The Choreiform Syndrome in Children

UMMARY While taking EEG'S on some hyperactive children who had been referred to the doctor because of poor school performance, the authors noticed that there were chorea-like twitchings of the limbs and head, showing up on the EEG as muscle artefacts from the cervical and temporal muscles. A group of 50 children were therefore investigated very fully. These children showed choreiform movements, by which we mean slight, jerky movements occurring quite irregularly and arythmically in different muscles. The muscles of the tongue, face, neck and trunk were involved in all cases, but in a small group (18 per cent) the distal muscles were not involved and these cases were grouped together as the ‘proximal form’. The question whether there was a causal relationship between these neurological phenomena and the behavioural problems for which the children were referred is discussed. The case-histories showed that 66 per cent of the patients had had one or more obstetrical or postnatal troubles. In another 12 per cent the histories suggested that complications had probably occurred, though detailed information was lacking. Finally, the authors refer to the work by Dijkstra on the hyperexcitability syndrome which he described in the neonatal period and which, on follow-up, was significantly related to the choreiform syndrome later. The choreiform syndrome seems to be a form of minimal brain damage and falls, by definition, into the category of cerebral palsy. No successful form of drug therapy has yet been discovered. RESUME Le syndrome choreiforme chez les enfants Pendant l'enregistrement de I'EEG de quelques enfants instables qui avaient ete renvoyes pour travail scolaire insuffisant, les auteurs ont remarque des mouvements choreiformes des extremites et de la tete qui se presentaient a I'EEG comme des artefacts provenant des muscles cervicaux et temporal. En consequence, un groupe de 50 enfants a ete examine de facon approfondie. Ces enfants presentaient des mouvements choreiformes, nous entendons par la des mouvements saccades, survenant de facon irreguliere et arythmique dans differents muscles. Les muscles de la langue, du visage, du cou et du tronc etaient impliques dans tous les cas, mais dans un petit groupe, 18 sur cent des cas, les muscles distaux n'intervenaient pas et ces cas ont ete groupes en une ‘forme proximale’. Les auteurs discutent pour savoir s'il y a un rapport de cause a effet entre ces phenomenes neurologiques et les troubles du comportement, pour lesquels ces enfants furent examines. Des etudes retrospectives ont montre que 66 sur cent de ces sujets avaient presente un ou davantage de troubles obstetricaux ou post-nataux. Dans 12 sur cent d'enfants autres, l'observation suggerait l'existence de complications tres probables, mais on a manque de details precis. Finalement, les auteurs mentionnent le travail de Dijkstra, qui a decrit le syndrome d'hyperexcitabilite pendant la periode neonatale et qui, dans la suite de l'evolution, presentait une correlation significative avec le syndrome choreiforme. Le syndrome choreiforme semble etre une forme de lesion cerebrale a minima et tombe par definition dans le groupe des paralysies cerebrales. On n'a pas decouvert jusqu'a present de therapeutique medicamenteuse efficace.