title: Very-late-onset adrenoleukodystrophy: possible precipitation of demyelination by cerebral contusion.
abstract: We report the clinical history, neuroradiology, and autopsy findings in a patient with very late onset, at age 57, of adrenoleukodystrophy (ALD) presenting with dementia, hemianopia, and gait apraxia. Open brain biopsy, elevated plasma very-long-chain fatty acids, and autopsy confirmed the diagnosis of ALD. Demyelinative lesions were most extensive at the site of a recent cerebral contusion. CNS trauma may have precipitated or accelerated demyelination in this patient carrying the ALD gene.
authors.affiliation: Department of Neurology, University of Tübingen, Germany.
authors.forename: M
authors.initials: M
authors.lastname: Weller
authors.forename: W
authors.initials: W
authors.lastname: Liedtke
authors.forename: D
authors.initials: D
authors.lastname: Petersen
authors.forename: H
authors.initials: H
authors.lastname: Opitz
authors.forename: M
authors.initials: M
authors.lastname: Poremba
mesh_terms: D000326:Adrenoleukodystrophy / Q000000981:diagnostic imaging / Q000473:pathology / Q000503:physiopathology*; D000367:Age Factors; D001924:Brain Concussion / Q000150:complications*; D003711:Demyelinating Diseases / Q000209:etiology*; D005227:Fatty Acids / Q000097:blood; D006801:Humans; D008279:Magnetic Resonance Imaging; D008297:Male; D008875:Middle Aged; D014057:Tomography, X-Ray Computed
chemical_list: D005227:Fatty Acids