par Farriaux, Jean Pierre;Dhondt, Jean Louis;Formstecher, Pierre;Lagrou, A;Mardens, Yves Léonce 
;Kint, J;Fontaine, C.;Martin, J.J.;Pollitt, RJ
Référence Acta neurologica belgica, 76, 1, page (26-34)
Publication Publié, 1976
;Kint, J;Fontaine, C.;Martin, J.J.;Pollitt, RJRéférence Acta neurologica belgica, 76, 1, page (26-34)
Publication Publié, 1976
Article révisé par les pairs
| Résumé : | A newborn infant affected by argininosuccinic aciduria was treated with a hypoproteinaemic diet and survived up to the age of three months. Post mortem biochemical studies have confirmed the absence of argininosuccinase activity in kidney, liver and brain and a marked storage of argininosuccinic acid in the brain. The histological study of the central nervous system shows no Alzheimer type II cells; there is neither demyelination nor spongiosis. Such results are totally different from those recorded in untreated cases; they represent very likely the effects of the diet which has controlled the hyperammoniaemia and prevented a deficiency of arginine. |
