Adrenoleukodystrophy progressed from frontal lobes--clinico-pathological, electron microscopic and chemical study of...

A young male patient with adrenoleukodystrophy progressed from the frontal to the parieto-occipital region was reported. The spreading pattern of the brain lesion in this patient was confirmed by a serial examination of Technetium scintigrams and the neuropathological findings. The high uptake area of the isotope in the scintigrams seemed to represent the active demyelinating and infiltrative-reactive process in the white matter. A chemical analysis of the frozen white matter tissue of the patient revealed the existence of long chain fatty acids mor than C 22 in cholesteryl esters and gangliosides. It is suggested by this case that the demyelination process can start in the frontal white matter also in adrenoleukodystrophy as well as in Schilder's disease.