title: Adrenoleukodystrophy indicated by abnormal electro-encephalography.
abstract: An 8 year old boy with adrenoleukodystrophy and his asymptomatic brother, both with abnormal electro-encephalography as the initial sign of adrenoleukodystrophy, are reported. After suffering from aseptic meningitis caused by Echo virus 30, the first case was suspected of having a complication of meningitis because of persistent abnormal electro-encephalography (i.e. slow wave bursts in the occipital region). This patient was finally diagnosed as having adrenoleukodystrophy because of the increased level of very long chain saturated fatty acids, and typical occipital lesions demonstrated by magnetic resonance imaging and computed tomography. Dietary therapy was tried, but the course was progressive. The brother's electro-encephalography was also abnormal and so was diagnosed as having asymptomatic adrenoleukodystrophy because of an increased level of very long chain saturated fatty acids, although brain magnetic resonance imaging was normal. Abnormal electro-encephalography can therefore be an initial sign of adrenoleukodystrophy even if magnetic resonance imaging shows no abnormality in the brain.
authors.affiliation: Department of Pediatrics, Nantan General Hospital, Yagi, Japan.
authors.forename: N
authors.initials: N
authors.lastname: Wada
authors.forename: Y
authors.initials: Y
authors.lastname: Takeuchi
authors.forename: F
authors.initials: F
authors.lastname: Fujiwara
authors.forename: I
authors.initials: I
authors.lastname: Odani
authors.forename: T
authors.initials: T
authors.lastname: Sawada
mesh_terms: D000324:Adrenocorticotropic Hormone / Q000097:blood; D000326:Adrenoleukodystrophy / Q000097:blood / Q000175:diagnosis* / Q000235:genetics; D001921:Brain / Q000473:pathology; D002648:Child; D018450:Disease Progression; D004569:Electroencephalography; D006801:Humans; D006854:Hydrocortisone / Q000097:blood; D008279:Magnetic Resonance Imaging; D008297:Male
chemical_list: D000324:Adrenocorticotropic Hormone; D006854:Hydrocortisone