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Electrophysiological studies in Kearns-Shy syndrome Ikuo Hoshino 1 , Yoshihiko Yamamoto 1 , Norio Takeda 2 1Dept of Ophthalmol, Sch of Med, Chiba Univ 2Dept of Ophthalmol, Fac of Med, Toyama Med and Pharmaceutical Univ pp.593-596
Published Date 1991/4/15
DOI https://doi.org/10.11477/mf.1410900609
  • Abstract
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We studied electrophysiologically on two cases of the Kearns-Shy syndrome, in which some struc-tural abnormalities of mitochondria were shown.

A 46-year-old female presented with bilateral ptosis, total external ophthalmoplegia, neurosen-sory hearing loss and muscular weakness. She manifested the fundus feature of tapetoretinal degeneration and no hyper pigmentation. The sin-gle-flash electroretinograms (ERGs) were almost extinguished. The pattern visually evoked cortical potentials (PVECPs) were almost normal.

A 46-year-old male presented with bilateral ptosis, total external ophthalmoplegia, neurosen-sory hearing loss and elevated CSF protein level. Both fundi showed optic atrophy. The single-flash ERGs were almost normal. The peak latency of the P100 of the PVECP was delayed.

In both cases, “ragged red fibers” were observed in muscle biopsy specimens with modified Gomori trichrome stain.

Thus the electrophysiological studies may pro-vide us a way of evaluating the damaged system of visual function in the Kearns-Shy syndrome.


Copyright © 1991, Igaku-Shoin Ltd. All rights reserved.

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電子版ISSN 1882-1308 印刷版ISSN 0370-5579 医学書院

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