TY - JOUR
T1 - Functional involvement of central nervous system in mitochondrial disorders
AU - Di Lazzaro, V.
AU - Restuccia, D.
AU - Servidei, S.
AU - Valeriani, M.
AU - Nardone, R.
AU - Manfredi, G.
AU - Silvestri, G.
AU - Ricci, E.
AU - Tonali, P.
PY - 1997/6
Y1 - 1997/6
N2 - Thirty-nine patients with mitochondrial diseases were studied with somatosensory and motor evoked potentials. Sixteen patients (41%) had clinical and 12 (31%) had neuroradiological evidence of central nervous system involvement. The overall incidence of electrophysiological abnormalities was 64%. Abnormal evoked potentials were also found in a significant percentage (33%) of patients with pure myopathic forms of mitochondrial diseases and in an asymptomatic carrier of MERRF mutation. Of the individual tests, somatosensory evoked potentials were abnormal in 49% of the patients and motor evoked potentials were abnormal in 46% of the patients. The outcome is that electrophysiological evidence of central nervous system involvement is present in a high percentage of patients with mitochondrial disorders, and that the threshold for central nervous system electrophysiological abnormalities is well below that for clinical and/or radiological manifestations.
AB - Thirty-nine patients with mitochondrial diseases were studied with somatosensory and motor evoked potentials. Sixteen patients (41%) had clinical and 12 (31%) had neuroradiological evidence of central nervous system involvement. The overall incidence of electrophysiological abnormalities was 64%. Abnormal evoked potentials were also found in a significant percentage (33%) of patients with pure myopathic forms of mitochondrial diseases and in an asymptomatic carrier of MERRF mutation. Of the individual tests, somatosensory evoked potentials were abnormal in 49% of the patients and motor evoked potentials were abnormal in 46% of the patients. The outcome is that electrophysiological evidence of central nervous system involvement is present in a high percentage of patients with mitochondrial disorders, and that the threshold for central nervous system electrophysiological abnormalities is well below that for clinical and/or radiological manifestations.
KW - Mitochondrial myopathies
KW - Motor evoked potentials
KW - Somatosensory evoked potentials
UR - http://www.scopus.com/inward/record.url?scp=0030993609&partnerID=8YFLogxK
UR - http://www.scopus.com/inward/citedby.url?scp=0030993609&partnerID=8YFLogxK
U2 - 10.1016/S0924-980X(97)96671-6
DO - 10.1016/S0924-980X(97)96671-6
M3 - Article
C2 - 9216485
AN - SCOPUS:0030993609
VL - 105
SP - 171
EP - 180
JO - Electroencephalography and Clinical Neurophysiology - Electromyography and Motor Control
JF - Electroencephalography and Clinical Neurophysiology - Electromyography and Motor Control
SN - 0924-980X
IS - 3
ER -