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肌无力通常表现为晨轻晚重，波动性明显。
|
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"entity": "波动性明显",
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2/3病例累及眼外肌，常为早期症状，10%长期局限于眼肌，颜面肌、咽喉肌、躯干肌和肢体肌均可受累。
|
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"entity": "咽喉肌",
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"entity": "躯干肌",
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"entity": "肢体肌",
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【流行病学】国外流行病学调查显示MG年发病率为7.4/10万。
|
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但近些年我国文献报道，患者发病年龄同期以儿童期多见，占MG56.4%，且发病年龄提前，多在1～5岁发病。
|
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我国尚无流行病学研究报道，但从国内多个成组病例资料以及我院的资料显示，儿童MG小年龄患病比例较高。
|
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女性患者所生新生儿，其中约10%经过胎盘转运获得烟碱型乙酰胆碱受体抗体（nAChRab），可暂时出现肌无力症状。
|
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【病因与发病机制】20世纪70年代由于烟碱型乙酰胆碱受体（nAChR）能够从电鱼放电器官中得到并纯化，可成功地产生实验性MG的模型，以及同位素标记的蛇毒α-神经毒素放射免疫分析的应用，MG的发病机制研究已经取得突破性的进展：MG其发病机制与遗传因素、致病性自身抗体、细胞因子、补体参与及胸腺肌细胞等复杂因素有关。
|
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"entity": "细胞因子",
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"entity": "补体",
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"entity": "胸腺肌细胞",
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（一）重症肌无力是横纹肌突触后膜nAChR自身免疫性疾病神经肌肉接头是通过接受乙酰胆碱（ACh）及烟碱等兴奋性递质传递与肌膜受体结合，导致离子通道开放，Na+</sup>内流，肌膜去极化，产生终板电位，肌丝滑行，因而引起肌肉收缩。
|
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"entity": "神经肌肉接头",
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"entity": "烟碱",
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"entity": "兴奋性递质",
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"entity": "肌膜受体",
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"entity": "离子通道",
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"entity": "Na+",
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"id": 10,
"entity": "肌膜",
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"id": 11,
"entity": "肌丝",
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"entity": "肌肉收缩",
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] |
已知nChR是造成MG自体免疫应答高度特异性的抗原。
|
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nAChR位于神经肌肉接头部的突触后膜中。
|
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实验证明MG患者胸腺上皮细胞内含肌原纤维骨骼肌存在共同抗原（nAChR）。
|
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该抗原致敏T细胞，产生抗nAChR的抗体（nAChRab）。
|
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该抗体对骨骼肌nAChR产生交差免疫应答，使受体被阻滞；并加速AChR的降解，通过激活补体，使肌膜受到损害。
|
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电镜检查显示突触后膜IgG和C3沉积辣根酶标记蛇毒神经毒素电镜检测运动终板超微结构显示：MG病理损害的特征是骨骼肌突触后膜皱襞表面面积减少nAChR活性降低肌无力症状。
|
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"entity": "骨骼肌",
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"entity": "骨骼肌突触后膜皱襞表面面积减少",
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"id": 9,
"entity": "nAChR",
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"entity": "nAChR活性降低",
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（二）重症肌无力是T细胞依赖的自身免疫疾病体液免疫大量研究资料阐明nAChR作为MG的靶子遭到损害，是由nAChRab介导的；而nAChRab对nAChR免疫应答是T细胞依赖性的。
|
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T细胞在MG自身免疫应答中起着关键作用。
|
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nAChRab的产生必须有nAChR特异性T细胞的参与。
|
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nAChR特异CD4+</sup>T细胞先通过其受体（TCR）对nAChR特异性位点的识别，然后由T辅助细胞（Th）将nAChR主要免疫原区特异性抗体提供给B细胞，促使B细胞分泌高致病性的nAChRab。
|
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Th细胞通过分泌细胞因子来实现对nAChRab分泌的调节。
|
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（三）遗传基因和病毒感染众所周知，重症肌无力是自身免疫应答异常，但启动自身免疫的病因尚未完全弄清。
|
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目前认为MG发病与人类白血病抗原（HLA）有关，其相关性与人种及地域有关，且存在性别差异。
|
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HLA-Ⅱ类抗原（包括D区的DP、DQ及DR等基因产物）在发生自体免疫过程中起重要作用。
|
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采用PCR-RFLP技术检测发现我国非胸腺瘤MG与HLADQA1*</sup>0301基因显著相关。
|
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此外还发现与DQB1*</sup>0303及DPD1*</sup>1910基因相关显著，说明MG发病与多基因遗传有关。
|
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MG的发病除了与遗传基因有关外，还包括外在环境影响，如本病常因病毒感染而诱发或使病情加重。
|
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胸腺为免疫中枢。
|
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不论是胸腺淋巴细胞（特别是T细胞），还是上皮细胞（特别是肌样细胞，含有nAChR特异性抗原），遭到免疫攻击，打破免疫耐受性，引起针对nAChR的自身免疫应答，因此使MG发病。
|
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{
"id": 0,
"entity": "胸腺淋巴细胞",
"start_offset": 3,
"end_offset": 9,
"label": "bod"
},
{
"id": 1,
"entity": "T细胞",
"start_offset": 13,
"end_offset": 16,
"label": "bod"
},
{
"id": 2,
"entity": "上皮细胞",
"start_offset": 20,
"end_offset": 24,
"label": "bod"
},
{
"id": 3,
"entity": "肌样细胞",
"start_offset": 28,
"end_offset": 32,
"label": "bod"
},
{
"id": 4,
"entity": "nAChR特异性抗原",
"start_offset": 35,
"end_offset": 45,
"label": "bod"
},
{
"id": 5,
"entity": "nAChR",
"start_offset": 66,
"end_offset": 71,
"label": "mic"
},
{
"id": 6,
"entity": "MG",
"start_offset": 82,
"end_offset": 84,
"label": "dis"
}
] |
（一）新生儿一过性重症肌无力（transientneonatalmyastheniagravis）仅见于母亲患MG所生的新生儿。
|
[
{
"id": 0,
"entity": "新生儿一过性重症肌无力",
"start_offset": 3,
"end_offset": 14,
"label": "dis"
},
{
"id": 1,
"entity": "transientneonatalmyastheniagravis",
"start_offset": 15,
"end_offset": 48,
"label": "dis"
},
{
"id": 2,
"entity": "MG",
"start_offset": 55,
"end_offset": 57,
"label": "dis"
}
] |
患儿出生后数小时～3天内出现肌无力，表现哭声低弱，吞咽及呼吸困难，患儿血中nAChR-Ab可增高呼吸衰竭。
|
[
{
"id": 0,
"entity": "肌无力",
"start_offset": 14,
"end_offset": 17,
"label": "dis"
},
{
"id": 1,
"entity": "表现哭声低弱",
"start_offset": 18,
"end_offset": 24,
"label": "sym"
},
{
"id": 2,
"entity": "吞咽及呼吸困难",
"start_offset": 25,
"end_offset": 32,
"label": "sym"
},
{
"id": 3,
"entity": "血",
"start_offset": 35,
"end_offset": 36,
"label": "bod"
},
{
"id": 4,
"entity": "nAChR-Ab",
"start_offset": 37,
"end_offset": 45,
"label": "bod"
},
{
"id": 5,
"entity": "患儿血中nAChR-Ab可增高",
"start_offset": 33,
"end_offset": 48,
"label": "sym"
},
{
"id": 6,
"entity": "呼吸衰竭",
"start_offset": 48,
"end_offset": 52,
"label": "dis"
}
] |
（二）先天性肌无力综合征（congenitalmyastheniasyndrome）出生后以对称、持续存在、不完全眼外肌无力为特点，血清中无nAChR-Ab常染色体遗传有关，同胞中可有此病，但其母亲未患MG。
|
[
{
"id": 0,
"entity": "先天性肌无力综合征",
"start_offset": 3,
"end_offset": 12,
"label": "dis"
},
{
"id": 1,
"entity": "congenitalmyastheniasyndrome",
"start_offset": 13,
"end_offset": 41,
"label": "dis"
},
{
"id": 2,
"entity": "不完全眼外肌无力",
"start_offset": 54,
"end_offset": 62,
"label": "dis"
},
{
"id": 3,
"entity": "血清",
"start_offset": 66,
"end_offset": 68,
"label": "bod"
},
{
"id": 4,
"entity": "nAChR-Ab",
"start_offset": 70,
"end_offset": 78,
"label": "bod"
},
{
"id": 5,
"entity": "血清中无nAChR-Ab",
"start_offset": 66,
"end_offset": 78,
"label": "sym"
},
{
"id": 6,
"entity": "常染色体",
"start_offset": 78,
"end_offset": 82,
"label": "bod"
},
{
"id": 7,
"entity": "MG",
"start_offset": 101,
"end_offset": 103,
"label": "dis"
}
] |
（三）少年时重症肌无力（juvenileneonatalmyastheniagravis）为后天获得性肌无力，可以查到血清中nAChR-Ab。
|
[
{
"id": 0,
"entity": "少年时重症肌无力",
"start_offset": 3,
"end_offset": 11,
"label": "dis"
},
{
"id": 1,
"entity": "juvenileneonatalmyastheniagravis",
"start_offset": 12,
"end_offset": 44,
"label": "dis"
},
{
"id": 2,
"entity": "后天获得性肌无力",
"start_offset": 46,
"end_offset": 54,
"label": "dis"
},
{
"id": 3,
"entity": "血清",
"start_offset": 59,
"end_offset": 61,
"label": "bod"
},
{
"id": 4,
"entity": "nAChR",
"start_offset": 62,
"end_offset": 67,
"label": "mic"
}
] |
国外病例大多在10岁以后发病，以全身型为主，而国内资料与香港及日本报道发病多在幼儿时期（2～3岁），眼肌型为主。
|
[
{
"id": 0,
"entity": "眼肌型",
"start_offset": 50,
"end_offset": 53,
"label": "dis"
}
] |
此为儿童MG最常见的类型，现重点叙述如下。
|
[
{
"id": 0,
"entity": "儿童MG",
"start_offset": 2,
"end_offset": 6,
"label": "dis"
}
] |
肌无力通常晨轻晚重，亦可多变，后期可处于不全瘫痪状态。
|
[
{
"id": 0,
"entity": "肌无力",
"start_offset": 0,
"end_offset": 3,
"label": "dis"
},
{
"id": 1,
"entity": "晨轻晚重",
"start_offset": 5,
"end_offset": 9,
"label": "sym"
},
{
"id": 2,
"entity": "后期可处于不全瘫痪状态",
"start_offset": 15,
"end_offset": 26,
"label": "sym"
}
] |
眼外肌最常受累亦可局限于眼肌睁眼无力上眼睑下垂眼球运动受限出现斜视和复视，甚或眼球固定不动眼内肌一般不受影响瞳孔反射多正常眼肌型重症无力。
|
[
{
"id": 0,
"entity": "眼外肌",
"start_offset": 0,
"end_offset": 3,
"label": "bod"
},
{
"id": 1,
"entity": "眼外肌最常受累",
"start_offset": 0,
"end_offset": 7,
"label": "sym"
},
{
"id": 2,
"entity": "眼肌",
"start_offset": 12,
"end_offset": 14,
"label": "bod"
},
{
"id": 3,
"entity": "亦可局限于眼肌",
"start_offset": 7,
"end_offset": 14,
"label": "sym"
},
{
"id": 4,
"entity": "眼",
"start_offset": 15,
"end_offset": 16,
"label": "bod"
},
{
"id": 5,
"entity": "睁眼无力",
"start_offset": 14,
"end_offset": 18,
"label": "sym"
},
{
"id": 6,
"entity": "上眼睑",
"start_offset": 18,
"end_offset": 21,
"label": "bod"
},
{
"id": 7,
"entity": "上眼睑下垂",
"start_offset": 18,
"end_offset": 23,
"label": "sym"
},
{
"id": 8,
"entity": "眼球",
"start_offset": 23,
"end_offset": 25,
"label": "bod"
},
{
"id": 9,
"entity": "眼球运动受限",
"start_offset": 23,
"end_offset": 29,
"label": "sym"
},
{
"id": 10,
"entity": "出现斜视和复视",
"start_offset": 29,
"end_offset": 36,
"label": "sym"
},
{
"id": 11,
"entity": "眼球",
"start_offset": 39,
"end_offset": 41,
"label": "bod"
},
{
"id": 12,
"entity": "甚或眼球固定不动",
"start_offset": 37,
"end_offset": 45,
"label": "sym"
},
{
"id": 13,
"entity": "眼内肌",
"start_offset": 45,
"end_offset": 48,
"label": "bod"
},
{
"id": 14,
"entity": "眼内肌一般不受影响",
"start_offset": 45,
"end_offset": 54,
"label": "sym"
},
{
"id": 15,
"entity": "瞳孔",
"start_offset": 54,
"end_offset": 56,
"label": "bod"
},
{
"id": 16,
"entity": "瞳孔反射多正常",
"start_offset": 54,
"end_offset": 61,
"label": "sym"
},
{
"id": 17,
"entity": "眼肌型重症无力",
"start_offset": 61,
"end_offset": 68,
"label": "dis"
}
] |
面肌、舌肌、咀嚼肌及咽喉肌亦易受累闭眼不全额纹及鼻唇沟变浅咀嚼无力，吞咽困难，舌运动不自如无肌束颤动。
|
[
{
"id": 0,
"entity": "面肌",
"start_offset": 0,
"end_offset": 2,
"label": "bod"
},
{
"id": 1,
"entity": "舌肌",
"start_offset": 3,
"end_offset": 5,
"label": "bod"
},
{
"id": 2,
"entity": "咀嚼肌",
"start_offset": 6,
"end_offset": 9,
"label": "bod"
},
{
"id": 3,
"entity": "咽喉肌",
"start_offset": 10,
"end_offset": 13,
"label": "bod"
},
{
"id": 4,
"entity": "面肌、舌肌、咀嚼肌及咽喉肌亦易受累",
"start_offset": 0,
"end_offset": 17,
"label": "sym"
},
{
"id": 5,
"entity": "眼",
"start_offset": 18,
"end_offset": 19,
"label": "bod"
},
{
"id": 6,
"entity": "闭眼不全",
"start_offset": 17,
"end_offset": 21,
"label": "sym"
},
{
"id": 7,
"entity": "额纹",
"start_offset": 21,
"end_offset": 23,
"label": "bod"
},
{
"id": 8,
"entity": "鼻唇",
"start_offset": 24,
"end_offset": 26,
"label": "bod"
},
{
"id": 9,
"entity": "额纹及鼻唇沟变浅",
"start_offset": 21,
"end_offset": 29,
"label": "sym"
},
{
"id": 10,
"entity": "咀嚼无力",
"start_offset": 29,
"end_offset": 33,
"label": "sym"
},
{
"id": 11,
"entity": "吞咽困难",
"start_offset": 34,
"end_offset": 38,
"label": "sym"
},
{
"id": 12,
"entity": "舌",
"start_offset": 39,
"end_offset": 40,
"label": "bod"
},
{
"id": 13,
"entity": "舌运动不自如",
"start_offset": 39,
"end_offset": 45,
"label": "sym"
},
{
"id": 14,
"entity": "无肌束颤动",
"start_offset": 45,
"end_offset": 50,
"label": "sym"
}
] |
软腭肌无力发音呈鼻音谈话片刻后音调低沉或声嘶。
|
[
{
"id": 0,
"entity": "软腭",
"start_offset": 0,
"end_offset": 2,
"label": "bod"
},
{
"id": 1,
"entity": "肌无力",
"start_offset": 2,
"end_offset": 5,
"label": "dis"
},
{
"id": 2,
"entity": "软腭肌无力",
"start_offset": 0,
"end_offset": 5,
"label": "sym"
},
{
"id": 3,
"entity": "鼻",
"start_offset": 8,
"end_offset": 9,
"label": "bod"
},
{
"id": 4,
"entity": "发音呈鼻音",
"start_offset": 5,
"end_offset": 10,
"label": "sym"
},
{
"id": 5,
"entity": "谈话片刻后音调低沉或声嘶",
"start_offset": 10,
"end_offset": 22,
"label": "sym"
}
] |
称为延髓型（或球型）重症肌无力。
|
[
{
"id": 0,
"entity": "延髓型（或球型）重症肌无力",
"start_offset": 2,
"end_offset": 15,
"label": "dis"
}
] |
颈肌、躯干及四肢肌也可患病，尤其以肢体近端无力明显抬头困难手托头胸闷气短洗脸及穿衣乏累，行走困难，不能久行。
|
[
{
"id": 0,
"entity": "颈肌",
"start_offset": 0,
"end_offset": 2,
"label": "bod"
},
{
"id": 1,
"entity": "躯干",
"start_offset": 3,
"end_offset": 5,
"label": "bod"
},
{
"id": 2,
"entity": "四肢肌",
"start_offset": 6,
"end_offset": 9,
"label": "bod"
},
{
"id": 3,
"entity": "肢体",
"start_offset": 17,
"end_offset": 19,
"label": "bod"
},
{
"id": 4,
"entity": "肢体近端无力明显",
"start_offset": 17,
"end_offset": 25,
"label": "sym"
},
{
"id": 5,
"entity": "头",
"start_offset": 26,
"end_offset": 27,
"label": "bod"
},
{
"id": 6,
"entity": "抬头困难",
"start_offset": 25,
"end_offset": 29,
"label": "sym"
},
{
"id": 7,
"entity": "手",
"start_offset": 29,
"end_offset": 30,
"label": "bod"
},
{
"id": 8,
"entity": "头",
"start_offset": 31,
"end_offset": 32,
"label": "bod"
},
{
"id": 9,
"entity": "手托头",
"start_offset": 29,
"end_offset": 32,
"label": "sym"
},
{
"id": 10,
"entity": "胸",
"start_offset": 32,
"end_offset": 33,
"label": "bod"
},
{
"id": 11,
"entity": "胸闷气短",
"start_offset": 32,
"end_offset": 36,
"label": "sym"
},
{
"id": 12,
"entity": "洗脸及穿衣乏累",
"start_offset": 36,
"end_offset": 43,
"label": "sym"
},
{
"id": 13,
"entity": "行走困难",
"start_offset": 44,
"end_offset": 48,
"label": "sym"
},
{
"id": 14,
"entity": "不能久行",
"start_offset": 49,
"end_offset": 53,
"label": "sym"
}
] |
有的只表现两下肢无力腱反射存在无感觉障碍。
|
[
{
"id": 0,
"entity": "下肢",
"start_offset": 6,
"end_offset": 8,
"label": "bod"
},
{
"id": 1,
"entity": "两下肢无力",
"start_offset": 5,
"end_offset": 10,
"label": "sym"
},
{
"id": 2,
"entity": "腱",
"start_offset": 10,
"end_offset": 11,
"label": "bod"
},
{
"id": 3,
"entity": "腱反射存在",
"start_offset": 10,
"end_offset": 15,
"label": "sym"
},
{
"id": 4,
"entity": "无感觉障碍",
"start_offset": 15,
"end_offset": 20,
"label": "sym"
}
] |
称全身型重症肌无力。
|
[
{
"id": 0,
"entity": "全身型重症肌无力",
"start_offset": 1,
"end_offset": 9,
"label": "dis"
}
] |
本病主要累及骨骼肌心肌损害MG患者尸检25%～50%有心肌损害重症肌无力伴有其他疾病，如胸腺瘤，其次为甲状腺功能亢进，并少数伴类风湿关节炎、多发性肌炎、红斑狼疮以及自身溶血性贫血等。
|
[
{
"id": 0,
"entity": "骨骼肌",
"start_offset": 6,
"end_offset": 9,
"label": "bod"
},
{
"id": 1,
"entity": "累及骨骼肌",
"start_offset": 4,
"end_offset": 9,
"label": "sym"
},
{
"id": 2,
"entity": "心肌",
"start_offset": 9,
"end_offset": 11,
"label": "bod"
},
{
"id": 3,
"entity": "心肌损害",
"start_offset": 9,
"end_offset": 13,
"label": "sym"
},
{
"id": 4,
"entity": "MG",
"start_offset": 13,
"end_offset": 15,
"label": "dis"
},
{
"id": 5,
"entity": "心肌",
"start_offset": 27,
"end_offset": 29,
"label": "bod"
},
{
"id": 6,
"entity": "心肌损害",
"start_offset": 27,
"end_offset": 31,
"label": "sym"
},
{
"id": 7,
"entity": "重症肌无力",
"start_offset": 31,
"end_offset": 36,
"label": "dis"
},
{
"id": 8,
"entity": "胸腺瘤",
"start_offset": 44,
"end_offset": 47,
"label": "dis"
},
{
"id": 9,
"entity": "甲状腺功能亢进",
"start_offset": 51,
"end_offset": 58,
"label": "dis"
},
{
"id": 10,
"entity": "类风湿关节炎",
"start_offset": 63,
"end_offset": 69,
"label": "dis"
},
{
"id": 11,
"entity": "多发性肌炎",
"start_offset": 70,
"end_offset": 75,
"label": "dis"
},
{
"id": 12,
"entity": "红斑狼疮",
"start_offset": 76,
"end_offset": 80,
"label": "dis"
},
{
"id": 13,
"entity": "自身溶血性贫血",
"start_offset": 82,
"end_offset": 89,
"label": "dis"
}
] |
2.MG分型为标明MG肌无力分布部位、程度及病程，一般还采用Ossernen改良法分为以下类型：Ⅰ型（眼肌型）病变仅眼外肌受累Ⅱ型（全身型）ⅡA</sub>型表现眼、面和肢体肌无力全身无力并有咽喉肌无力球麻痹型。
|
[
{
"id": 0,
"entity": "MG",
"start_offset": 2,
"end_offset": 4,
"label": "dis"
},
{
"id": 1,
"entity": "MG肌无力",
"start_offset": 9,
"end_offset": 14,
"label": "dis"
},
{
"id": 2,
"entity": "Ⅰ型",
"start_offset": 48,
"end_offset": 50,
"label": "dis"
},
{
"id": 3,
"entity": "眼肌型",
"start_offset": 51,
"end_offset": 54,
"label": "dis"
},
{
"id": 4,
"entity": "眼外肌",
"start_offset": 58,
"end_offset": 61,
"label": "bod"
},
{
"id": 5,
"entity": "眼外肌受累",
"start_offset": 58,
"end_offset": 63,
"label": "sym"
},
{
"id": 6,
"entity": "Ⅱ型",
"start_offset": 63,
"end_offset": 65,
"label": "dis"
},
{
"id": 7,
"entity": "全身型",
"start_offset": 66,
"end_offset": 69,
"label": "dis"
},
{
"id": 8,
"entity": "眼",
"start_offset": 81,
"end_offset": 82,
"label": "bod"
},
{
"id": 9,
"entity": "面",
"start_offset": 83,
"end_offset": 84,
"label": "bod"
},
{
"id": 10,
"entity": "肢体",
"start_offset": 85,
"end_offset": 87,
"label": "bod"
},
{
"id": 11,
"entity": "肌无力",
"start_offset": 87,
"end_offset": 90,
"label": "dis"
},
{
"id": 12,
"entity": "眼、面和肢体肌无力",
"start_offset": 81,
"end_offset": 90,
"label": "sym"
},
{
"id": 13,
"entity": "全身无力",
"start_offset": 90,
"end_offset": 94,
"label": "sym"
},
{
"id": 14,
"entity": "咽喉",
"start_offset": 96,
"end_offset": 98,
"label": "bod"
},
{
"id": 15,
"entity": "咽喉肌无力",
"start_offset": 96,
"end_offset": 101,
"label": "sym"
},
{
"id": 16,
"entity": "球麻痹型",
"start_offset": 101,
"end_offset": 105,
"label": "dis"
}
] |
Ⅲ型（爆发型）突发全身无力，极易发生肌无力危象Ⅳ型（迁缓型）病程反复2年以上，常由Ⅰ型或Ⅱ型发展而来。
|
[
{
"id": 0,
"entity": "Ⅲ型",
"start_offset": 0,
"end_offset": 2,
"label": "dis"
},
{
"id": 1,
"entity": "爆发型",
"start_offset": 3,
"end_offset": 6,
"label": "dis"
},
{
"id": 2,
"entity": "肌无力",
"start_offset": 18,
"end_offset": 21,
"label": "bod"
},
{
"id": 3,
"entity": "极易发生肌无力危象",
"start_offset": 14,
"end_offset": 23,
"label": "sym"
},
{
"id": 4,
"entity": "Ⅳ型",
"start_offset": 23,
"end_offset": 25,
"label": "dis"
},
{
"id": 5,
"entity": "迁缓型",
"start_offset": 26,
"end_offset": 29,
"label": "dis"
}
] |
Ⅴ型（肌萎缩型）少数病人有肌萎缩。
|
[
{
"id": 0,
"entity": "Ⅴ型",
"start_offset": 0,
"end_offset": 2,
"label": "dis"
},
{
"id": 1,
"entity": "肌萎缩型",
"start_offset": 3,
"end_offset": 7,
"label": "dis"
},
{
"id": 2,
"entity": "肌萎缩",
"start_offset": 13,
"end_offset": 16,
"label": "dis"
}
] |
感冒、腹泻、激动、疲劳、月经、分娩或手术等常使病情加重，甚至出现危象，危及生命。
|
[
{
"id": 0,
"entity": "感冒",
"start_offset": 0,
"end_offset": 2,
"label": "dis"
},
{
"id": 1,
"entity": "腹泻",
"start_offset": 3,
"end_offset": 5,
"label": "dis"
},
{
"id": 2,
"entity": "手术",
"start_offset": 18,
"end_offset": 20,
"label": "pro"
},
{
"id": 3,
"entity": "危象",
"start_offset": 32,
"end_offset": 34,
"label": "sym"
}
] |
3.MG危象是指肌无力突然加重呼吸肌（包括膈肌及肋间肌）及咽喉肌严重无力导致呼吸困难。
|
[
{
"id": 0,
"entity": "MG危象",
"start_offset": 2,
"end_offset": 6,
"label": "dis"
},
{
"id": 1,
"entity": "肌无力",
"start_offset": 8,
"end_offset": 11,
"label": "dis"
},
{
"id": 2,
"entity": "指肌无力突然加重",
"start_offset": 7,
"end_offset": 15,
"label": "sym"
},
{
"id": 3,
"entity": "呼吸肌",
"start_offset": 15,
"end_offset": 18,
"label": "bod"
},
{
"id": 4,
"entity": "膈肌",
"start_offset": 21,
"end_offset": 23,
"label": "bod"
},
{
"id": 5,
"entity": "肋间肌",
"start_offset": 24,
"end_offset": 27,
"label": "bod"
},
{
"id": 6,
"entity": "咽喉肌",
"start_offset": 29,
"end_offset": 32,
"label": "bod"
},
{
"id": 7,
"entity": "呼吸肌（包括膈肌及肋间肌）及咽喉肌严重无力",
"start_offset": 15,
"end_offset": 36,
"label": "sym"
},
{
"id": 8,
"entity": "导致呼吸困难",
"start_offset": 36,
"end_offset": 42,
"label": "sym"
}
] |
多在重型基础上诱发，感染是危象发生的最常见的诱发因素，伴有胸腺瘤者易发生危象。
|
[
{
"id": 0,
"entity": "感染",
"start_offset": 10,
"end_offset": 12,
"label": "dis"
},
{
"id": 1,
"entity": "胸腺瘤",
"start_offset": 29,
"end_offset": 32,
"label": "dis"
}
] |
危象可分为三种①肌无力危象：为疾病本身肌无力加重所致，此时胆碱酯酶抑制剂往往剂量不足，加大药量或静脉注射腾喜龙后肌力好转。
|
[
{
"id": 0,
"entity": "肌无力危象",
"start_offset": 8,
"end_offset": 13,
"label": "dis"
},
{
"id": 1,
"entity": "肌无力",
"start_offset": 19,
"end_offset": 22,
"label": "dis"
},
{
"id": 2,
"entity": "胆碱酯酶抑制剂",
"start_offset": 29,
"end_offset": 36,
"label": "dru"
},
{
"id": 3,
"entity": "静脉注射",
"start_offset": 48,
"end_offset": 52,
"label": "pro"
},
{
"id": 4,
"entity": "腾喜龙",
"start_offset": 52,
"end_offset": 55,
"label": "dru"
},
{
"id": 5,
"entity": "肌力",
"start_offset": 56,
"end_offset": 58,
"label": "ite"
}
] |
常由感冒诱发，也可发生于应用神经-肌肉阻滞作用的药剂（如链霉素）、大剂量皮质类固醇、胸腺放射治疗或手术后。
|
[
{
"id": 0,
"entity": "感冒",
"start_offset": 2,
"end_offset": 4,
"label": "dis"
},
{
"id": 1,
"entity": "神经",
"start_offset": 14,
"end_offset": 16,
"label": "bod"
},
{
"id": 2,
"entity": "肌肉",
"start_offset": 17,
"end_offset": 19,
"label": "bod"
},
{
"id": 3,
"entity": "链霉素",
"start_offset": 28,
"end_offset": 31,
"label": "dru"
},
{
"id": 4,
"entity": "皮质类固醇",
"start_offset": 36,
"end_offset": 41,
"label": "dru"
},
{
"id": 5,
"entity": "胸腺放射治疗",
"start_offset": 42,
"end_offset": 48,
"label": "pro"
},
{
"id": 6,
"entity": "手术",
"start_offset": 49,
"end_offset": 51,
"label": "pro"
}
] |
②胆碱能危象：是由于胆碱酯酶抑制剂过量，使ACh免于水解，在突触积聚过多表现胆碱能毒性反应：肌无力加重肌束颤动烟碱样反应，终板膜过度除极化瞳孔缩小出汗，唾液增多毒素碱样反应）；头痛精神紧张（中枢神经反应注射腾喜龙无力症状不见好转，反而加重。
|
[
{
"id": 0,
"entity": "胆碱能危象",
"start_offset": 1,
"end_offset": 6,
"label": "dis"
},
{
"id": 1,
"entity": "胆碱酯酶抑制剂",
"start_offset": 10,
"end_offset": 17,
"label": "dru"
},
{
"id": 2,
"entity": "ACh",
"start_offset": 21,
"end_offset": 24,
"label": "dru"
},
{
"id": 3,
"entity": "突触",
"start_offset": 30,
"end_offset": 32,
"label": "bod"
},
{
"id": 4,
"entity": "在突触积聚过多",
"start_offset": 29,
"end_offset": 36,
"label": "sym"
},
{
"id": 5,
"entity": "表现胆碱能毒性反应",
"start_offset": 36,
"end_offset": 45,
"label": "sym"
},
{
"id": 6,
"entity": "肌无力",
"start_offset": 46,
"end_offset": 49,
"label": "ite"
},
{
"id": 7,
"entity": "肌无力加重",
"start_offset": 46,
"end_offset": 51,
"label": "sym"
},
{
"id": 8,
"entity": "肌束",
"start_offset": 51,
"end_offset": 53,
"label": "bod"
},
{
"id": 9,
"entity": "肌束颤动",
"start_offset": 51,
"end_offset": 55,
"label": "sym"
},
{
"id": 10,
"entity": "烟碱样反应",
"start_offset": 55,
"end_offset": 60,
"label": "sym"
},
{
"id": 11,
"entity": "终板膜",
"start_offset": 61,
"end_offset": 64,
"label": "bod"
},
{
"id": 12,
"entity": "终板膜过度除极化",
"start_offset": 61,
"end_offset": 69,
"label": "sym"
},
{
"id": 13,
"entity": "瞳孔",
"start_offset": 69,
"end_offset": 71,
"label": "bod"
},
{
"id": 14,
"entity": "瞳孔缩小",
"start_offset": 69,
"end_offset": 73,
"label": "sym"
},
{
"id": 15,
"entity": "出汗",
"start_offset": 73,
"end_offset": 75,
"label": "sym"
},
{
"id": 16,
"entity": "唾液",
"start_offset": 76,
"end_offset": 78,
"label": "bod"
},
{
"id": 17,
"entity": "唾液增多",
"start_offset": 76,
"end_offset": 80,
"label": "sym"
},
{
"id": 18,
"entity": "毒素碱样反应",
"start_offset": 80,
"end_offset": 86,
"label": "sym"
},
{
"id": 19,
"entity": "头",
"start_offset": 88,
"end_offset": 89,
"label": "bod"
},
{
"id": 20,
"entity": "头痛",
"start_offset": 88,
"end_offset": 90,
"label": "sym"
},
{
"id": 21,
"entity": "精神紧张",
"start_offset": 90,
"end_offset": 94,
"label": "sym"
},
{
"id": 22,
"entity": "中枢神经",
"start_offset": 95,
"end_offset": 99,
"label": "bod"
},
{
"id": 23,
"entity": "中枢神经反应",
"start_offset": 95,
"end_offset": 101,
"label": "sym"
},
{
"id": 24,
"entity": "注射腾喜龙",
"start_offset": 101,
"end_offset": 106,
"label": "pro"
},
{
"id": 25,
"entity": "无力症状不见好转",
"start_offset": 106,
"end_offset": 114,
"label": "sym"
},
{
"id": 26,
"entity": "反而加重",
"start_offset": 115,
"end_offset": 119,
"label": "sym"
}
] |
③反拗性危象：对胆碱酯酶抑制剂暂时失效，加大药量无济于事。
|
[
{
"id": 0,
"entity": "反拗性危象",
"start_offset": 1,
"end_offset": 6,
"label": "dis"
},
{
"id": 1,
"entity": "胆碱酯酶抑制剂",
"start_offset": 8,
"end_offset": 15,
"label": "dru"
}
] |
儿科无此危象的报告。
|
[
{
"id": 0,
"entity": "儿科",
"start_offset": 0,
"end_offset": 2,
"label": "dep"
}
] |
【诊断】（一）确定是否重症肌无力主要根据病史，典型的临床表现即受累骨骼肌活动后疲劳无力肌群可分成眼外肌、颜面肌、咽喉肌、颈肌、躯干肌和肢体肌等，经休息或用胆碱酯酶抑制剂可以缓解；且无神经系统其他体征。
|
[
{
"id": 0,
"entity": "重症肌无力",
"start_offset": 11,
"end_offset": 16,
"label": "dis"
},
{
"id": 1,
"entity": "骨骼肌",
"start_offset": 33,
"end_offset": 36,
"label": "bod"
},
{
"id": 2,
"entity": "受累骨骼肌活动后疲劳无力",
"start_offset": 31,
"end_offset": 43,
"label": "sym"
},
{
"id": 3,
"entity": "肌群",
"start_offset": 43,
"end_offset": 45,
"label": "bod"
},
{
"id": 4,
"entity": "眼外肌",
"start_offset": 48,
"end_offset": 51,
"label": "bod"
},
{
"id": 5,
"entity": "颜面肌",
"start_offset": 52,
"end_offset": 55,
"label": "bod"
},
{
"id": 6,
"entity": "咽喉肌",
"start_offset": 56,
"end_offset": 59,
"label": "bod"
},
{
"id": 7,
"entity": "颈肌",
"start_offset": 60,
"end_offset": 62,
"label": "bod"
},
{
"id": 8,
"entity": "躯干肌",
"start_offset": 63,
"end_offset": 66,
"label": "bod"
},
{
"id": 9,
"entity": "肢体肌",
"start_offset": 67,
"end_offset": 70,
"label": "bod"
},
{
"id": 10,
"entity": "休息",
"start_offset": 73,
"end_offset": 75,
"label": "pro"
},
{
"id": 11,
"entity": "胆碱酯酶抑制剂",
"start_offset": 77,
"end_offset": 84,
"label": "pro"
}
] |
1.疲劳实验阳性受累肌群连续运动后症状明显加重肌疲劳现象肌无力较轻、检查配合的年长儿童可选择疲劳试验。
|
[
{
"id": 0,
"entity": "疲劳实验",
"start_offset": 2,
"end_offset": 6,
"label": "pro"
},
{
"id": 1,
"entity": "肌群",
"start_offset": 10,
"end_offset": 12,
"label": "bod"
},
{
"id": 2,
"entity": "受累肌群连续运动后症状明显加重",
"start_offset": 8,
"end_offset": 23,
"label": "sym"
},
{
"id": 3,
"entity": "肌",
"start_offset": 23,
"end_offset": 24,
"label": "bod"
},
{
"id": 4,
"entity": "肌疲劳现象",
"start_offset": 23,
"end_offset": 28,
"label": "sym"
},
{
"id": 5,
"entity": "肌无",
"start_offset": 28,
"end_offset": 30,
"label": "dis"
},
{
"id": 6,
"entity": "肌无",
"start_offset": 28,
"end_offset": 30,
"label": "pro"
},
{
"id": 7,
"entity": "童可选择",
"start_offset": 42,
"end_offset": 46,
"label": "pro"
}
] |
成人MG患者强调定量疲劳实验，即选择不同的受累肌群，让其持续用力收缩，测量出现病态疲劳现象所需的时间及疲劳程度，并且制定有专项的评定量表。
|
[
{
"id": 0,
"entity": "成人MG",
"start_offset": 0,
"end_offset": 4,
"label": "dis"
},
{
"id": 1,
"entity": "疲劳实验",
"start_offset": 10,
"end_offset": 14,
"label": "pro"
},
{
"id": 2,
"entity": "肌群",
"start_offset": 23,
"end_offset": 25,
"label": "bod"
}
] |
但儿童MG以年幼儿童发病为主，检查依从性差，尚缺少年龄相关的儿童专项定量疲劳实验量表。
|
[
{
"id": 0,
"entity": "儿童MG",
"start_offset": 1,
"end_offset": 5,
"label": "dis"
}
] |
2.药物实验阳性甲基硫酸新斯的明实验：0.03～0.04mg/kg，肌注，比较注射前后半小时各受累肌群的肌力的变化，肌力明显改善者有助于MG的诊断腾喜龙试验：腾喜龙0.2mg/kg，以注射用水稀释至1ml，静脉注射，症状迅速缓解则为阳性，持续10分钟左右又恢复原状。
|
[
{
"id": 0,
"entity": "药物实验",
"start_offset": 2,
"end_offset": 6,
"label": "pro"
},
{
"id": 1,
"entity": "甲基硫酸新斯的明实验",
"start_offset": 8,
"end_offset": 18,
"label": "pro"
},
{
"id": 2,
"entity": "肌注",
"start_offset": 34,
"end_offset": 36,
"label": "pro"
},
{
"id": 3,
"entity": "肌群",
"start_offset": 49,
"end_offset": 51,
"label": "bod"
},
{
"id": 4,
"entity": "肌力",
"start_offset": 52,
"end_offset": 54,
"label": "ite"
},
{
"id": 5,
"entity": "肌力",
"start_offset": 58,
"end_offset": 60,
"label": "ite"
},
{
"id": 6,
"entity": "MG",
"start_offset": 68,
"end_offset": 70,
"label": "dis"
},
{
"id": 7,
"entity": "肌力明显改善者有助于MG的诊断",
"start_offset": 58,
"end_offset": 73,
"label": "sym"
},
{
"id": 8,
"entity": "腾喜龙试验",
"start_offset": 73,
"end_offset": 78,
"label": "pro"
},
{
"id": 9,
"entity": "腾喜龙",
"start_offset": 79,
"end_offset": 82,
"label": "dru"
},
{
"id": 10,
"entity": "静脉注射",
"start_offset": 103,
"end_offset": 107,
"label": "pro"
}
] |
对疲劳实验改善不明显者、肌无力较重病例以及疲劳实验不合作的年幼儿童选择药物试验。
|
[
{
"id": 0,
"entity": "疲劳实验",
"start_offset": 1,
"end_offset": 5,
"label": "pro"
},
{
"id": 1,
"entity": "肌无",
"start_offset": 12,
"end_offset": 14,
"label": "dis"
},
{
"id": 2,
"entity": "肌无",
"start_offset": 12,
"end_offset": 14,
"label": "pro"
},
{
"id": 3,
"entity": "病例以及",
"start_offset": 17,
"end_offset": 21,
"label": "pro"
},
{
"id": 4,
"entity": "儿童选择",
"start_offset": 31,
"end_offset": 35,
"label": "pro"
}
] |
3.肌电图神经低频重复电刺激示复合肌肉动作电位波幅衰减10%单纤维肌电图检查显示颤抖增宽，是目前敏感性及准确性最高的电生理检测手段。
|
[
{
"id": 0,
"entity": "肌电图",
"start_offset": 2,
"end_offset": 5,
"label": "pro"
},
{
"id": 1,
"entity": "神经低频重复电刺激",
"start_offset": 5,
"end_offset": 14,
"label": "pro"
},
{
"id": 2,
"entity": "复合肌肉动作电位波幅",
"start_offset": 15,
"end_offset": 25,
"label": "ite"
},
{
"id": 3,
"entity": "复合肌肉动作电位波幅衰减10%",
"start_offset": 15,
"end_offset": 30,
"label": "sym"
},
{
"id": 4,
"entity": "单纤维肌电图检查",
"start_offset": 30,
"end_offset": 38,
"label": "pro"
},
{
"id": 5,
"entity": "颤抖增宽",
"start_offset": 40,
"end_offset": 44,
"label": "sym"
},
{
"id": 6,
"entity": "电生理检测",
"start_offset": 58,
"end_offset": 63,
"label": "pro"
}
] |
前者阴性不能排除MG，后者在国内，特别是儿童尚未广泛开展。
|
[
{
"id": 0,
"entity": "MG",
"start_offset": 8,
"end_offset": 10,
"label": "dis"
}
] |
4.血清AChRab的检测AChRab检测是MG诊断重要的参考依据，若阳性者有助于诊断，阴性者不能排除MG。
|
[
{
"id": 0,
"entity": "血清AChRab的检测",
"start_offset": 2,
"end_offset": 13,
"label": "pro"
},
{
"id": 1,
"entity": "AChRab检测",
"start_offset": 13,
"end_offset": 21,
"label": "pro"
},
{
"id": 2,
"entity": "MG",
"start_offset": 22,
"end_offset": 24,
"label": "dis"
},
{
"id": 3,
"entity": "MG",
"start_offset": 51,
"end_offset": 53,
"label": "dis"
}
] |
眼肌型及儿童MG病例AChRab多阴性。
|
[
{
"id": 0,
"entity": "眼肌型",
"start_offset": 0,
"end_offset": 3,
"label": "dis"
},
{
"id": 1,
"entity": "儿童MG",
"start_offset": 4,
"end_offset": 8,
"label": "dis"
},
{
"id": 2,
"entity": "AChRab",
"start_offset": 10,
"end_offset": 16,
"label": "bod"
}
] |
（二）明确是否合并胸腺瘤成人病例约75%胸腺增生，15%MG合并胸腺瘤；我院（复旦大学附属儿童医院）资料4%胸腺瘤，42%胸腺增生。
|
[
{
"id": 0,
"entity": "胸腺瘤",
"start_offset": 9,
"end_offset": 12,
"label": "dis"
},
{
"id": 1,
"entity": "胸腺增生",
"start_offset": 20,
"end_offset": 24,
"label": "dis"
},
{
"id": 2,
"entity": "MG",
"start_offset": 28,
"end_offset": 30,
"label": "dis"
},
{
"id": 3,
"entity": "胸腺瘤",
"start_offset": 32,
"end_offset": 35,
"label": "dis"
},
{
"id": 4,
"entity": "胸腺瘤",
"start_offset": 54,
"end_offset": 57,
"label": "dis"
},
{
"id": 5,
"entity": "胸腺增生",
"start_offset": 61,
"end_offset": 65,
"label": "dis"
}
] |
肿瘤常位于前上纵隔，除表现肌无力，一般无占位病变的症状和体征，易漏诊。
|
[
{
"id": 0,
"entity": "肿瘤",
"start_offset": 0,
"end_offset": 2,
"label": "dis"
},
{
"id": 1,
"entity": "肌无力",
"start_offset": 13,
"end_offset": 16,
"label": "dis"
},
{
"id": 2,
"entity": "无占位病变的症状和体征",
"start_offset": 19,
"end_offset": 30,
"label": "sym"
}
] |
胸腺瘤多见于40岁以后男性患者，肌无力症状较重对胆碱酯酶抑制剂疗效不佳易发生危象。
|
[
{
"id": 0,
"entity": "胸腺瘤",
"start_offset": 0,
"end_offset": 3,
"label": "dis"
},
{
"id": 1,
"entity": "肌无力",
"start_offset": 16,
"end_offset": 19,
"label": "dis"
},
{
"id": 2,
"entity": "肌无力症状较重",
"start_offset": 16,
"end_offset": 23,
"label": "sym"
},
{
"id": 3,
"entity": "胆碱酯酶抑制剂",
"start_offset": 24,
"end_offset": 31,
"label": "dru"
},
{
"id": 4,
"entity": "对胆碱酯酶抑制剂疗效不佳",
"start_offset": 23,
"end_offset": 35,
"label": "sym"
},
{
"id": 5,
"entity": "易发生危象",
"start_offset": 35,
"end_offset": 40,
"label": "sym"
}
] |
侧位或正位X光胸片偶可发现异常，纵隔CT扫描可直接显示肿瘤部位、大小、形状以及与邻近器官的关系。
|
[
{
"id": 0,
"entity": "侧位或正位X光胸片",
"start_offset": 0,
"end_offset": 9,
"label": "pro"
},
{
"id": 1,
"entity": "纵隔CT扫描",
"start_offset": 16,
"end_offset": 22,
"label": "pro"
},
{
"id": 2,
"entity": "肿瘤",
"start_offset": 27,
"end_offset": 29,
"label": "dis"
}
] |
免疫学检查：CAEab（又称胸腺瘤相关抗体）对MG患者提示胸腺瘤具有重要价值。
|
[
{
"id": 0,
"entity": "免疫学检查",
"start_offset": 0,
"end_offset": 5,
"label": "pro"
},
{
"id": 1,
"entity": "CAEab",
"start_offset": 6,
"end_offset": 11,
"label": "bod"
},
{
"id": 2,
"entity": "胸腺瘤",
"start_offset": 14,
"end_offset": 17,
"label": "dis"
},
{
"id": 3,
"entity": "MG",
"start_offset": 23,
"end_offset": 25,
"label": "dis"
},
{
"id": 4,
"entity": "胸腺瘤",
"start_offset": 29,
"end_offset": 32,
"label": "dis"
}
] |
MG合并胸腺瘤CAEab阳性率高达80%～90%。
|
[
{
"id": 0,
"entity": "MG",
"start_offset": 0,
"end_offset": 2,
"label": "dis"
},
{
"id": 1,
"entity": "胸腺瘤CAEab",
"start_offset": 4,
"end_offset": 12,
"label": "dis"
}
] |
诊断尚需结合临床和CT纵隔扫描，综合分析。
|
[
{
"id": 0,
"entity": "CT纵隔扫描",
"start_offset": 9,
"end_offset": 15,
"label": "pro"
}
] |
（三）明确有无其他并存症MG作为自身免疫疾病中一种“姐妹病”，可伴有以下夹杂症：如甲状腺功能亢进，类风湿关节炎，系统性红斑狼疮，溶血性贫血，多发性肌炎或多发性硬化等。
|
[
{
"id": 0,
"entity": "MG",
"start_offset": 12,
"end_offset": 14,
"label": "dis"
},
{
"id": 1,
"entity": "姐妹病",
"start_offset": 26,
"end_offset": 29,
"label": "dis"
},
{
"id": 2,
"entity": "甲状腺功能亢进",
"start_offset": 41,
"end_offset": 48,
"label": "dis"
},
{
"id": 3,
"entity": "类风湿关节炎",
"start_offset": 49,
"end_offset": 55,
"label": "dis"
},
{
"id": 4,
"entity": "系统性红斑狼疮",
"start_offset": 56,
"end_offset": 63,
"label": "dis"
},
{
"id": 5,
"entity": "溶血性贫血",
"start_offset": 64,
"end_offset": 69,
"label": "dis"
},
{
"id": 6,
"entity": "多发性肌炎",
"start_offset": 70,
"end_offset": 75,
"label": "dis"
},
{
"id": 7,
"entity": "多发性硬化",
"start_offset": 76,
"end_offset": 81,
"label": "dis"
}
] |
（四）鉴别诊断MG急性肌无力应与其他急性瘫痪疾病鉴别：包括①周期性瘫痪。
|
[
{
"id": 0,
"entity": "MG",
"start_offset": 7,
"end_offset": 9,
"label": "dis"
},
{
"id": 1,
"entity": "肌无力",
"start_offset": 11,
"end_offset": 14,
"label": "dis"
},
{
"id": 2,
"entity": "瘫痪疾病",
"start_offset": 20,
"end_offset": 24,
"label": "dis"
},
{
"id": 3,
"entity": "周期性瘫痪",
"start_offset": 30,
"end_offset": 35,
"label": "dis"
}
] |
常在夜间发病，醒来时发现四肢无力血钾低心电图出现U波补钾治疗有效。
|
[
{
"id": 0,
"entity": "四肢",
"start_offset": 12,
"end_offset": 14,
"label": "bod"
},
{
"id": 1,
"entity": "四肢无力",
"start_offset": 12,
"end_offset": 16,
"label": "sym"
},
{
"id": 2,
"entity": "血钾",
"start_offset": 16,
"end_offset": 18,
"label": "bod"
},
{
"id": 3,
"entity": "血钾低",
"start_offset": 16,
"end_offset": 19,
"label": "sym"
},
{
"id": 4,
"entity": "心电图",
"start_offset": 19,
"end_offset": 22,
"label": "pro"
},
{
"id": 5,
"entity": "心电图出现U波",
"start_offset": 19,
"end_offset": 26,
"label": "sym"
},
{
"id": 6,
"entity": "补钾治疗",
"start_offset": 26,
"end_offset": 30,
"label": "pro"
}
] |
②急性炎症性脱髓鞘多发神经根病。
|
[
{
"id": 0,
"entity": "急性炎症性脱髓鞘多发神经根病",
"start_offset": 1,
"end_offset": 15,
"label": "dis"
}
] |
病初有发热或腹泻，除肢体瘫痪外，尚有神经根牵拉痛脑脊液有蛋白-细胞分离现象脊髓炎。
|
[
{
"id": 0,
"entity": "发热",
"start_offset": 3,
"end_offset": 5,
"label": "sym"
},
{
"id": 1,
"entity": "腹泻",
"start_offset": 6,
"end_offset": 8,
"label": "sym"
},
{
"id": 2,
"entity": "肢体瘫痪",
"start_offset": 10,
"end_offset": 14,
"label": "dis"
},
{
"id": 3,
"entity": "神经根",
"start_offset": 18,
"end_offset": 21,
"label": "bod"
},
{
"id": 4,
"entity": "神经根牵拉痛",
"start_offset": 18,
"end_offset": 24,
"label": "sym"
},
{
"id": 5,
"entity": "脑脊液",
"start_offset": 24,
"end_offset": 27,
"label": "bod"
},
{
"id": 6,
"entity": "蛋白",
"start_offset": 28,
"end_offset": 30,
"label": "bod"
},
{
"id": 7,
"entity": "细胞",
"start_offset": 31,
"end_offset": 33,
"label": "bod"
},
{
"id": 8,
"entity": "脑脊液有蛋白-细胞分离现象",
"start_offset": 24,
"end_offset": 37,
"label": "sym"
},
{
"id": 9,
"entity": "脊髓炎",
"start_offset": 37,
"end_offset": 40,
"label": "dis"
}
] |
有发热及脊髓损害上运动神经元型瘫痪、横截型感觉障碍及排尿障碍）。
|
[
{
"id": 0,
"entity": "发热",
"start_offset": 1,
"end_offset": 3,
"label": "sym"
},
{
"id": 1,
"entity": "脊髓",
"start_offset": 4,
"end_offset": 6,
"label": "bod"
},
{
"id": 2,
"entity": "脊髓损害",
"start_offset": 4,
"end_offset": 8,
"label": "sym"
},
{
"id": 3,
"entity": "上运动神经元型瘫痪",
"start_offset": 8,
"end_offset": 17,
"label": "dis"
},
{
"id": 4,
"entity": "横截型感觉障碍",
"start_offset": 18,
"end_offset": 25,
"label": "dis"
},
{
"id": 5,
"entity": "排尿障碍",
"start_offset": 26,
"end_offset": 30,
"label": "dis"
}
] |
慢性肌无力需要和以下疾病鉴别：包括①动眼神经麻痹。
|
[
{
"id": 0,
"entity": "慢性肌无力",
"start_offset": 0,
"end_offset": 5,
"label": "dis"
},
{
"id": 1,
"entity": "动眼神经麻痹",
"start_offset": 18,
"end_offset": 24,
"label": "dis"
}
] |
麻痹侧除上睑下垂可见瞳孔散大眼球向上、下及内收运动受限神经炎或颅内动脉瘤。
|
[
{
"id": 0,
"entity": "上睑",
"start_offset": 4,
"end_offset": 6,
"label": "bod"
},
{
"id": 1,
"entity": "上睑下垂",
"start_offset": 4,
"end_offset": 8,
"label": "sym"
},
{
"id": 2,
"entity": "瞳孔",
"start_offset": 10,
"end_offset": 12,
"label": "bod"
},
{
"id": 3,
"entity": "可见瞳孔散大",
"start_offset": 8,
"end_offset": 14,
"label": "sym"
},
{
"id": 4,
"entity": "眼球",
"start_offset": 14,
"end_offset": 16,
"label": "bod"
},
{
"id": 5,
"entity": "眼球向上、下及内收运动受限",
"start_offset": 14,
"end_offset": 27,
"label": "sym"
},
{
"id": 6,
"entity": "神经炎",
"start_offset": 27,
"end_offset": 30,
"label": "dis"
},
{
"id": 7,
"entity": "颅内动脉瘤",
"start_offset": 31,
"end_offset": 36,
"label": "dis"
}
] |
②多发性肌炎。
|
[
{
"id": 0,
"entity": "多发性肌炎",
"start_offset": 1,
"end_offset": 6,
"label": "dis"
}
] |
四肢近端肌无力肌痛肌酶升高肌营养不良。
|
[
{
"id": 0,
"entity": "四肢",
"start_offset": 0,
"end_offset": 2,
"label": "bod"
},
{
"id": 1,
"entity": "近端肌",
"start_offset": 2,
"end_offset": 5,
"label": "bod"
},
{
"id": 2,
"entity": "四肢近端肌无力",
"start_offset": 0,
"end_offset": 7,
"label": "sym"
},
{
"id": 3,
"entity": "肌",
"start_offset": 7,
"end_offset": 8,
"label": "bod"
},
{
"id": 4,
"entity": "肌痛",
"start_offset": 7,
"end_offset": 9,
"label": "sym"
},
{
"id": 5,
"entity": "肌酶",
"start_offset": 9,
"end_offset": 11,
"label": "bod"
},
{
"id": 6,
"entity": "肌酶升高",
"start_offset": 9,
"end_offset": 13,
"label": "sym"
},
{
"id": 7,
"entity": "肌营养不良",
"start_offset": 13,
"end_offset": 18,
"label": "dis"
}
] |
缓慢进行性肢体无力，肌萎缩，儿童患者翼状肩胛，腓肠肌假肥大血肌酶升高线粒体肌病。
|
[
{
"id": 0,
"entity": "缓慢进行性肢体无力",
"start_offset": 0,
"end_offset": 9,
"label": "dis"
},
{
"id": 1,
"entity": "肌萎缩",
"start_offset": 10,
"end_offset": 13,
"label": "dis"
},
{
"id": 2,
"entity": "翼状肩胛",
"start_offset": 18,
"end_offset": 22,
"label": "sym"
},
{
"id": 3,
"entity": "腓肠肌",
"start_offset": 23,
"end_offset": 26,
"label": "bod"
},
{
"id": 4,
"entity": "腓肠肌假肥大",
"start_offset": 23,
"end_offset": 29,
"label": "sym"
},
{
"id": 5,
"entity": "血肌酶",
"start_offset": 29,
"end_offset": 32,
"label": "bod"
},
{
"id": 6,
"entity": "血肌酶升高",
"start_offset": 29,
"end_offset": 34,
"label": "sym"
},
{
"id": 7,
"entity": "线粒体肌病",
"start_offset": 34,
"end_offset": 39,
"label": "dis"
}
] |
骨骼肌极度不能耐受疲劳血乳酸升高，肌活体组织检查可见不整红边纤维电镜示异常线粒体糖原累积病。
|
[
{
"id": 0,
"entity": "骨骼肌",
"start_offset": 0,
"end_offset": 3,
"label": "bod"
},
{
"id": 1,
"entity": "骨骼肌极度不能耐受疲劳",
"start_offset": 0,
"end_offset": 11,
"label": "sym"
},
{
"id": 2,
"entity": "血乳酸升高",
"start_offset": 11,
"end_offset": 16,
"label": "sym"
},
{
"id": 3,
"entity": "肌活体组织检查",
"start_offset": 17,
"end_offset": 24,
"label": "pro"
},
{
"id": 4,
"entity": "纤维",
"start_offset": 30,
"end_offset": 32,
"label": "bod"
},
{
"id": 5,
"entity": "肌活体组织检查可见不整红边纤维",
"start_offset": 17,
"end_offset": 32,
"label": "sym"
},
{
"id": 6,
"entity": "电镜",
"start_offset": 32,
"end_offset": 34,
"label": "pro"
},
{
"id": 7,
"entity": "线粒体",
"start_offset": 37,
"end_offset": 40,
"label": "bod"
},
{
"id": 8,
"entity": "电镜示异常线粒体",
"start_offset": 32,
"end_offset": 40,
"label": "sym"
},
{
"id": 9,
"entity": "糖原累积病",
"start_offset": 40,
"end_offset": 45,
"label": "dis"
}
] |
其中尤其以Ⅱ型患者，酸性麦芽糖酶缺乏引起肢带肌无力，可出现呼吸肌麻痹，易误诊，肌活体组织检查PAS染色可见糖原积累癌性肌无力，主要多见于年老患者小细胞肺癌，肢体无力活动后缓解，高频反复电刺激神经肌电图示肌电位递增运动神经元病。
|
[
{
"id": 0,
"entity": "酸性麦芽糖酶缺乏",
"start_offset": 10,
"end_offset": 18,
"label": "dis"
},
{
"id": 1,
"entity": "肢带肌无力",
"start_offset": 20,
"end_offset": 25,
"label": "dis"
},
{
"id": 2,
"entity": "呼吸肌麻痹",
"start_offset": 29,
"end_offset": 34,
"label": "dis"
},
{
"id": 3,
"entity": "肌活体组织检查PAS染色",
"start_offset": 39,
"end_offset": 51,
"label": "pro"
},
{
"id": 4,
"entity": "糖原",
"start_offset": 53,
"end_offset": 55,
"label": "bod"
},
{
"id": 5,
"entity": "糖原积累",
"start_offset": 53,
"end_offset": 57,
"label": "sym"
},
{
"id": 6,
"entity": "癌性肌无力",
"start_offset": 57,
"end_offset": 62,
"label": "dis"
},
{
"id": 7,
"entity": "小细胞肺癌",
"start_offset": 72,
"end_offset": 77,
"label": "dis"
},
{
"id": 8,
"entity": "肢体",
"start_offset": 78,
"end_offset": 80,
"label": "bod"
},
{
"id": 9,
"entity": "肢体无力",
"start_offset": 78,
"end_offset": 82,
"label": "sym"
},
{
"id": 10,
"entity": "活动后缓解",
"start_offset": 82,
"end_offset": 87,
"label": "sym"
},
{
"id": 11,
"entity": "高频反复电刺激神经肌电图",
"start_offset": 88,
"end_offset": 100,
"label": "pro"
},
{
"id": 12,
"entity": "肌电位",
"start_offset": 101,
"end_offset": 104,
"label": "ite"
},
{
"id": 13,
"entity": "高频反复电刺激神经肌电图示肌电位递增",
"start_offset": 88,
"end_offset": 106,
"label": "sym"
},
{
"id": 14,
"entity": "运动神经元病",
"start_offset": 106,
"end_offset": 112,
"label": "dis"
}
] |
早期仅表现舌及肢体肌无力肌萎缩、肌纤维颤动或锥体束征则鉴别不难。
|
[
{
"id": 0,
"entity": "舌",
"start_offset": 5,
"end_offset": 6,
"label": "bod"
},
{
"id": 1,
"entity": "肢体",
"start_offset": 7,
"end_offset": 9,
"label": "bod"
},
{
"id": 2,
"entity": "舌及肢体肌无力",
"start_offset": 5,
"end_offset": 12,
"label": "sym"
},
{
"id": 3,
"entity": "肌萎缩",
"start_offset": 12,
"end_offset": 15,
"label": "dis"
},
{
"id": 4,
"entity": "肌纤维颤动",
"start_offset": 16,
"end_offset": 21,
"label": "dis"
},
{
"id": 5,
"entity": "锥体束征",
"start_offset": 22,
"end_offset": 26,
"label": "dis"
}
] |
【治疗】（一）胆碱酯酶抑制剂（AchEI）可选用溴化新斯的明，剂量每次0.5mg/kg日服3～4次；吡啶新斯的明，剂量每次2mg/kg，日服4次；溴化吡啶新斯的明，每次剂量7mg/kg，日服3次。
|
[
{
"id": 0,
"entity": "胆碱酯酶抑制剂",
"start_offset": 7,
"end_offset": 14,
"label": "dru"
},
{
"id": 1,
"entity": "AchEI",
"start_offset": 15,
"end_offset": 20,
"label": "dru"
},
{
"id": 2,
"entity": "溴化新斯的明",
"start_offset": 24,
"end_offset": 30,
"label": "dru"
},
{
"id": 3,
"entity": "吡啶新斯的明",
"start_offset": 50,
"end_offset": 56,
"label": "dru"
},
{
"id": 4,
"entity": "溴化吡啶新斯的明",
"start_offset": 73,
"end_offset": 81,
"label": "dru"
}
] |
总之，胆碱酯酶抑制剂作为一种有效的对症、辅助治疗药物，不宜长期单独应用。
|
[
{
"id": 0,
"entity": "胆碱酯酶抑制剂",
"start_offset": 3,
"end_offset": 10,
"label": "dru"
}
] |
长期依赖，滥用胆碱酯酶抑制剂，有碍AchR修复，须避免此类药物的弊端。
|
[
{
"id": 0,
"entity": "胆碱酯酶抑制剂",
"start_offset": 7,
"end_offset": 14,
"label": "dru"
},
{
"id": 1,
"entity": "AchR",
"start_offset": 17,
"end_offset": 21,
"label": "bod"
}
] |
辅助药物如氯化钾和麻黄碱等可加强新斯的明的作用。
|
[
{
"id": 0,
"entity": "氯化钾",
"start_offset": 5,
"end_offset": 8,
"label": "dru"
},
{
"id": 1,
"entity": "麻黄碱",
"start_offset": 9,
"end_offset": 12,
"label": "dru"
},
{
"id": 2,
"entity": "新斯的明",
"start_offset": 16,
"end_offset": 20,
"label": "dru"
}
] |
忌用对神经-肌肉传递阻滞的药物，如各种氨基糖苷类的抗生素、奎宁、奎宁丁、普鲁卡因胺、普萘洛尔、氯丙嗪以及各种肌肉松弛剂。
|
[
{
"id": 0,
"entity": "神经",
"start_offset": 3,
"end_offset": 5,
"label": "bod"
},
{
"id": 1,
"entity": "肌肉",
"start_offset": 6,
"end_offset": 8,
"label": "bod"
},
{
"id": 2,
"entity": "氨基糖苷类的抗生素",
"start_offset": 19,
"end_offset": 28,
"label": "dru"
},
{
"id": 3,
"entity": "奎宁",
"start_offset": 29,
"end_offset": 31,
"label": "dru"
},
{
"id": 4,
"entity": "奎宁丁",
"start_offset": 32,
"end_offset": 35,
"label": "dru"
},
{
"id": 5,
"entity": "普鲁卡因胺",
"start_offset": 36,
"end_offset": 41,
"label": "dru"
},
{
"id": 6,
"entity": "普萘洛尔",
"start_offset": 42,
"end_offset": 46,
"label": "dru"
},
{
"id": 7,
"entity": "氯丙嗪",
"start_offset": 47,
"end_offset": 50,
"label": "dru"
},
{
"id": 8,
"entity": "肌肉松弛剂",
"start_offset": 54,
"end_offset": 59,
"label": "dru"
}
] |
（二）免疫抑制剂1.皮质类固醇为最常用的免疫治疗药物，无论是眼肌型还是全身型都可选用泼尼松，1～1.5mg/（kg•d）。
|
[
{
"id": 0,
"entity": "免疫抑制剂",
"start_offset": 3,
"end_offset": 8,
"label": "dru"
},
{
"id": 1,
"entity": "皮质类固醇",
"start_offset": 10,
"end_offset": 15,
"label": "dru"
},
{
"id": 2,
"entity": "眼肌型",
"start_offset": 30,
"end_offset": 33,
"label": "dis"
},
{
"id": 3,
"entity": "全身型",
"start_offset": 35,
"end_offset": 38,
"label": "dis"
},
{
"id": 4,
"entity": "泼尼松",
"start_offset": 42,
"end_offset": 45,
"label": "dru"
}
] |
或病初使用甲泼尼龙冲击疗法，儿童20mg/（kg•d），静脉滴注，连用3～5天，起效快，适用重症或危象患者，用药方便，甚至可取代血浆交换疗法。
|
[
{
"id": 0,
"entity": "甲泼尼龙冲击疗法",
"start_offset": 5,
"end_offset": 13,
"label": "pro"
},
{
"id": 1,
"entity": "静脉滴注",
"start_offset": 28,
"end_offset": 32,
"label": "pro"
},
{
"id": 2,
"entity": "血浆交换疗法",
"start_offset": 64,
"end_offset": 70,
"label": "pro"
}
] |
但有一过性高血糖、高血压、继发感染及胃出血等不良反应，值得重视。
|
[
{
"id": 0,
"entity": "一过性高血糖",
"start_offset": 2,
"end_offset": 8,
"label": "dis"
},
{
"id": 1,
"entity": "高血压",
"start_offset": 9,
"end_offset": 12,
"label": "dis"
},
{
"id": 2,
"entity": "继发感染",
"start_offset": 13,
"end_offset": 17,
"label": "dis"
},
{
"id": 3,
"entity": "胃出血",
"start_offset": 18,
"end_offset": 21,
"label": "dis"
}
] |
病情缓解后逐渐减量改为泼尼松小剂量，隔日晨服，维持至少1年以上。
|
[
{
"id": 0,
"entity": "泼尼松",
"start_offset": 11,
"end_offset": 14,
"label": "dru"
}
] |
大剂量类固醇可使病情加重，多发生在用药1周内，可促发危象。
|
[
{
"id": 0,
"entity": "类固醇",
"start_offset": 3,
"end_offset": 6,
"label": "dru"
}
] |
发生机制是直接阻抑AChR离子通道。
|
[
{
"id": 0,
"entity": "AChR离子通道",
"start_offset": 9,
"end_offset": 17,
"label": "bod"
}
] |
2.其他免疫抑制剂可选用环磷酰胺、硫唑嘌呤或环孢素，对难治病例、发生危象病例以及胸腺切除术后疗效不佳者有效。
|
[
{
"id": 0,
"entity": "环磷酰胺",
"start_offset": 12,
"end_offset": 16,
"label": "dru"
},
{
"id": 1,
"entity": "硫唑嘌呤",
"start_offset": 17,
"end_offset": 21,
"label": "dru"
},
{
"id": 2,
"entity": "环孢素",
"start_offset": 22,
"end_offset": 25,
"label": "dru"
},
{
"id": 3,
"entity": "胸腺切除术",
"start_offset": 40,
"end_offset": 45,
"label": "pro"
}
] |
需注意血象和肝、肾功能的变化。
|
[
{
"id": 0,
"entity": "血象",
"start_offset": 3,
"end_offset": 5,
"label": "ite"
},
{
"id": 1,
"entity": "肝",
"start_offset": 6,
"end_offset": 7,
"label": "bod"
},
{
"id": 2,
"entity": "肾功能",
"start_offset": 8,
"end_offset": 11,
"label": "ite"
}
] |
（三）放射治疗至今胸腺放射治疗还是对MG一种确实有效的治疗方法。
|
[
{
"id": 0,
"entity": "放射治疗",
"start_offset": 3,
"end_offset": 7,
"label": "pro"
},
{
"id": 1,
"entity": "胸腺放射治疗",
"start_offset": 9,
"end_offset": 15,
"label": "pro"
},
{
"id": 2,
"entity": "MG",
"start_offset": 18,
"end_offset": 20,
"label": "dis"
}
] |
被称作是“非手术的手术治疗”。
|
[
{
"id": 0,
"entity": "非手术的手术治疗",
"start_offset": 5,
"end_offset": 13,
"label": "pro"
}
] |
适用于：①MG药物疗效不明显者，最好于发病2～3年内及早放射治疗；②巨大或多个胸腺瘤，无法手术或作为术前准备治疗；③恶性肿瘤术后追加放射治疗。
|
[
{
"id": 0,
"entity": "MG",
"start_offset": 5,
"end_offset": 7,
"label": "dis"
},
{
"id": 1,
"entity": "胸腺瘤",
"start_offset": 39,
"end_offset": 42,
"label": "dis"
},
{
"id": 2,
"entity": "恶性肿瘤",
"start_offset": 58,
"end_offset": 62,
"label": "dis"
},
{
"id": 3,
"entity": "放射治疗",
"start_offset": 66,
"end_offset": 70,
"label": "pro"
}
] |
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