Functional involvement of central nervous system in mitochondrial disorders

V. Di Lazzaro; D. Restuccia; S. Servidei; M. Valeriani; R. Nardone; G. Manfredi; G. Silvestri; E. Ricci; P. Tonali

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

Functional involvement of central nervous system in mitochondrial disorders

V. Di Lazzaro, D. Restuccia, S. Servidei, M. Valeriani, R. Nardone, G. Manfredi, G. Silvestri, E. Ricci, P. Tonali

Ospedale Pediatrico Bambino Gesu

Research output: Contribution to journal › Article

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.

Original language	English
Pages (from-to)	171-180
Number of pages	10
Journal	Electroencephalography and Clinical Neurophysiology - Electromyography and Motor Control
Volume	105
Issue number	3
DOIs	https://doi.org/10.1016/S0924-980X(97)96671-6
Publication status	Published - Jun 1997

Keywords

Mitochondrial myopathies
Motor evoked potentials
Somatosensory evoked potentials

ASJC Scopus subject areas

Clinical Neurology
Neuroscience(all)

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Di Lazzaro, V., Restuccia, D., Servidei, S., Valeriani, M., Nardone, R., Manfredi, G., Silvestri, G., Ricci, E., & Tonali, P. (1997). Functional involvement of central nervous system in mitochondrial disorders. Electroencephalography and Clinical Neurophysiology - Electromyography and Motor Control, 105(3), 171-180. https://doi.org/10.1016/S0924-980X(97)96671-6

Functional involvement of central nervous system in mitochondrial disorders. / Di Lazzaro, V.; Restuccia, D.; Servidei, S.; Valeriani, M.; Nardone, R.; Manfredi, G.; Silvestri, G.; Ricci, E.; Tonali, P.

In: Electroencephalography and Clinical Neurophysiology - Electromyography and Motor Control, Vol. 105, No. 3, 06.1997, p. 171-180.

Research output: Contribution to journal › Article

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AU - Nardone, R.

AU - Manfredi, G.

AU - Silvestri, G.

AU - Ricci, E.

AU - Tonali, P.

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