免疫吸着療法を主体とするアフェレシス治療が奏功したLambert-Eaton筋無力症候群の1例  [in Japanese] A Case Report of Lambert-Eaton Myasthenic Syndrome Improved by an Apheresis Therapy Mainly Composed of Immunoadsorption Plasmapheresis  [in Japanese]

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Abstract

乳癌・結核の既往のある72歳女性.4か月前からの下肢脱力感,2か月前からの易疲労感,1か月前からの上肢脱力感および眼瞼下垂のため当院神経内科に入院した.反復刺激筋電図にて50Hzの高頻度刺激でwaxing現象が認められ,抗VGCC抗体陽性でありLambert-Eaton筋無力症候群(LEMS)と診断された.また,胸部CT上,左下葉に結節影・リンパ節腫大を認め, pro-GRPも上昇しており肺小細胞癌を合併していると診断された.呼吸器内科に転科し化学療法(CDDP+CPT-11)を2クール施行したが,LEMSの増悪による呼吸筋低下のため20日目(入院第70病日)に呼吸状態が悪化し,意識障害(CO_2ナルコーシス)を来した. LEMSの増悪に対し,血漿交換を1回,免疫吸着療法を3回施行したところ,意識レベル・呼吸状態の改善がみられ,免疫吸着療法を主体とするアフェレシス治療が奏効したと考えられた.

We report a 72-year-old female patient with a history of breast cancer and tuberculosis. She was admitted to our hospital because of weakness of the lower limbs for four months, muscle fatigability for two months, weakness of the upper limbs and ptosis for a month. She had a diagnosis of Lambert-Eaton myasthenic syndrome (LEMS) based on the result of electromyography (EMG) showing waxing phenomenon with 50 Hz repetitive stimulation and having circulating anti-P/Q-type voltage-gated calcium channel (VGCC) antibodies. Computed tomography and a high level of pro-GRP revealed that she also had small-cell lung carcinoma. Although chemotherapy of CDDP+CPT-11 was administered, she suffered from CO_2 narcosis associated with LEMS. One plasma exchange and three times immunoadsorption plasmapheresis (IAPP) had improved her consciousness and respiratory status. Apheresis therapy mainly composed of IAPP was considered to be effective for Lambert-Eaton myasthenic syndrome.

Journal

  • 日本アフェレシス学会雑誌

    日本アフェレシス学会雑誌 31(3), 242-246, 2012

    日本アフェレシス学会

Codes

  • NII Article ID (NAID)
    110009543815
  • NII NACSIS-CAT ID (NCID)
    AA11604174
  • Text Lang
    JPN
  • Data Source
    NII-ELS  NDL-Digital 
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