Les rythmes rapides dans les maladies metaboliques chez l'enfant

Misès, J; Moussalli-Salefranque, F.; Hagenmuller, M. P.; Plouin, Perrine

doi:10.1016/s0370-4475(83)80019-7

Cited by 9 publications

(4 citation statements)

References 10 publications

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“…Subsequent reports of this rhythm have emphasized its tendency to occur during the first month of life, in bursts often reaching 150 mV, lasting several seconds in duration; this rhythm can occur when awake and asleep. 2,7 Similar findings were subsequently described by Clow et al 5 and Tharp. 8 Korein et al 3 described EEG patterns ranging from normal to abnormal, with spikes, polyspikes, spike–wave complexes, triphasic waves, severe slowing, and bursts of periodic suppression in patients with MSUD.…”

Section: Discussionsupporting

confidence: 76%

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The Electroencephalogram in Neonatal Maple Syrup Urine Disease

et al. 2012

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Untreated maple syrup urine disease (MSUD) leads to encephalopathy in neonates and causes abnormalities on the electroencephalogram (EEG). A case is presented of MSUD with unique features consisting of a comb-like rhythm before the therapy and its disappearance with therapy is presented. This case illustrates the potential use of the EEG in the identification of this specific cause of a neonatal encephalopathy.

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Section: Discussionsupporting

confidence: 76%

“…4 Fast rolandic rhythms have been described as occurring infrequently in other neonatal metabolic disorders, including methylmalonic aciduria, isovaleric aciduria, and hyperleucinosis. 5…”

Section: Discussionmentioning

confidence: 99%

The Electroencephalogram in Neonatal Maple Syrup Urine Disease

et al. 2012

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“…This EEG feature often occurred in sleep, but quite commonly also in the awake state. Generalized fast activity with high amplitude is also seen in children suffering from infantile neuroaxonal dystrophy (11) and, albeit with low amplitude, in progressive encephalopathies due to aminoacidopathies (19). In the control group of 823 patients with various pathology, these patterns were rare.…”

Section: Discussionmentioning

confidence: 99%

EEG and Evoked Potentials in a Series of 21 Patients with Lissencephaly Type I

1992

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Twenty-one Dutch patients were the subject of an extensive study into lissencephaly type I. One hundred and fourteen EEG's of these patients were studied. The EEG's were compared to 52 EEG's recorded from 21 patients with an atypical cortical dysplasia and to a control group consisting of 882 EEG's recorded from 823 patients for various reasons. The EEG's in the lissencephaly patients showed the following patterns significantly more often: (a) generalized fast activity (8-18/s) with an amplitude higher than 50 microV, (c) sharp- and slow-wave complexes with an amplitude higher than 500 microV, (d) an alternating pattern consisting of bursts of sharp waves alternating with periods of electrocerebral depression. Ninety-five percent of the lissencephaly patients showed pattern (a) or (c) or both compared to only 5% of the patients with an atypical cortical dysplasia and 0.4% in the controls. The SSEP's recorded in ten patients after stimulation of the median nerve were abnormal in all. EEG and evoked potentials appear to be valuable examinations in the (differential) diagnosis of lissencephaly type I.

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“…Another recent study of 72 aminoacidopathies addressed, among others, the neonatal period (Mises et al 1983). The authors included PKU but did not specify citrullinemia.…”

Section: Discussionmentioning

confidence: 99%

THE EEGs OF INFANTS WITH CITRULLINEMIA

Engel

Buist

1985

Develop Med Child Neuro

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SUMMARY Three female infants with citrullinemia were followed clinically, biochemically and by electroencephalography. All three had episodes of vomiting, lethargy and hyperammonemia shortly after birth. The two more severe cases developed convulsions. They were saved by peritoneal dialysis, or repeated exchange transfusions followed by dietary adjustment. Multifocal spikes or repetitive paroxysmal activity of various kinds were seen in the EEGs at times of crisis. There was a lag in the EEG returning to normal after ammonia levels had returned to normal. Citrulline remained elevated in all cases. Follow‐up over years revealed mild spasticity, mental retardation and, in one case, cortical atrophy. RÉSUMÉ L';EEG au cours de la citrullinémie infantile Trois fillettes présentant une citrullinémie ont été suivies cliniquement, biochimiquement et par electroencéphalographie. Toutes les trois avaient présenté des é isodes de vomissements, de somnolence et d'hyperammoniémie peu de temps après la naissance. Des convulsions étaient apparues dans les deux cas les plus graves. Les fillettes furent sauvées par dyalise péritoneale, ou exsanguinotransfusions suivies par un régime diététique. Des pointes multifocales et des activités paroxistiques répétitives de type varié ont été observées dans les EEG au moment des crises. Il y a eu un intervalle avant le retour de l'EEG à l'état normal, après que les taux d'ammoniémie soient retournés à la normale. La citrulline est restée élevée dans tous les cas. Le suivi au cours des années a révélé une spasticité modérée, un retard mental et dans un cas une atrophie corticale. ZUSAMMENFASSUNG Das EEG bei kindlicher Zitrullinämie Drei weibliche Säuglinge mit Zitrullinämie wurden klinisch, biochemisch und elektroencephalographisch kontrolliert. Alle drei hatten kurz nach der Geburt Anfälle von Erbrechen, Lethargie und Hyperammonämie. Die zwei schwer betroffenen Kinder entwickelten Krampfanfälle. Sie wurden durch Peritonealdialyse oder wiederholte Austauschtransfusionen mit anschliessender Diät gerettet. In kritischen Situationen wurden im EEG multifokale Spikes oder wiederholte verschiedenartige paroxysmale Aktivitäten gesehen. Nach der Normalisierung der Ammoniumspiegel kehrte das EEG verzögert zur Norm zuruck. Das Zitrullin blieb in alien Fällen erhöht. Jahrelange Kontrolluntersuchungen ergaben eine geringe Spastik, eine geistige Retardierung und, in einem Fall, eine kortikale Atrophie. RÉSUMÉN El EEG en el lactante con citrulinemia Tres lactantes hembras con citrulinemia fueron seguidos clínica, bioquímica y electroencefalográficamente. Los tres tenían episodios de vómito, letargia e hiperamoniemia poco después de nacer. Los dos casos más graves desarrollaron convulsiones. Se salvaron gracias a una dialisis peritoneal o por exsanguino transfusión repetidas, seguidas de un ajuste diétético en el EEG en el momento de las crisis se observaron espigas o actividad paroxistíca repetitiva de diversos tipos. Hubo un retraso en el retorno a la normal del EEG despues de la normalizacion de la amo...

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Les rythmes rapides dans les maladies metaboliques chez l'enfant

Cited by 9 publications

References 10 publications

The Electroencephalogram in Neonatal Maple Syrup Urine Disease

The Electroencephalogram in Neonatal Maple Syrup Urine Disease

EEG and Evoked Potentials in a Series of 21 Patients with Lissencephaly Type I

THE EEGs OF INFANTS WITH CITRULLINEMIA

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