[Morphologic study of so-called periodic anomalies in children].
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Biomedical subjects
Publications and source records attributed to J Mises.
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The authors analyse the technical problems encountered in using E.E.G. in children's reanimation. They show some examples which illustrate the importance of E.E.G. in diagnosis and therapy of such cases. Emphasis is laid upon the need for close collaboration between the EEGers and the reanimation staff.
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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 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.
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.
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.
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 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.
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.
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.