A case of maternally inherited diabetes with deafness (MIDD) occurring at an advanced age

  • Takeuchi Jun
    Department of Medicine II, Hokkaido University Graduate School of Medicine
  • Tashiro Jun
    Department of Neurology, Hokkaido University Graduate School of Medicine
  • Nakagaki Osamu
    Department of Medicine II, Hokkaido University Graduate School of Medicine
  • Yoshida Masahiro
    Department of Medicine II, Hokkaido University Graduate School of Medicine
  • Kamoshima Hikaru
    Department of Medicine II, Hokkaido University Graduate School of Medicine
  • Oku Kenji
    Department of Medicine II, Hokkaido University Graduate School of Medicine
  • Yoshioka Narihito
    Department of Medicine II, Hokkaido University Graduate School of Medicine

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Other Title
  • 高齢発症のミトコンドリア糖尿病の1例
  • 症例報告 高齢発症のミトコンドリア糖尿病の1例
  • ショウレイ ホウコク コウレイ ハッショウ ノ ミトコンドリア トウニョウビョウ ノ 1レイ

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Abstract

We report an elderly patient with maternally inherited diabetes with deafness (MIDD). A 69-year-old woman was found to be diabetic for the first time when she visited her local medical doctor for the symptoms of a common cold. Her casual plasma glucose level was 311mg/dl and HbA1c was 8.3%.She had been aware of muscle atrophy of the lower extremities and hearing disturbance since age 66. As for her family history, her mother, older sister and younger brother were diabetic with hearing difficulty and all of them had died suddenly in their middle age. Her 45-year-old daughter was also diabetic with some difficulty in hearing. Therefore, we suspected both the patient and her daughter had MIDD, and found alterations in mitochondrial DNA3243A-G. MIDD is a condition that needs to be diagnosed accurately and treated at an early stage, since diabetic complications can progress rapidly and could cause myocardial complications and mitochondrial encephalomyopathy, lactic acidosis and stroke-like episodes (MELAS). According to a report of 115 cases of MIDD in Japan, MIDD had been diagnosed at the age of 32.8 on average and our case was strikingly old for the age of onset of the disease.<br>

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