Absence status triggered by pallid syncopal spells.
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Humans↗
Biomedical subjects
H Gastaut
Publications and source records attributed to H Gastaut.
The enforced nap: a simple effective method of inducing sleep activation in epileptics.
The authors routinely carry out sleep activation in suspected cases of epilepsy when the EEG performed in the waking state gives insufficient data. The technique used, referred to as 'the forced nap', allows the induction of 60 min sleep in 80% of all subjects and 90% of those under 16 years of age. This method gives results comparable with (and probably better than) those obtained by EEG recording after 24 h sleep deprivation, but is much simpler to carry out and can be used by all hospital-based EEG laboratories. The widespread use of this method should allow to change the current situation. It was found that more than 50% of hospital-based EEG laboratories do not perform sleep activation, and the remaining centers restrict this technique to a very limited number of patients, thus depriving almost all epileptics of an effective diagnostic tool.
Arousal↗
Syncopal attacks compulsively self-induced by Valsalva's maneuver associated with typical absence seizures. A case report.
A girl of borderline intelligence developed repeated syncopal attacks that were compulsively self-induced, apparently by forced expiration against a closed glottis (Valsalva's maneuver). In addition, she had typical absence seizures triggered by her apneic attacks, which could also be induced by hyperventilation. The absence seizures were suppressed by treatment with valproate sodium, which had no effect on the self-induced apneas. These were considerably improved with fenfluramine hydrochloride treatment, which was also associated with marked improvement of her previously abnormal behavior.
Apnea↗
[Appearance of EEG abnormalities in two patients during long-term treatment with B vitamins].
Two female patients, one treated for epilepsy by phenobarbital (PB) and carbamazepine (CBZ), the other suffering from neurosis and free of any drug therapy, were given group B vitamins (riboflavin and nicotinic acid respectively) and showed during the long-term administration of these vitamins 2-3 Hz generalized electro-encephalographic (EEG) abnormalities. These EEG changes were detected incidentally and were not associated with any clinical manifestation; they disappeared progressively within several weeks after discontinuation of vitamin therapy. In our opinion, it is likely that the treatment with B vitamins was entirely responsible for the EEG changes, as one of the patients did not receive any other drug, while the other did not show any significant change of PB and CBZ plasma levels during vitamin therapy. However, other cases reported will be necessary to confirm our impressions.
Aged↗
Compulsive respiratory stereotypies in children with autistic features: polygraphic recording and treatment with fenfluramine.
Syncopes due to compulsive respiratory stereotypies were studied in eight patients with autistic features. Most had been referred for episodes thought to be intractable epileptic seizures. Polygraphic recording showed two types of syncope, one induced by prolonged apnea and the other by a prolonged Valsalva maneuver. Fenfluramine, 1.5-3 mg/kg per day, was given in an open trial. In four of five cases with frequent Valsalva maneuvers, respiratory stereotypies and syncopes were suppressed for 2-18 months. Patients with periodic apneas were more severely retarded and had less clear benefit. Side effects consisted of dose-dependent sedation and mild weight loss which stabilized without interrupting treatment. We suggest that these syncopes are volitional and may be associated with pleasant sensations. A double-blind placebo-controlled trial of fenfluramine seems warranted in such patients.
Apnea↗
Lissencephaly (agyria-pachygyria): clinical findings and serial EEG studies.
Fifteen cases of lissencephaly were studied and the literature reviewed. The authors conclude that the clinical findings of lissencephaly in infancy are non-specific, consisting of developmental delay and hypotonia. While the CT scan establishes the diagnosis, it may also be strongly suggested by an EEG showing 'major fast dysrhythmia', characterized by abnormally rapid, very high-voltage activity, predominantly in the alpha and beta frequency bands. Some possible mechanisms for this highly suggestive EEG pattern are proposed.
Brain↗
The risk of automobile accidents with seizures occurring while driving: relation to seizure type.
Automobile accidents occurred with 55% of seizures in epileptic drivers with both well-described seizures at the wheel and a well-classified epilepsy diagnosis. A further 38% of seizures would have led to accidents in less fortunate settings. Complex partial seizures occurred in 81% of patients and were responsible for 88% of accidents. Immediate alterations of consciousness were significantly more likely to lead to accidents than were seizures beginning with an aura. Motor activity during simple partial seizures was also responsible for some accidents. Differences in seizure frequency may account for different crude accident rates among studies of epileptic drivers. We suggest guidelines for licensing restrictions that seem safe and reasonable.
Accidents, Traffic↗
Prolonged ictal paralysis: electroencephalographic confirmation of its epileptic nature.
Somato-inhibitory status epilepticus is a rare condition which may pose some problems of differential diagnosis. The authors describe the case of a girl who suffered from episodes of prolonged ictal paralysis lasting from some minutes to some hours. One seizure was described 2 hours from onset. The EEG showed a continuous spike activity on the right side, predominantly on the parieto-rolandic areas. Status was stopped by the administration of clobazam per os. The physiopathological mechanism of this rare form of status epilepticus is discussed.
Adolescent↗
Benign versive or circling epilepsy with bilateral 3-cps spike-and-wave discharges in late childhood.
Twenty-eight of 920 patients seen between the ages of 8 and 20 years for a first seizure manifested versive or circling events associated with bilateral rhythmic 3-cps spike-and-wave discharges. In 21 (75%), the seizures appeared between the ages of 10 and 14 years. Paroxysmal adversion or gyration was always toward the same side in a given patient. "Break of contact" during the ictus was found in 70% of the seizures, and secondary generalization occurred in half. Ictal electroencephalographic recordings failed to show a focal hemispheric origin. Additional features were: absence of prior major diseases; normal results of neurological and neuroradiological examinations; excellent response to anticonvulsant treatment with sodium valproate and/or phenobarbital; frequent family history of epileptic seizures (25% of patients); and association with generalized seizures (57.2% of patients). We conclude that versive or circling epilepsy with bilateral rhythmic 3-cps spike-and-wave discharges represents a benign form of primary generalized epilepsy in late childhood.
Adolescent↗
Use of clobazam in certain forms of status epilepticus and in startle-induced epileptic seizures.
Sixteen patients with status epilepticus (SE) and 13 patients suffering from startle-induced epilepsy were treated with clobazam (CLB). Status epilepticus was of the absence (six cases), myoclonic-absence (one case) myoclonic (one case), tonic (one case), and single or complex partial (seven cases) type. In all patients, consciousness was sufficiently retained during SE to allow CLB ingestion. Clobazam was administered in a single oral dose (0.5 to 1.70 mg/kg, mean 1.08 mg/kg) under electroencephalographic monitoring. In all patients but one, SE terminated within 15 to 31 min (mean 24.4 min) after CLB administration without significant side effects. Treatment of SE depends on the clinical context and type of SE. We conclude that CLB is a useful drug in the management of some forms of SE. In 13 patients suffering from startle-induced epileptic seizures, CLB was added to the previous ineffective treatment protocol. Of these 13 patients, 15.4% were completely unresponsive, 23.1% experienced drug resistance, and 61.5% achieved a good control (91.5% reduction of the reflex seizures) for a mean of 22.7 months. Drowsiness was observed in four patients, whereas favorable side-effects were observed in four patients.
Adolescent↗
The long-term course of primary generalized epilepsy with persisting absences.
The long-term course of primary generalized epilepsy with absences persisting after the age of 30 to 61 years was studied in 26 patients, each followed clinically and by EEG for 20 to 37 years. Whether treated or not, absences became less frequent, but only rarely ceased, and 92% of patients with persisting absences eventually developed generalized convulsions. EEG background activity did not deteriorate, and the classic spike and wave complexes remained typical in 84% of patients. In 36% of cases, almost all of them women, psychomotor slowing was observed, which did not affect job performance. It was not related solely to duration or severity of epilepsy, or to antiepileptic drugs, and some hormonal mechanism may be partly responsible.
Adult↗
Treatment of self-induced photosensitive epilepsy with fenfluramine.
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Adolescent↗
[Interictal EEG activity specific for a particular variety of temporal epilepsy. Epileptic temporal theta rhythm].
The authors stress the importance of a local theta-rhythm first described by Chatrian in 1953 and largely neglected by clinical electroencephalographers. This rhythm is constituted of waves at 3-5 c/sec; it is localised on the anterior temporal region in one hemisphere and has the same value as isolated temporal spikes or spike-waves for the diagnosis of temporal epilepsy. It furthermore presents the advantage of being specific for a particular form of temporal epilepsy of precocious appearance subsequent to a sclerosis of the anterior median temporal cortex acquired at birth or in early childhood.
Adolescent↗
[Extraordinary variant of the alpha rhythm].
The authors describe a variant of alpha rhythm, which is remarkable because of its great amplitude (200-300 microV). It is observed quite exceptionally in children and adolescents with a severe encephalopathy of either ante- or perinatal etiology. They suggest that the surplus amplitude of this alpha rhythm could depend upon a deafferentation of the occipital cortex in patients with severe amblyopia.
Agenesis of Corpus Callosum↗
Classification of the epilepsies.
We review the development of the classification of the epilepsies. Primary epilepsies are relatively benign, usually age-limited syndromes without clinical or radiologic evidence of brain lesions, and are related to a heritable constitutional predisposition to epilepsy. They usually respond well to antiepileptic drugs. The biochemical correlates of primary generalized epilepsy have been demonstrated in animals and man and have been related to diffuse cortical hyperexcitability, which has been linked to this disorder. The pathophysiology of the primary partial or focal epilepsies is poorly understood but does not appear to depend on focal brain lesions. We suggest that these are due to relatively localized areas of cortical hyperexcitability confined to isolated corticothalamic sectors and depend on interrelations between a constitutional cortical hyperexcitability and normal cortical maturation. The secondary epilepsies are associated with clinical and radiologic evidence of brain lesions and are often resistant to anticonvulsants. Secondary generalized epilepsies, with an associated diffuse encephalopathy, are typified by the Lennox-Gastaut syndrome. The presentation of the secondary partial epilepsies depends on the site of the lesion. The pathogenesis of epilepsy is multifactorial, and a preexisting constitutional predisposition can interact with an acquired diffuse or focal encephalopathy, facilitating the clinical expression of one or the other.
Adolescent↗
Absence status appearing on eye-closure.
Two personal observations of absence status appearing on eye closure are described and similar cases in the literature considered. Treatment of such an absence status with clobazam is proposed.
Adolescent↗
[Efficacy of fenfluramine for the treatment of compulsive behavior disorders in psychotic children].
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Child↗
[Treatment of certain forms of status epilepticus by means of a single oral dose of clobazam].
The authors administered clobazam (CLB) to treat status epilepticus in 16 patients from 3 to 62 years old. Status epilepticus was of absence type (6 cases), myoclonic-absence type (1 case), myoclonic type (1 case), tonic (1 case) and elementary or complex partial (7 cases). In all patients consciousness was sufficiently retained to allow ingestion of the drug. CLB administration was performed under EEG monitoring, in a single oral dose averaging 1.0 mg/kg. In 15 patients, the status was stopped after a period ranging from 19 to 31 min without significant sedation or other side effects. In one patient a somatomotor status epilepticus, lasting 8 days, terminated 6 h after drug administration. It is well known that the efficacy of a drug on status epilepticus depends on the clinical context and semiological form of the status. Nevertheless, the authors conclude that the pharmacokinetic properties of CLB, its mild sedative effects and ease of administration, make this a useful drug in the management of some forms of status epilepticus.
Adolescent↗