Very-late-onset adrenoleukodystrophy: possible precipitation of demyelination by cerebral contusion.
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.
Weller
M
M
Department of Neurology, University of Tübingen, Germany.
Liedtke
W
W
Petersen
D
D
Opitz
H
H
Poremba
M
M
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
D005227:Fatty Acids