Summary
The fatty acid composition of cerebrosides and sulfatides in the white matter of a brain from a patient with adult MLD was analysed by gas-liquid chromatography and compared to the results obtained from a normal control of the same age. In adult MLD a relative increase of short chain fatty acids affecting mainly the cerebrosides and also a diminution of unsaturated fatty acids affecting mainly the sulfatides were found. The whole composition of the sulfatides was altered to a larger extent than of the cerebrosides and this result was in contrast to results obtained in infantile MLD. These findings were discussed on the basis of a similar enzymatic defect in both adult and infantile cases and with regard to a chronic demyelinating process.
Zusammenfassung
Aus der zentralen Marksubstanz des Gehirnes eines Patienten mit adulter MLD wurde die Fettsäurezusammensetzung von Cerebrosiden und Sulfatiden mittels Gaschromatographie analysiert und mit Ergebnissen eines normalen Vergleichgehirns verglichen. Bei adulter MLD wurden eine relative Vermehrung kurzkettiger Fettsäuren (besonders bei den Cerebrosiden) und eine relative Verminderung ungesättigter Fettsäuren (besonders bei den Sulfatiden) gefunden. Die Fettsäurezusammensetzung der Sulfatide war im Gegensatz zu kindlichen Fällen stärker verändert als die der Cerebroside. Dieser Befund wird im Hinblick auf einen gleichartigen Enzymdefekt bei infantiler und adulter MLD und den chronischen Entmarkungsprozeß bei adulter MLD diskutiert.
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Dedicated with gratitude and affection to Prof. H. J. Bauer on the occasion of his 60th birthday.
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Pilz, H., Heipertz, R. The fatty acid composition of cerebrosides and sulfatides in a case of adult metachromatic leukodystrophy. Z. Neurol. 206, 203–208 (1974). https://doi.org/10.1007/BF00316534
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DOI: https://doi.org/10.1007/BF00316534