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.

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.

Ictal visual hallucinations of numerals.

We studied three children with benign primary occipital epilepsy who had ictal numerical visual hallucinations. This unusual ictal event may occur in patients with left-sided, right-sided, or bilateral interictal spikes, and such attacks may begin in either occipital lobe. This event seems to be occipital, without limbic participation, although the ictal discharge may spread to limbic structures as the seizure evolves.

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.

Lipomas of the corpus callosum and epilepsy.

An analysis of four cases of lipomas of the corpus callosum with epilepsy, and a review of the literature, have led to the following conclusions: (1) Epilepsy as an almost constant feature is often severe, nearly always partial, and begins before the age of 15. (2) Pathophysiology of the seizures appears to be essentially an interhemispheric disconnection (rather than the classic theory that seizures depend upon an infiltration of the cingulate gyri by fibrous tissue growing out from the capsule of the lipoma). This disconnection is responsible for a facilitatory and disinhibitory action that favors the appearance of seizures caused by an epileptogenic lesion (the effects of which remain subthreshold in the presence of an intact corpus callosum playing its normal inhibitory role). This hypothesis is clearly applicable in explaining the epilepsies--in every way comparable--that are observed in agenesis of the corpus callosum and in Marchiafava-Bignami disease.

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.

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.

[Appearance of EEG abnormalities in two patients during long-term treatment with B vitamins]. / Apparition d'anomalies EEG chez deux patients en traitement chronique par des vitamines B.

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.

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.

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.

Long-term results of conventional surgical treatment for epilepsy. Delayed recurrence after a period of 10 years.

The results of the surgical treatment of epilepsy were studied in 44 patients 10 or more years after operation. Thirty-seven patients underwent operation only once; these patients were observed 11-26 years postoperatively. Seven patients had a recurrence within 5 years after operation and required a second operation; these patients were observed 11-17 years after the second operation. Recovery persisted for 15-27 years in 32 patients. There was a recurrence in 12 cases 11-19 years after operation. With one exception, these recurrences were satisfactorily treated medically.

[Gustave Flaubert's illness]. / La maladie de Gustave Flaubert.

All those interested in Gustave Flaubert's illness, during his lifetime as well as after his death, have agreed that he had epilepsy. The one important exception is Jean-Paul Sartre, who, in the 2800 pages of his "Idiot de la famille" claimed that Flaubert was a hysteric with very moderate intelligence who somatized his neurosis in the form of seizures. These, in Sartre's views, were moreover probably hysterical, but possibly epileptic resulting from the existence of a psychogenic epilepsy bred from the neurosis. The basis for this neurosis could have originated at the time of Gustave's birth, as this occurred between those of two brothers who both died young, and as his mother had wished for a daughter. Further development of the neurosis might have taken place during a temporary phase of learning difficulties, exaggerated and exploited by his father to make his youngest son the idiot of a family in which the eldest son was the dauphin. Destroyed in this way, Gustave would have sought refuge in passivity and could have developed a hatred for his father and for his elder brother, who he would have liked to kill before killing himself. But, unable to carry out his wishes and desiring both to die and to survive, Gustave, adolescent, might have chosen the pathway of "false deaths", as exemplified by the seizures. Modern epileptology data enables not only to confirm the epileptic etiology and to discount the hysterical nature of the fits, but also: 1. to establish precise details of the site and nature of the cerebral lesions responsible for the attacks: neonatal atrophy or vascular malformation of the occipitotemporal cortex of the left hemisphere, the only lesion capable of provoking: a) the phosphenes marking the onset of the seizures; b) the intellectual manifestations (forced thoughts or flight of ideas), affective features (panic terror), and psychosensory (ecmnesic hallucinations) or psychomotor (confusional automatism) symptoms accompanying some attacks; c) the loss of speech preceding the loss of consciousness and terminal generalized convulsions. 2. to establish the consequences of the lesion and of the temporal fits on the behaviour of the writer; a) associated hypoactivity (slowness of ideation and writing) and paroxysmal impulsiveness (violent unmotivated angry outbursts); b) disturbed verbal functions (difficulty in finding words); and c) diminished sexuality. As a conclusion, it must be admitted; a) that Flaubert suffered from an organic and not a psychogenic epilepsy, the existence of the latter currently being disputed; b) that this epilepsy modified the behaviour of the author without affecting his genius; c) that, as a result of this, the epilepsy of Flaubert, as that of Dostoïevski, should serve as a defense witness for the unfortunate epileptics thought to be destined for intellectual deterioration only because of the repetition of their seizures.

[Partial epilepsy with complex symptoms and atrophic occipito-parietal lesions. Value of tachoencephalographic examination]. / Epilepsie partielle a sémiologie complexe et lésions atrophiques occipito-pariétales. Intérêt de l'examen tacoencéphalographique

Eleven of the first 500 epileptics examined by axial tomography presented pseudo-porencephalic dilation (cavitary atrophy) of an occipital horn, associated or not with more extensive ventricular dilation. This lesion was found in 2.2% of all the epileptics and in 5% of the epileptics under the age of 20 in this group, a high incidence when compared with that of lesions of the same type located elsewhere. Clinical investigation and E.E.G. carried out on these 11 children showed that they were suffering from either hemiphegia or hemiparesis (8 out of 11 cases) and hemianopsia (7 out of 11 cases), and had serious epilepsy with partial fits (6 cases), usual temporal lobe epilepsy (5 cases), generalized secondary epilepsy (3 cases) or epilepsy defying classification (2 cases). These cases are compared with those recently published by Remillard, Ethier and Anderman. These authors consider that these cerebral lesions are the result of peri-natal thrombosis of the posterior cerebral artery. But in our group of cases, angiographic results do not appear to justify this claim.

[Tomodensitometric study of cerebral accidents causing acute hemiplegia in children]. / Etude tomodensitométrique des accidents cérébraux responsables des hémiplégies aigues de l'enfant.

Computerized Axial Tomography (C.A.T.) easily distinguishes between the two types of cerebral accident responsible for the two major categories of acute hemiplegia in childhood. 1) In hemiplegias which develop in association with hemiclonic status epilepticus (H. H. and H. H. E. syndromes), in the majority of cases there is an appearance of cortico-sub-cortical atrophy involving the whole hemisphere contralateral to the hemiplegia. This atrophy develops following oedema of the hemisphere which accompanies the initial status epilepticus and which is clearly shown by a very early T.A.C. 2) In congenital or acquired hemiplegias not associated with status epilepticus there is, in most cases, an appearance of cerebral infarction or, very rarely, haemorrhage. It is thus possible, from a physiopathogenic standpoint, to draw a clear distinction between these two major forms of acute infantile hemiplegia. The first (H. H. and H. H. E. syndromes) usually result from hemispheric atrophy which develops in association with a unilateral or predominantly lateral episode of status epilepticus, whilst the second group are usually the result of cerebral infarction.