[Adrenoleukomyeloneuropathy presenting as a mental disorder].
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.
Turpin
J C
JC
Service de Neurologie, C.H.U. de Reims, rue Cognacq-Jay, 51100 Reims.
Gross
J C
JC
D000326:Adrenoleukodystrophy / Q000150:complications* / Q000175:diagnosis* / Q000235:genetics; D000328:Adult; D001284:Atrophy / Q000473:pathology; D001921:Brain / Q000473:pathology; D003937:Diagnosis, Differential; D006801:Humans; D008279:Magnetic Resonance Imaging; D008297:Male; D010375:Pedigree; D011569:Psychiatric Status Rating Scales; D012559:Schizophrenia / Q000175:diagnosis* / Q000209:etiology*; D012720:Severity of Illness Index