L'E.E.G. Dans Les Acidémies Méthylmaloniques

Summary 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 matabolic 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.