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Results for “Epilepsy, Generalized”

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PubMed1976

Lennox-Gastaut's syndrome--prognosis of the secondary generalized epilepsies.

On 62 cases with Lennox-Gastaut's syndrome aged four to 31, the clinical-electroencephalographic findings were summarized as follows; (1) Age of onset was over 10 years in 10 cases (16.1%). (2) Mental deficiencies were more severe in those with onset earlier than age three. (3) Behavioral problems were observed in 34 cases (54.8%); 21 with hyperactive and 13 with hypoactive ones -- 18 hyperactive cases (85.7%) with the onset taking place before age six, and 12 hypoactive cases (92.3%), all whose age is now over 10. (4) The number of clinical seizures showed a tendency in which monoictal manifestation decreased from 25 to three whereas polyictal one increased from 13 to 59 cases during the course of a decade. (5) Interictal EEG findings were pseudorhythm of slow spike-wave with or without focal spikes, and so-called runs of rapid spikes during sleep recording. Focal spikes were observed in 25 cases (40.3%); mainly in the frontal area among those under 10 years old, and in the anterior temporal area among those over 20. The rapid spikes were demonstrated in 19 cases (30.6%) in the over-15 age group and appeared to be correlated with epileptic drop seizures and atypical complex absences.

Adolescent↗
PubMed2003

A deletion in SCN1B is associated with febrile seizures and early-onset absence epilepsy.

Generalized epilepsy with febrile seizures plus (GEFS+) is a clinically and genetically heterogeneous syndrome with childhood onset, characterized by febrile seizures (FS) and a variety of afebrile epileptic seizure types. The authors performed a mutational analysis of SCN1B on 74 unrelated probands with GEFS+, FS, or FS plus (FS+). In a family with FS+ and early-onset absence epilepsy, a mutation was identified that predicts a deletion of five amino acids in the extracellular immunoglobulin-like domain of SCN1B and potential loss of function. SCN1B mutations are associated with GEFS+ and may have a role in the elicitation of absence seizures.

Age of Onset↗
PubMed2006

Thalamic lesion and epilepsy with generalized seizures, ESES and spike-wave paroxysms--report of three cases.

We report three patients, who have thalamic lesion and secondary generalized epilepsy with generalized spike wave pattern. The first two patients have unilateral perinatal lesion, one with generalized tonic-clonic seizures on awakening the other with Landau-Kleffner-like syndrome. During the course of the disease both children developed electrical status epilepticus in slow wave sleep (ESES). The third patient has a dominantly unilateral thalamic tumor and epilepsy that mimics juvenile myoclonic epilepsy. All the patients have a lesion located in the inferior-medial-posterior part of the thalamus. The role of some thalamic and subthalamic nuclei in the generalized spike-wave electrical pattern patophysiology is discussed, with emphasis on the possible role of the inhibitory system from the zona incerta.

Adult↗
PubMed1989

Positron emission tomography in epilepsy: correlative study.

Positron emission tomography (PET) was performed with the 18F-fluoro-deoxy-glucose method on 29 patients with epilepsy (generalized epilepsy, 4; partial epilepsy, 24; undetermined type, 1). The subjects were restricted to patients with epilepsy without focal abnormality on X-CT. All the patients with generalized epilepsy showed a normal pattern on PET. Fourteen out of the 24 patients with partial epilepsy and the 1 with epilepsy of undetermined type showed focal hypometabolism on PET. The hypometabolic zone was localized in areas including the temporal cortex in 11 patients, frontal in 2 and thalamus in 1. The location of hypometabolic zone and that of interictal paroxysmal activity on EEG were well correlated in most patients. The patients with poorly-controlled seizure showed a higher incidence of PET abnormality (12 out of 13) than those with well-controlled seizures (2 out of 11). The incidence of abnormality on PET and MRI and the location of both abnormality were not necessarily coincident. These results indicated that the PET examination in epilepsy provides valuable information about the location of epileptic focus, and that the findings on PET in patients with partial epilepsy may be one of the good indicators about the intractability of partial epilepsy, and that PET and MRI provide complementary information in the diagnosis of epilepsy.

Blood Glucose↗
PubMed2004

Noninactivating voltage-gated sodium channels in severe myoclonic epilepsy of infancy.

Mutations in SCN1A, the gene encoding the brain voltage-gated sodium channel alpha(1) subunit (Na(V)1.1), are associated with at least two forms of epilepsy, generalized epilepsy with febrile seizures plus and severe myoclonic epilepsy of infancy (SMEI). We examined the functional properties of five SMEI mutations by using whole-cell patch-clamp analysis of heterologously expressed recombinant human SCN1A. Two mutations (F902C and G1674R) rendered SCN1A channels nonfunctional, and a third allele (G1749E) exhibited minimal functional alterations. However, two mutations within or near the S4 segment of the fourth repeat domain (R1648C and F1661S) conferred significant impairments in fast inactivation, including persistent, noninactivating channel activity resembling the pattern of channel dysfunction observed for alleles associated with generalized epilepsy with febrile seizures plus. Our data provide evidence for a range of SCN1A functional abnormalities in SMEI, including gain-of-function defects that were not anticipated in this disorder. Our results further indicate that a complex relationship exists between phenotype and aberrant sodium channel function in these inherited epilepsies.

Epilepsies, Myoclonic↗
PubMedDate not supplied

Feline generalized penicillin epilepsy.

Feline generalized penicillin epilepsy represents an experimental model of generalized spike-and-wave discharges occurring during clinical absence attacks. Spike-and-wave discharges of feline generalized penicillin epilepsy also have a pharmacological profile that is similar to that encountered in human absence attacks. Studies on the respective roles played by the thalamus and cortex in the generation of spike-and-wave discharges indicate that both structures are important in the elaboration of such generalized activity. Moreover, GABAA-mediated, intracortical inhibitory mechanisms are preserved and eventually enhanced at a time when generalized spike-and-wave discharges of feline generalized penicillin epilepsy are recorded. A preservation of GABA-mediated mechanisms in pure absence epilepsy might explain the differences in prognostic outlook that characterizes this type of epilepsy from seizures in which GABAergic mechanisms break down (e.g., generalized convulsive and partial epileptic attacks).

Animals↗
PubMed1993

The development of a questionnaire to assess knowledge of epilepsy: 1--General knowledge of epilepsy.

It is well documented that knowledge is a vital factor in the ability to cope successfully with epilepsy. Misconceptions and deficits in knowledge have implications not only for psychosocial well-being but also for medical compliance. However, at present there is no commonly accepted objective measure of knowledge of epilepsy. The development of the Epilepsy Knowledge Profile--General (E.K.P.--G) is presented in this paper. Fifty-five true/false items (34 medical knowledge items, 21 social knowledge items) were selected by a range of experts in the field of epilepsy. A clinical trial of the questionnaire was then completed by 82 people with epilepsy attending a city centre outpatient clinic. Results indicated that the scale has both good internal reliability and test-retest reliability. Also the range of scores indicates that it is sensitive to differences in knowledge. Potential uses of the questionnaire are discussed.

Adaptation, Psychological↗
PubMed1984

[Status epilepticus in adult epileptics followed in a neurologic hospital].

One or several status were observed in 90 chronic adult epileptics. Partial status, especially motor and of similar type, is rare in the clinical course of usual partial epilepsy. Generalized status, chiefly petit mal (PM) status, appears in more severe generalized epilepsies. Partial status epilepticus is observed in motor attacks and rarely in partial complex epilepsies, although the latter are more frequent. In 40% of cases the aetiology is unknown. Delay of the first status is variable, from 2 to 30 years. Status does not make previous epilepsy worse. Generalized status, mainly PM status, appears in patients with absences and generalized attacks, sometimes some decades after the beginning of the disease. In half of the cases PM status are frequent but are sometimes the only expression of the epilepsy.

Adolescent↗
PubMed1989

National General Practice Study of Epilepsy and Epileptic Seizures: objectives and study methodology of the largest reported prospective cohort study of epilepsy. National General Practice Study of Epilepsy and Epileptic Seizures (NGPSE).

Most available data on the prognosis of epileptic seizures come from hospital-based clinics in which patients with chronic or severe disease are over-represented. The National General Practice Study of Epilepsy and Epileptic Seizures is a large prospective community-based study of people with newly diagnosed seizures which aims to address questions related to the early prognosis of epilepsy. 275 general practitioners throughout the United Kingdom have registered a total of 1,195 patients. In this paper we discuss the background to the study and the methodology used.

Cohort Studies↗