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NEUROLOGY 1992;42:85
© 1992 American Academy of Neurology

Brain MRI and electrophysiologic abnormalities in preclinical and clinical adrenomyeloneuropathy

P. Aubourg, MD, C. Adamsbaum, MD, M. C. Lavallard-Rousseau, MD, A. Lemaitre, MD, F. Boureau, MD, M. Mayer, MD and G. Kalifa, MD

From Unité INSERM U342 (Dr. Aubourg) and the Departments of Pediatric Neurology (Drs. Aubourg, Lemaitre, and Mayer) and Pediatric Radiology (Drs. Adamsbaum and Kalifa), HBpital Saint-Vincent de Paul, Paris; and the Department of Neurophysiology (Drs. Lavallard-Rousseau and Boureau), Hôpital SaintAntoine, Paris, France.

We describe the neurophysiologic abnormalities in 17 patients with clinical adrenomyeloneuropathy (AMN), an adult phenotype of X-linked adrenoleukodystrophy (ALD). These abnormalities also allowed the early recognition of the AMN phenotype in three asymptomatic boys with biochemically proven ALD. In 14/16 adult AMN patients, there were cerebral white matter abnormalities on MRI which were severe and comparable with cerebral ALD in four of the patients.

Address correspondence and reprint requests to Dr. P. Aubourg, Unité INSERM U342, Hôpital Saint-Vincent de Paul, 82 avenue Denfert Rochereau, 75014 Paris, France.

Received March 28, 1991. Accepted for publication in final form June 11, 1991.




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