A case of primary biliary cirrhosis associated with mitochondrial encephalomyopathy

DOI PubMed 15 References
  • SATO Shin-ichiro
    Department of Internal Medicine, Hanamaki Onsen Hospital, Iwate Medical University
  • INABA Hirotsugu
    Department of Internal Medicine, Hanamaki Onsen Hospital, Iwate Medical University
  • OANA Shuhei
    Department of Internal Medicine, Hanamaki Onsen Hospital, Iwate Medical University
  • MIURA Masanori
    Department of Internal Medicine, Hanamaki Onsen Hospital, Iwate Medical University
  • KONDO Koryo
    Department of Internal Medicine, Hanamaki Onsen Hospital, Iwate Medical University
  • TAKIKAWA Yasuhiro
    First Department of Internal Medicine, Iwate Medical University School of Medicine
  • SUZUKI Kazuyuki
    First Department of Internal Medicine, Iwate Medical University School of Medicine
  • UESUGI Noriyuki
    Division of Pathology, Central Clinical Laboratory, Iwate Medical University School of Medicine
  • MASUDA Tomoyuki
    Department of Pathology, Iwate Medical University School of Medicine
  • KUKI Hiroyuki
    Department of Neurology, Hachinohe Red Cross Hospital

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  • ミトコンドリア脳筋症を合併した原発性胆汁性肝硬変の1例

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Abstract

症例は68歳男性．1996年より無症候性原発性胆汁性肝硬変の診断で通院中であった．2002年10月麻痺性腸閉塞の診断で入院，内科的治療で改善したが同時に四肢，体幹の筋萎縮と四肢の筋力低下を認めたため精査を行った．筋生検でragged-red fiberを認め，ミトコンドリア脳筋症の診断となった．原発性胆汁性肝硬変とミトコンドリア脳筋症の合併は過去に報告はなく極めてまれである．両者の合併に関連性があるかは不明であるが，抗ミトコンドリア抗体（anti-mitochondrial antibody；AMA）がミトコンドリア脳筋症の発症やその後の経過に影響を与えた可能性は否定できず，AMAの病因論的意義を考える上で興味深い症例と思われた．<br>

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