関節拘縮を主症状とし骨格筋にアミロイド沈着をみとめたALアミロイドーシスの68歳男性例  [in Japanese] A representative case of joint contracture as a main feature of AL amyloid deposits identified in the skeletal muscles  [in Japanese]

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Abstract

症例は68歳の男性である.8年前より糖尿病,慢性腎不全と診断され,4年前より腎不全,膝屈曲拘縮,下肢のしびれが進行した.IgA λ型M蛋白,下肢筋MRI選択的脂肪抑制T<sub>2</sub>強調画像で網状高信号病変,針筋電図で筋原性と神経原性変化の混在所見をみとめた.大腿四頭筋の生検で筋内の血管周囲にCongo red染色陽性かつ抗λ鎖抗体陽性の沈着物をみとめ,ALアミロイドーシスによるアミロイドミオパチーと診断した.アミロイドミオパチーでは関節拘縮をみとめることがあり,選択的脂肪抑制T<sub>2</sub>強調画像での網状高信号病変が診断に有用である.このような症例では,筋生検にてアミロイド沈着を証明できることが示された.

A 68-year-old man, with a history of type 2 diabetes mellitus and chronic kidney impairment, had been suffering from progressive knee joint contracture and dysesthesia of the lower extremities for 4 years. When he walked, his knees remained bent owing to contracture of the knee joints. There was no evidence of muscle pseudohypertrophy, intramuscular nodules, or muscle weakness. Clinical examination revealed IgA λ M-protein, reticular high-signal intensity lesions demonstrated by magnetic resonance T<sub>2</sub>-short TI IR(STIR) imaging of the lower extremity muscles, and a mixture of neurogenic and myogenic changes demonstrated by needle electromyography. A biopsy specimen from the vastus lateralis muscle identified Aλ amyloid deposits around the vessels, establishing a diagnosis of amyloid myopathy based on systemic AL amyloidosis. This case demonstrated that joint contracture and reticular lesions shown by magnetic resonance STIR imaging of the muscles can alert the physician to consider muscle biopsy to investigate deposition of amyloid in the skeletal muscles even in the absence of muscle pseudohypertrophy or weakness, both of which are characteristic of amyloid myopathy.

Journal

  • Rinsho Shinkeigaku

    Rinsho Shinkeigaku 54(11), 907-910, 2014

    Societas Neurologica Japonica

Codes

  • NII Article ID (NAID)
    130004706050
  • NII NACSIS-CAT ID (NCID)
    AN00253207
  • Text Lang
    JPN
  • ISSN
    0009-918X
  • NDL Article ID
    025922809
  • NDL Call No.
    Z19-298
  • Data Source
    NDL  J-STAGE 
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