title: Abnormal evoked potentials of Kearns-Sayre syndrome.
abstract: We examined eight patients with Kearns-Sayre syndrome (KSS) to investigate a dysfunction in the central nervous system (CNS) using PTN-SEP, MN-SEP and BAEP. We found a significant increase in the P37 latency of PTN-SEPs and the central conduction time of MN-SEPs, and interpeak latencies of BAEPs. Delayed SEPs or BAEPs were caused by a dysfunction of the somatosensory or lateral lemniscus pathways which could be related to mitochondrial abnormalities in the CNS. Long-term therapy with CoQ showed an improvement of the latencies of SEPs after about half a year from the start of CoQ therapy in our patients. The improvement of the latencies of SEPs were preserved during CoQ therapy. It could be demonstrated that CoQ therapy had the beneficial effects on abnormal functions of the CNS in patients with KSS.
authors.affiliation: Department of Neurology, Kinki University School of Medicine, Japan.
authors.forename: Y
authors.initials: Y
authors.lastname: Nakamura
authors.forename: M
authors.initials: M
authors.lastname: Takahashi
authors.forename: M
authors.initials: M
authors.lastname: Kitaguchi
authors.forename: S
authors.initials: S
authors.lastname: Yorifuji
authors.forename: Y
authors.initials: Y
authors.lastname: Nishikawa
authors.forename: H
authors.initials: H
authors.lastname: Imaoka
authors.forename: S
authors.initials: S
authors.lastname: Tarui
mesh_terms: D000328:Adult; D003067:Coenzymes; D016057:Evoked Potentials, Auditory, Brain Stem / Q000187:drug effects / Q000502:physiology*; D005073:Evoked Potentials, Somatosensory / Q000187:drug effects / Q000502:physiology*; D005260:Female; D006801:Humans; D007625:Kearns-Sayre Syndrome / Q000188:drug therapy / Q000503:physiopathology*; D008297:Male; D008475:Median Nerve / Q000503:physiopathology; D008875:Middle Aged; D009431:Neural Conduction / Q000187:drug effects; D009434:Neural Pathways / Q000503:physiopathology; D011930:Reaction Time / Q000187:drug effects; D013003:Somatosensory Cortex / Q000503:physiopathology; D013116:Spinal Cord / Q000503:physiopathology; D013979:Tibial Nerve / Q000503:physiopathology; D014451:Ubiquinone / Q000008:administration & dosage / Q000031:analogs & derivatives / Q000627:therapeutic use
chemical_list: D003067:Coenzymes; D014451:Ubiquinone; C024989:coenzyme Q10