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Adrenoleukodystrophy Progressed from Frontal Lobes –Clinico‐pathological, Electron Microscopic and Chemical Study of a Case–
Author(s) -
Suetsugu Motohiro,
Torichigai Shinichi,
Fukuhara Takefumi,
Tateishi Jun,
Okamura Takashi,
Nakayama Chikashi,
Igarashi Masahiro
Publication year - 1980
Publication title -
psychiatry and clinical neurosciences
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.609
H-Index - 74
eISSN - 1440-1819
pISSN - 1323-1316
DOI - 10.1111/j.1440-1819.1980.tb01514.x
Subject(s) - adrenoleukodystrophy , pathological , pathology , poison control , medicine , anatomy , medical emergency , peroxisome , receptor
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 more 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.