[An electroencephalographic study of disorders of amino-acid metabolism during the first days of life (author's transl)].
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.
D000592:Amino Acid Metabolism, Inborn Errors / Q000503:physiopathology*; D000641:Ammonia / Q000097:blood; D004569:Electroencephalography; D005998:Glycine / Q000097:blood*; D006801:Humans; D007231:Infant, Newborn; D007232:Infant, Newborn, Diseases / Q000503:physiopathology*; D007930:Leucine / Q000652:urine; D008764:Methylmalonic Acid / Q000097:blood
D000641:Ammonia; D008764:Methylmalonic Acid; D007930:Leucine; D005998:Glycine
Etude du tracé des premiers jours de vie dans les amino-acidopathies.