Article
Brain MRI and electrophysiologic abnormalities in preclinical and clinical adrenomyeloneuropathy.
Neurology - 1 Jan 1992
Aubourg P, Adamsbaum C, Lavallard-Rousseau M C, Lemaitre A, Boureau F, Mayer M, Kalifa G
Abstract excerpt
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...
Topics
- Addison Disease
- Adolescent
- Adrenoleukodystrophy
- Adult
- Brain
- Demyelinating Diseases
- Evoked Potentials
- Follow-Up Studies
- Genetic Linkage
- Humans
- Magnetic Resonance Imaging
