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Multimodal evoked potentials in progressive external ophthalmoplegia with mitochondrial myopathy
Author(s) -
Versino M.,
Piccolo G.,
Callieco R.,
Bergamaschi R.,
Banfi P.,
Azan G.,
Rizzuto R.,
Cosi V.
Publication year - 1991
Publication title -
acta neurologica scandinavica
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.967
H-Index - 95
eISSN - 1600-0404
pISSN - 0001-6314
DOI - 10.1111/j.1600-0404.1991.tb04917.x
Subject(s) - mitochondrial myopathy , external ophthalmoplegia , subclinical infection , medicine , brainstem , somatosensory evoked potential , central nervous system , internuclear ophthalmoplegia , myopathy , evoked potential , neuroscience , pathology , audiology , psychology , anesthesia , multiple sclerosis , biology , mitochondrial dna , biochemistry , psychiatry , gene
Multimodal evoked potentials were studied in 13 patients affected by progressive external ophthalmoplegia with histologically proven mitochondrial myopathy. Progressive external ophthalmoplegia occurred with craniosomatic spreading in all the patients and with a varying degree of nervous and/or other system involvement in most of them. In all but one of the subjects, at least one evoked potential modality was abnormal; 11 of them demonstrated an abnormal visual evoked potential, but this finding might have been influenced by concurrent retinal dysfunction. Abnormalities in brainstem auditory evoked potentials and/or somatosensory evoked potentials, revealing an impairment of central sensory pathways, were detected in 7 subjects, 5 of whom lacked clinical evidence of central nervous system involvement. Thus, evoked potentials represent an useful tool for the detection of subclinical central nervous system involvement in patients affected by progressive external ophthalmoplegia with mitochondrial myopathy.