Di Lazzaro V, Restuccia D, Servidei S, Valeriani M, Nardone R, Manfredi G, Silvestri G, Ricci E, Tonali P
Istituto di Neurologia, Università Cattolica, Rome, Italy.
Electroencephalogr Clin Neurophysiol. 1997 Jun;105(3):171-80. doi: 10.1016/s0924-980x(97)96671-6.
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.
对39例线粒体疾病患者进行了体感和运动诱发电位研究。16例患者（41%）有中枢神经系统受累的临床证据，12例（31%）有神经放射学证据。电生理异常的总体发生率为64%。在患有单纯肌病形式线粒体疾病的患者中，也有相当比例（33%）发现诱发电位异常，以及在一名MERRF突变的无症状携带者中也有异常。在各项检查中，49%的患者体感诱发电位异常，46%的患者运动诱发电位异常。结果表明，线粒体疾病患者中，有很高比例存在中枢神经系统受累的电生理证据，而且中枢神经系统电生理异常的阈值远低于临床和/或放射学表现的阈值。
