Adrenoleukodystrophy progressed from frontal lobes--clinico-pathological, electron microscopic and chemical study of a case.
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.
D000293:Adolescent; D000307:Adrenal Gland Diseases / Q000473:pathology*; D001921:Brain / Q000000981:diagnostic imaging / Q000473:pathology; D001923:Brain Chemistry; D002549:Diffuse Cerebral Sclerosis of Schilder / Q000473:pathology*; D005227:Fatty Acids / Q000032:analysis; D005625:Frontal Lobe / Q000648:ultrastructure; D006801:Humans; D008607:Intellectual Disability / Q000150:complications; D008297:Male; D008854:Microscopy, Electron; D011877:Radionuclide Imaging; D014057:Tomography, X-Ray Computed
D005227:Fatty Acids