Adrenoleukomyeloneuropathy presenting as a mental disorder
Turpin, J.C.; Gross, J.C.
L'Encephale 19(6): 609-613
1993
ISSN/ISBN: 0013-7006 PMID: 12404779 Document Number: 421955
Troubles of cognitive functions are generally observed in the X-linked adrenoleucodystrophy. This disease can be revealed at late onset (adrenomyeloneuropathy) or contemporary of the demyelinisation in central nervous system (adrenoleucodystrophy). Cerebral variants with only psychic involvement remain uncommon, the lesions are located in the temporo-parieto-occipital areas. The diagnosis occurs by biological investigations and/or the familial history before any defect of neurological signs. Therapeutics hopes in this storage disorder lay by the earlier diagnosis. In this way, we found interest to investigate the adult forms.