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.
Di Lazzaro, V., Restuccia, D., Servidei, S., Valeriani, M., Nardone, R., Manfredi, G., et al. (1997). Functional involvement of central nervous system in mitochondrial disorders. ELECTROENCEPHALOGRAPHY AND CLINICAL NEUROPHYSIOLOGY. ELECTROMYOGRAPHY AND MOTOR CONTROL, 105(3), 171-180 [10.1016/s0924-980x(97)96671-6].
Functional involvement of central nervous system in mitochondrial disorders
Valeriani, M.;Silvestri, G.;Ricci, E.;
1997-06-01
Abstract
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.File | Dimensione | Formato | |
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