[Antioxidative therapy in ceroid lipofuscinosis].
The pathogenesis of the ceroid lipofuscinoses (CLF) is unknown. The various types (infantile, late-infantile, juvenile and adult type of CLF) are characterized by accumulation of ceroïd and lipofuscin material in neuronal and extraneuronal tissues. One of the leading hypotheses claims this accumulation to be due to lipid peroxidation. Therapeutical formula's were introduced by Zeman and subsequently by Santavuori and Westermarck, consisting of antioxidants. We applied the Westermarck-formula in two patients with late-infantile CLF, M. Jansky-Bielschowsky, however, without any therapeutical effect. Application of the formula in 3 patients with the juvenile CLF, the M. Batten-Spielmeyer-Vogt, resulted in a mitigated course of the disease. We concluded that antioxidant therapy in CLF, as applied thus far, has to be considered to be symptomatic rather than causal.
Rotteveel
J J
JJ
Interdisciplinair Kinderneurologisch Centrum, St. Radboudziekenhuis, Nijmegen.
Mullaart
R A
RA
D000293:Adolescent; D000328:Adult; D000975:Antioxidants / Q000627:therapeutic use*; D002648:Child; D005231:Fatty Acids, Unsaturated / Q000378:metabolism; D006801:Humans; D015227:Lipid Peroxidation; D008214:Lymphocytes / Q000648:ultrastructure; D009472:Neuronal Ceroid-Lipofuscinoses / Q000097:blood / Q000188:drug therapy* / Q000378:metabolism
D000975:Antioxidants; D005231:Fatty Acids, Unsaturated
Anti-oxydatieve therapie bij de ceroïd lipofuscinoses.