Ｍｉｔｏｃｈｏｎｄｒｉａｌ　ｍｙｏｐａｔｈｙ，ｅｎｃｅｐｈａｌｏｐａｔｈｙ，ｌａｃｔｉｃ　ａｃｉｄｏｓｉｓ，ａｎｄ　ｓｔｒｏｋｅ‐ｌｉｋｅ　ｅｐｉｓｏｄｅｓ（ＭＥＬＡＳ）を伴うミトコンドリア糖尿病に合併した難治性慢性偽性腸閉塞にネオスチグミンが奏効した１例

DOI Web Site 被引用文献2件 参考文献19件

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タイトル別名
  • A Case of Diabetes Mellitus with MELAS Complicated by Chronic Intestinal Pseudoobstruction Responding Dramatically to Anticholinesterase Inhibitor.
  • ショウレイ ホウコク Mitochondrial myopathy, encephalopathy, lactic acidosis, and stroke-like episodes (MELAS)オ トモナウ ミトコンドリア トウニョウビョウ ニ ガッペイ シタ ナンチセイ マンセイ ギセイ チョウ ヘイソク ニ ネオスチグミン ガ ソウコウ シタ 1レイ

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A 57-year-old woman with a 25-year history of diabetes reported nausea, severe emaciation and hearingdisturbance, and was admitted to our diabetes center. The diagnosis of mitochondrial myopathy, encephalo-pathy, lactic acidosis, and stroke-like episodes (MELAS) was based on severe muscle atrophy, progressive deafness, highly elevated lactate and pyruvate, and positive mitochondrial DNA 3243 point mutation. A high dose of coenzyme Q (150mg/day) was administered. On hospital day 37, she developed rapidly increasing abdominal fullness. Although abdominal X-ray showed severe accumulation of colonic gas, gastrointestinal and colonic examinations ruled out mechanical obstruction. Chronic intestinal pseudoobstruction (CIP) due to MELAS was suspected. Bethanechol chloride and erythromycin were started and cytochrome c added from the 40th hospital day, but did not improve clinical symptoms such as colonic gas accumulation. Anticholinesterase inhibitor (Neostigmine) was administered from hospital day 57th instead of bethanechol chloride and symptoms and gas accumulation markedly improved. CIP complicated by MELAS has been reported to be difficult to cure. Our case suggests that combined coenzyme Q and anticholinesterase inhibitor therapy are synergistically effective for CIP due to MELAS and diabetes mellitus.

収録刊行物

  • 糖尿病

    糖尿病 44 (10), 825-830, 2001

    一般社団法人 日本糖尿病学会

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