a secondary bilateral generalization. This patient was a 69-year-old male with a history of epileptic seizures that began 3 months following a head injury at the age of 17. His seizures occurred two or three times a day, but after medication the frequency was halved. The seizures began with a feeling of heaviness of the head, accompanied by myoclonus at both angles of the mouth. Consciousness was clear for most of the seizures, which ranged from several seconds to many minutes. When seizures lasted for longer than 10 minutes, a temporary generalization accompanied by a loss of consciousness was sometimes observed. Neurological tests and cranial MRI yielded no abnormal local findings. The secondary generalized seizure seen in this case is the so called "secondary bilateral synchrony (SBS)." We succeeded in making an EEG recording of the bilateral generalization of a seizure discharge originating in the left frontal lobe, which until now has not been done. This case is direct proof that a primary frontal focus can give rise to SBS.

Aged↗

Association analysis of gamma 2 subunit of gamma- aminobutyric acid type A receptor polymorphisms with febrile seizures.

An alternation of gamma-aminobutyric acid (GABA)-ergic neurotransmission has been implicated as an etiologic factor in epileptogenesis. Missense mutations in the GABRG2 gene, which encodes the gamma2 subunit of central nervous GABAA receptors, have recently been described in one family with childhood absence epilepsy and febrile seizures (FSs). FSs represent the majority of childhood seizures and have a genetic predisposition. It is not known, however, whether polymorphisms in those genes involved in familial epilepsies also contribute to the pathogenesis of FSs. By performing an association study, we used single-nucleotide polymorphisms to investigate the distribution of genotypes of GABRG2 in patients with FSs. A total of 104 children with FSs and 83 normal control subjects were included in the study. PCR was used to identify the C/T and A/G polymorphisms of the GABRG2 gene on chromosome 5q33. Genotypes and allelic frequencies for the GABRG2 gene polymorphisms in both groups were compared. The GABRG2 (nucleotide position 3145 in intron G-->A) gene in both groups was not significantly different. In contrast, the number of individuals with the GABRG2 (SNP211037)-C/C genotype in patients with FSs was significantly greater compared with that in healthy control subjects (p = 0.017), and the GABRG2 (SNP211037)-C allele frequency in patients with FSs was significantly higher than that in healthy control subjects (p = 0.009). The odds ratio for developing FSs in individuals with the GABRG2 (SNP211037)-C/C genotype was 2.56 compared with individuals with the GABRG2 (SNP211037)-T/T genotype. These data suggest that the GABRG2 gene might be one of the susceptibility factors for FSs.

Child↗

Eterobarb therapy in epilepsy.

A group of 27 patients with various types of epilepsy were selected for a 6-month double-blind crossover study to compare the anticonvulsant effect and toxicity of eterobarb and phenobarbital. No statistically significant differences in seizure frequency were found among the 21 patients who completed the 6-month trial, but three others, in whom status epilepticus developed during the crossover from eterobarb to phenobarbital, had to be removed from the trial. The study provided some indication that when eterobarb and phenobarbital were used in high dosage with corresponding high serum barbiturate levels (over 30 mug per milliliter), eterobarb had a superior therapeutic effect. Side effects from both drugs included tiredness, sleepiness, nystagmus, and infrequently ataxia, but serious systemic toxicity did not occur. This study showed that eterobarb is a safe and potent anticonvulsant comparable in efficacy to phenobarbital, and the superior results obtained in some patients with eterobarb therapy indicate that it is an effective alternative anticonvulsant.

Adolescent↗

Clinical and electroencephalographic correlation of independent multifocal spike discharges.

One hundred and eight patients with independent, multifocal, spike discharges in the EEG were studied. The abnormality occurred at all ages but was more frequent between 4 and 7 years. The basic EEG activity in wake tracing was invariably slow for the age of the patient. The majority of the patients with independent multifocal spike discharges had extensive bilateral cerebral lesions that occurred in the prenatal, perinatal, or postnatal periods. Clinical seizures were of different types, and patients had varying degrees of intellectural and neurologic deficits. A significant number had had previous tracings with either hypsarrhythmic or slow spike-wave patterns, suggesting a close relationship of these three EEG abnormalities.

Adolescent↗