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Very-late-onset adrenoleukodystrophy

Possible precipitation of demyelination by cerebral contusion

Departments of Neurology (Drs. Weller, Liedtke, and Poremba) and Neuroradiology (Dr. Petersen), and the Institute of Brain Research (Dr. Opitz), University of Tubingen, Germany.

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.

Address correspondence and reprint requests to Dr. Michael Poremba, Department of Neurology, University of Tubingen, Hoppe-Seyler-Strasse 3, 7400 Tubingen, Germany.

Received March 19, 1991. Accepted for publication in final form July 22, 1991.

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