Functional involvement of central nervous system in mitochondrial disorders

被引：9

作者：

DiLazzaro, V ^{[1
]}

Restuccia, D ^{[1
]}

Servidei, S ^{[1
]}

Valeriani, M ^{[1
]}

Nardone, R ^{[1
]}

Manfredi, G ^{[1
]}

Silvestri, G ^{[1
]}

Ricci, E ^{[1
]}

Tonali, P ^{[1
]}

机构：

[1] IRCCS, SAN GIOVANNI ROTONDO, ITALY

来源：

ELECTROMYOGRAPHY AND MOTOR CONTROL-ELECTROENCEPHALOGRAPHY AND CLINICAL NEUROPHYSIOLOGY | 1997年 / 105卷 / 03期

关键词：

mitochondrial myopathies; motor evoked potentials; somatosensory evoked potentials;

D O I：

10.1016/S0924-980X(97)96671-6

中图分类号：

R318 [生物医学工程];

学科分类号：

0831 ;

摘要：

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. (C) 1997 Elsevier Science Ireland Ltd.

引用

页码：171 / 180

页数：10

共 27 条

[1]

DAY BL, 1990, J PHYSL, V430, pP617

[2] SPINAL AND FAR-FIELD COMPONENTS OF HUMAN SOMATOSENSORY EVOKED-POTENTIALS TO POSTERIOR TIBIAL NERVE-STIMULATION ANALYZED WITH ESOPHAGEAL DERIVATIONS AND NON-CEPHALIC REFERENCE RECORDING [J].