TY - JOUR
T1 - Abnormalities of somatosensory and motor evoked potentials in adrenomyeloneuropathy
T2 - Comparison with magnetic resonance imaging and clinical findings
AU - Restuccia, Domenico
AU - Di Lazzaro, Vincenzo
AU - Valeriani, Massimiliano
AU - Oliviero, Antonio
AU - Le Pera, Domenica
AU - Barba, Carmen
AU - Cappa, Marco
AU - Bertini, Enrico
AU - Tonali, Pietro
PY - 1997
Y1 - 1997
N2 - We studied 6 patients with adrenomyeloneuropathy (AMN) showing mild signs of central nervous system involvement. All patients underwent brain and spinal magnetic resonance imaging (MRI) and somatosensory (SEP) and motor (MEP) evoked potential study. Whereas SEPs and MEPs were abnormal in all patients, only 1 patient showed brain MRI abnormalities; spinal MRI showed hypotrophy without focal abnormalities in 4 of 6 patients. Median nerve SEPs, which were recorded with noncephalic reference montage, revealed delayed or absent scalp P14 far-field potential in all patients and abnormal spinal N13 in 2. Moreover, tibial nerve SEPs revealed abnormalities of the subcortical P30 response in all 4 patients in whom scalp-to-ear recording was employed. These findings strongly suggest that in the early stages of disease neurological dysfunction is localized in the spinal cord, where it is difficult to assess using MRI. However, SEPs and MEPs, which show a typical pattern of abnormality in these patients, could be useful in disclosing signs of long tract involvement and in monitoring treatment.
AB - We studied 6 patients with adrenomyeloneuropathy (AMN) showing mild signs of central nervous system involvement. All patients underwent brain and spinal magnetic resonance imaging (MRI) and somatosensory (SEP) and motor (MEP) evoked potential study. Whereas SEPs and MEPs were abnormal in all patients, only 1 patient showed brain MRI abnormalities; spinal MRI showed hypotrophy without focal abnormalities in 4 of 6 patients. Median nerve SEPs, which were recorded with noncephalic reference montage, revealed delayed or absent scalp P14 far-field potential in all patients and abnormal spinal N13 in 2. Moreover, tibial nerve SEPs revealed abnormalities of the subcortical P30 response in all 4 patients in whom scalp-to-ear recording was employed. These findings strongly suggest that in the early stages of disease neurological dysfunction is localized in the spinal cord, where it is difficult to assess using MRI. However, SEPs and MEPs, which show a typical pattern of abnormality in these patients, could be useful in disclosing signs of long tract involvement and in monitoring treatment.
KW - Adrenoleukodystrophy
KW - Adrenomyeloneuropathy
KW - Magnetic resonance imaging
KW - Motor evoked potentials
KW - Somatosensory evoked potentials
UR - http://www.scopus.com/inward/record.url?scp=0030867314&partnerID=8YFLogxK
UR - http://www.scopus.com/inward/citedby.url?scp=0030867314&partnerID=8YFLogxK
U2 - 10.1002/(SICI)1097-4598(199710)20:10<1249::AID-MUS7>3.0.CO;2-C
DO - 10.1002/(SICI)1097-4598(199710)20:10<1249::AID-MUS7>3.0.CO;2-C
M3 - Article
C2 - 9324081
AN - SCOPUS:0030867314
VL - 20
SP - 1249
EP - 1257
JO - Muscle and Nerve
JF - Muscle and Nerve
SN - 0148-639X
IS - 10
ER -