ABNORMALITIES OF SOMATOSENSORY AND MOTOR EVOKED-POTENTIALS IN ADRENOMYELONEUROPATHY - COMPARISON WITH MAGNETIC-RESONANCE-IMAGING AND CLINICAL FINDINGS

We studied 6 patients with adrenomyeloneuropathy (AMN) showing mild si gns of central nervous system involvement. All patients underwent brai n and spinal magnetic resonance imaging (MRI) and somatosensory (SEP) and motor (MEP) evoked potential study. Whereas SEPs and MEPs were abn ormal in all patients, only 1 patient showed brain MRI abnormalities; spinal MRI showed hypotrophy without focal abnormalities in 4 of 6 pat ients. Median nerve SEPs, which were recorded with noncephalic referen ce montage, revealed delayed or absent scalp P14 tar-field potential i n all patients and abnormal spinal N13 in 2. Moreover, tibial nerve SE Ps revealed abnormalities of the subcortical P30 response in all 4 pat ients in whom scalp-to-ear recording was employed. These findings stro ngly suggest that in the early stages of disease neurological dysfunct ion is localized in the spinal cord, where it is difficult to assess u sing MRI. However, SEPs and MEPs, which show a typical pattern of abno rmality in these patients, could be useful in disclosing signs of long tract involvement and in monitoring treatment. (C) 1997 John Wiley & Sons, Inc.