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.

[Brain abscess in the newborn infant (27 cases: initial electroclinical study, course]. / Abcès cérébraux néo-nataux (27 cas): étude électroclinique initiale, évolution.

The authors studied 27 brain abscesses in neonates. The neonates were divided in two groups: neonates with seizures and neonates without seizures. The authors tested the diagnostic and predictive value of the initial EEG aspect. They noted the absence of correlation between EEG focal abnormalities and localizations shown by the CT scan. They observed a severe electroclinical evolution in more than fifty percent of the cases presented.

[Significance of discontinuous traces in the full-term newborn infant]. / Significations du tracé discontinu chez le nouveau-né à terme.

This article is devoted to a consideration of the clinical prognostic significance of "tracé discontinu". The authors first distinguish "tracé discontinu" from the "tracé paroxystique" and tracé alternant" patterns in the newborn. The distinction is very important. The bad prognosis of the "tracé paroxystique" is well known, but prognosis of the "tracé discontinu" seems to be different. If the "tracé discontinu" is permanent, there was a 50% favourable outcome in our 22 newborn babies brought to the reanimation department. If some continuous activity can be observed: beginning of sleep organisation, then the prognosis seems to be better: 61% of our cases. As blood levels of anticonvulsants are rarely measured it seems difficult, given our present knowledge, to appreciate anticonvulsive drug effects on EEG recordings. Thus 3 aims should be pointed out: --Measurements of anticonvulsant blood level each time a "tracé discontinu" is observed in a treated newborn. --Early EEG recordings of sufficient duration to obtain eventually some continuous tracing. --Correct differentiation between "tracé paroxystique" and "tracé discontinu" for the clinician: "tracé discontinu" in a full-term newborn should never allow the paediatrician to stop treatment.

[Fast rhythms in metabolic diseases in children]. / Les rythmes rapides dans les maladies métaboliques chez l'enfant.

The authors study fast EEG rhythms in amino acidopathies of the newborn and in progressive encephalopathy of the central nervous system in children, when an inborn error of metabolism has either been found or is suspected. In the first group: amino acidopathies (including phenylketonuria), fast rhythms on the EEG of the neonates are of low amplitude, spindle-like bursts of 7-13 Hz and usually located in rolandic areas. This activity progressively disappears. Later, fast rhythms of beta-frequency and low voltage are spread on the whole scalp with a normal EEG organisation. They are seen in the favourable or rather favourable development of children receiving a diet. In the second group: progressive encephalopathy of the central nervous system, fast rhythms of 14-24 Hz appearing at a rather late stage of the disease, while the normal background activity completely disappears. In neuro-axonal dystrophy, where they are considered as 'specific,' they are of very high voltage, i.e., 50-200 microV. The authors insist on the importance of EEG fast rhythms both in amino acidopathies of the newborn, as a contributive factor to diagnosis and prognosis, and in progressive encephalopathies of the central nervous system, as a valuable tool in the diagnosis of numerous affections.

[EEG recordings in prolonged hypercapnia during surgery of a cervico-mediastinal tumor in a 3-year-old child]. / Enregistrement EEG pendant une hypercapnie prolongée au cours d'une intervention pour tumeur cervico-mediastinale chez un enfant de 3 ans.

An EEG has been recorded during surgery for a cervico-mediastinal desmoid fibroma in a 3-year-old child. Soon after anesthetic induction a bronchospasm occurred, followed by major respiratory difficulties. During more than 3 h, hypercapnia reaching 15-24 kpa was noted without hypoxia. Meanwhile the EEG recording was inactive. The respiratory difficulties subsided only with the surgical liberation of the trachea. As the hypercapnia decreased, the EEG began to show "burst suppressive" activity. Progressively the tracing was more and more continuous with slow waves until complete recovery. The following day the EEG was quite normal. The mechanisms of these EEG features are discussed with regards to anesthetic drugs and to brain hypercapnic edema.

[Convulsive seizures in the neonatal metabolic diseases (author's transl)]. / Les crises convulsives dans les maladies metaboliques a revelation neo-natale.

Of 61 newborns with disorders in amino acid metabolism, 20 had seizures; 15 of them have been recorded. Clinically, seizures are mostly partial clonic jerks. There seems to be some homogeneity of EEG features in relation to causal amino acidopathy. In every case where seizures, either electroclinical or only infraclinical, occurred the evolution was very poor, even in those diseases that would have had a good prognosis. Therefore the EEG in the newborns is especially interesting, both as an aid to diagnosis by interictal recording and as a prognostic tool by registration of seizures.

[Unilateral epilepsy with hemiplegia in the child (author's transl)]. / A propos d'une forme d'epilepsie hemiconvulsive avec hemiplegie chez l'enfant.

Fifteen children between the age of 3 and 7 years who had had unilateral seizure followed by a transitory hemiplegia (without fever) were observed. This seizure was the only one in 13 of the children; a second fit appeared in 2 others. The average duration of the observation period was 10 years, 6 months. The evolution of the EEG shows that after a post-ictal delta focus contralateral to the hemiplegia, spike foci of variable localisations were formed in 12 cases. These cases, chosen because of their benign evolution, seem to be related closely to rolandic paroxysmal epilepsy (EPR).

[EEG appearance during waking and falling asleep in children aged 1-4 years (without serious pathology)(author's transl)]. / Aspects EEG de l'endormissement et du réveil chez l'enfant de 1 à 4 ans (pathologie lourde exclue) (author's transl)]

The authors have studied the EEG characteristics of 188 children aged 1-4, without serious pathology, during waking and falling asleep spontaneously in the afternoon. The children were divided into 3 groups: aged 12-23 months, 24-35 months and 36-47 months. On falling asleep the EEG appearances varied, but hypersynchrony was most marked in the youngest children and in the older group theta activity was commonest. Only 3 of the 188 children had runs of paroxysmal activity on falling asleep. The waking study again showed appearances governed by the patients' age with respect to 7 c/sec activity on provoked waking, but the appearances were more diverse on spontaneous waking and anterior theta activity only occurred in the oldest children. In general, a relation appeared to exist between the EEG appearance on falling asleep and on waking, in the respect that children falling asleep without hypersynchrony never wake up with it.

Vasomotor origin of intracranial pressure waves in hydrocephalic infants.

By measuring cerebral blood volume (CBV) and intracranial pressure (ICP) variations at the same running time during sleep, it has been demonstrated that the ICP wave which appears during the REM sleep in hydrocephalic infants is produced by intracerebral vaso-dilatation. Nine infants with stabilized hydrocephalus were investigated by non-invasive means: REM phases were distinguished with the usual polysomnographic electrodes. Intracranial pressure was measured with a fontanel palpation transducer and CBV variations were obtained by recording 99mTc activity at the head level after in vivo labelling of red cells with 99mTc--pertechnetate. The time-activity curves, obtained from regions of interest and selected on the sequential radioisotope images, show that an increased ICP wave, occurring during the REM period, is related to a simultaneous increase in the blood volume, limited to the cerebral sector and not to the area of the external carotid artery.

[Sleep polygraphy with recording of intracranial pressure and measure of cerebral blood volume in hydrocephalic infants (author's transl)]. / Polygraphie du sommeil avec enregistrement de la pression intracranienne et mesure du volume sanguin cérébral chez le nourrisson hydrocéphale.

This study was carried with the intention of explaining the causes of modifications in intracranial pressure (ICP) during paradoxical sleep (PS) in normal and hydrocephalic infants, and establishing relationships between these modifications and cerebral blood volume (CBV). All tests (conventional sleep polygraphy, ICP measured by a transducer on the fontanel, CBV measured by isotopic labelling of red blood cells in vivo) were carried out without use of surgical procedures. During paradoxical sleep there was a sustained wave of increased intracranial pressure lasting from 10 to 20 minutes, as well as phase-type variations lasting no longer than 1 minute. Increased ICP was also observed in the normal subjects. Recordings show that there is well-defined correlation between the sustained wave of high intracranial pressure and an increase in CBV.

[The E.E.G. in non-ketotic hyperglycinaemia (author's transl)]. / l'E.E.G. dans les hyperglycinémies sans cétose.

12 cases of non-ketotic hyperglycinemia in neonates diagnosed at a time of neurological distress were studied. A characteristic tracing was observed permitting correct diagnosis in 6 cases. It consisted of a burst suppression characterized by high voltage complexes separated by low amplitude sequences. This appearance remained until the 15th day, with no electroclinical changes. In cases followed the trace closely resembled hypsarrhythmia.

[E.E.G. appearances in hyperphenylalaninaemia (author's transl)]. / Aspects évoluties de l'E.E.G. dans les hyperphénylalaninémies.

Since 1973 the authors have studied the EEG appearances in hyperphenylalaninaemia : 82 children were studied, comprising : 68 children with phenylketonuria, 33 of whom had been treated within the first 3 months of life, whilst 35 had only started therapy after 9 months; 14 patients with hyperphenylalaninaemia. The morphological appearances seen commonly were compared with those described in the literature: the abnormalities and maturational changes were observed, including background rhythm changes, characteristic appearance of high amplitude, sharp spindles and percentages of generalised fast rhythms. Incidence of re-evaluation tests was also studied. Serial changes represented the most important aspect of this study, correlations being possible between the tracings and the child's development. The effects of correct dietary management, nonadherence to treatment, and the changes occurring on stopping treatment were also assessed. An assessment of the value of the EEG in following these cases could thus be made.

[The E.E.G. in methylmalonic acidaemia (author's transl)]. / l'E.E.G. dans les acidémies méthylmaloniques.

The EEG study was carried out on 8 cases of methylmalonic acidaemia, in the neonatal period (3 cases), developing later with a ketotic coma, hyperglycinemia, hyperammonaemia, leucopenia and thrombocytopenia (3 cases) and detected before birth (2 cases). The tracings of the neonates in the first group had a periodic appearance. The 2 children detected pre-natally had essentially normal EEGs bar a slightly faster rhythm. The tracings of the children in ketotic coma were similar to those seen in metabolic coma of other cause. The value of this study was, besides characterizing the EEC pattern which resembled that seen in other metabolic illnesses in the neonatal period, to study the changes in the EEG trace and their improvement during dialysis treatment. In one case recordings were made throughout the duration of dialysis.

["Spike focus" during the evolution of febrile convulsion: an electrophysiological and clinical study of 35 patients (author's transl)]. / Les "foyers de pointes" au cours de l'évolution des convulsions hyperpyrétiques étude électro-clinique à propos de 35 cas.

Out of 500 patients with febrile convulsions, 41 were found to have a spike focus on E.E.G., 35 of these were then followed up. The clinical features of the seizure in these 35 children did not differ from the group in general. The initial focus was practically always unilateral, except for one case with an asynchronous bioccipital focus. The most frequent site was occipital (43 p. 100). Migration of the focus was relatively rare. In 35 patients the focus disappeared, in 45 p. 100 within a year and in 88 p. 100 within 3 years. Following the disappearance of the focus, generalised sharp wave activity was seen in 30 p. 100 cases, all 35 children being under treatment. All the seizures with the exception of two children who have become epileptic were benign rendering long term anticonvulsant therapy unnecessary.

[Clinical course following a neonatal E.E.G. recording reported as severely abnormal (author's transl)]. / Evolution clinique après un tracé néonatal considéré comme grave.

The purpose of this study was to evaluate the prognostic value of neonatal E.E.G. tracings in children born at term. The clinical course of 45 children was followed and related to E.E.G. abnormalities reported during the first 5 days of life. Essentially the findings confirmed those previously reported by others. However some differences were noted: paroxysmal tracings were not associated with a poor clinical state, and moderately abnormal tracings (the prognostic significance of which has never been defined) led on sometimes to a severe encephalopathy. We wish to stress certain aspects of our findings: -recordings in the first 24 hours of life may be misleading. -recordings, to be of value, must be taken before any treatment which could induce paroxystic E.E.G. patterns. -E.E.Gs should be repeated during the post-natal period when the findings are non-specific. -the prognostic significance of tracings reported as "generalised or localised overactivity" should be evaluated.

[An electroencephalographic study of disorders of amino-acid metabolism during the first days of life (author's transl)]. / Etude du tracé des premiers jours de vie dans les amino-acidopathies.

The authors have attempted a systematic E.E.G. study in 32 neonates suffering from disorders of amino-acid metabolism, during the first days of life. These consisted of cases with ketosis (13 cases of leucinosis, 5 methylmalonic acidaemia, isovaleric acidaemia and 3 with hyperlactacidaemia) and cases without ketosis (6 cases of hyperglycinaemia and 3 with congenital hyperammonaemia). A study of the E.E.G. showed some characteristic features, the most typical of which were: -a periodic tracing with large sharps complexes intermingled with less active periods occurring in every case of hyperglycinaemia without ketosis, in 2 cases of leucinosis and 2 cases of methylmalonic acidaemia. This record indicates a poor prognosis. -a less stereotyped periodic tracing with variable evolution. -distinctive figures characterised by rapid Rolandic rhythms always found in cases of leucinosis compared with sharp spindles (between the 10th and 30th day). It is concluded that the E.E.G. patterns are not in close correlation with the anatomical lesions.

[The E.E.G. during althesin anaesthesia in paediatrics (preliminary study) (author's transl)]. / Etude de l'E.E.G. au cours de l'anesthésie par l'alfatésine en pédiatrie.

The authors studied 11 children (mean age 5 1/2 years) before and during anaesthesia, and then several times from 1 to 6 hours after the injection of althesin. The anaesthetic agent was either pure althesin or althesin combined with dextromoramide. The electroclinical correlations described for adults are also found in children: slow waves, discontinuous and then isoelectric recording during the operation stage, then rapid recovery after ending drug administration. The special interest of this study was the analysis of E.E.G. recordings during the hours following clinical recovery: only once was the recording like that of full consciousness. Repeated recordings were all of drowsiness and even sleep, despite the clinical state of the subject. A short associated study concerns the E.E.G. of neonates during their first 24 hours, born to mothers anaesthetised with alfathesin.