Twelve patients with benign juvenile myoclonic epilepsy (BJME) representing 4% of our population of epileptics (n = 275) are presented. Only two patients (17%) had myoclonic jerks as the only seizure type. Seven (58%) had generalized tonic-clonic seizures (GTCS) and myoclonus. Three patients (25%) had absence seizures (AS), GTCS, and myoclonic jerks. Electroencephalographic evidence of photosensitivity was found in four (33%). Auditory precipitation of seizures was found in one patient. As is the case with other primary generalized epilepsies, the onset of BJME seems to be age specific. In our series the mean age of onset in years was 4.3 for AS, 14.75 for myoclonic jerks, and 16.4 for GTCS. It took an average of 8.5 years from the onset of BJME (range, 2-20 years) and 6.5 years from the onset of GTCS (range, 2 months-6 years) until the condition was properly recognized. Five patients experienced at least one episode of myoclonic status epilepticus. Generalized, paroxysmal, symmetric polyspike and slow wave discharges are the typical EEG finding. These complexes, however, showed considerable interpatient variability. Sleep deprivation proved to be the most valuable activating procedure. Valproic acid monotherapy effectively controlled myoclonic jerks as well as associated GTCS in most patients.
Induction by suggestion has previously been reported to be effective in the diagnosis of psychogenic seizures (PS). However, the sensitivity and specificity of this procedure has not previously been studied. Results of induction of PS by suggestion were analyzed in 93 patients with purely PS. The diagnosis of PS was based on the recording of a clinical event on video-electroencephalography, the absence of clinical or electroencephalography the absence of clinical or electroencephalographic evidence of epilepsy, and the subsequent followup and withdrawal of anticonvulsants supporting the diagnosis of PS. A control-group was composed of 20 patients with epilepsy in which induction was tried. Both groups were comparable for age, sex, and educational level. Induction was performed following a standardized protocol. The test was carried out placing a colored patch on the neck. The test was considered positive when the induced clinical events were typical, according to a witness familiar with the patient's seizures. Induction was positive in 72 of 93 cases with PS and in none with epilepsy. Sensitivity of this test for the diagnosis of PS was 77.4%, specificity 100%, positive predictive value 100%, and negative predictive value 48.7%.
AUTOMATISMS have long attracted the neurologist's attention, but their occurrence has become too widely regarded as a pathognomonic sign of temporal lobe epilepsy.Since Penfield1 reported the production of automatisms by stimulating gray matter of mesial temporal lobe adjacent to the insula, many neurologists have inferred that an automatism is synonymous with a discrete discharging focus in the temporal lobe. The designation of "temporal lobe automatisms" used by investigators2 in describing clinical neurophysiologic studies has led to the general assumption that automatisms occurring in temporal lobe epilepsy have specific characteristics which set them apart from other automatisms.3 This notion evolved in spite of well documented descriptions of automatisms in association with the absence attacks of petit mal epilepsy; indeed "absence with automatisms" is an integral part of the nonfocal section of the international classification.4This study was conducted to determine the nature of automatisms which occur in association with absence attacks and generalized spike-wave abnormalities in the elec¬ troencephalogram. The study was specifi¬ cally designed to determine the influence of activity and different environmental stimuli upon the occurrence and character of automatisms. Material Twelve patients, ranging in age from 6 to 24 years, were studied in the EEG Laboratory, University of Virginia Medical Center. Each patient had a history of recurring absence at¬ tacks with aura or postictal abnormalities of the mental state. None had a history of nor were there neurologic findings to suggest either a lesion or intermittent dysfunction of a tem¬ poral lobe. In other words, they all met criteria for the diagnosis of petit mal epilepsy accord¬ ing to the international classification4 in having absence attacks with generalized spike-wave discharge and without complex symptomatolo¬ gy. Those patients who exhibited absence at¬ tacks on their scheduled visit to the neurology clinic were studied. The EEG reported below in each case report included recording from 21 electrodes and six different montages. Report of CasesCase 1.-An 11-year-old boy had onset of seizures at age 5 when he was noted to have brief staring spells lasting less than a minute, characterized by unresponsiveness. At age 6, these spells began to be accompanied by motor activity when he would push something which might be in front of him, place objects in his mouth, fumble with his hands, and moan. He has had as many as 100 of these a day. At age 10, he had two generalized grand mal seizures. In the family history, his father and his pater¬ nal grandfather had convulsions as children, and one sibling had febrile convulsions.In the course of examination, during hyperventilation, the boy had two seizures character¬ ized by sudden cessation of activity followed by fumbling of the hands, grasping the examiner, and scratching the genitalia, accompanied by a blank stare and an occasional moan. Duration of attack was 40 seconds; cessation, sudden. Neurological ex...
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