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作 者:梁波 江其龙[2] 杨晓军[2] 刘小斌[2] LIANG Bo;JIANG Qiong;YANG Xiaojun;LIU Xiaobin(Guangzhou University of Chinese Medicine,Guangzhou 510405,China;The First Affiliated Hospital of GuangzhouUniversity of Chinese Medicine,Guangzhou 510405,China)
机构地区:[1]广州中医药大学,广州510405 [2]广州中医药大学第一附属医院,广州510405
出 处:《长春中医药大学学报》2023年第1期27-31,共5页Journal of Changchun University of Chinese Medicine
基 金:国家自然科学基金项目(81904133);广州中医药大学校科研团队培育项目(2019KYTD101)。
摘 要:Lambert-Eaton肌无力综合征(LEMS)是一种罕见的发生于神经-肌肉接头处的自身免疫性疾病,发病率低,临床误诊及漏诊率高,救治率低,其发病机制与功能性P/Q型电压门控钙离子通道(VGCC)抗体密切相关,VGCC抗体的攻击导致突触前膜释放乙酰胆碱功能受限,最终导致终板电位紊乱,肌肉收缩无力、失用性萎缩。目前,医院疾病编码名称为“副肿瘤综合征,相关性肌无力综合征”。沙丁胺醇是一种选择性β2-AR激动剂,协调T细胞因子平衡并可抑制B细胞的活化,降低乙酰胆碱受体抗体产生发挥免疫抑制作用。刘小斌教授认为,肿瘤相关性LEMS若以其四肢近端肌力减退、肌肉萎缩、深反射减弱等为主要症状,属祖国医学“痿病”范畴,虚则为痿,实则为积。其病因病机为脾胃虚损,气血失衡。刘小斌教授在长期临证实践基础上,提出肿瘤相关性LEMS以“脾胃虚损,五脏相关”的病理机制理论。辨证用药抓住“独取阳明,峻补脾肾;五脏相关,兼顾整体;调整阴阳,补养气血”要义,运用补中益气汤、麻黄附子细辛汤为基础方加减治疗肿瘤相关性LEMS,配合血肉有情之物,调整气血阴阳。Lambert-Eaton myasthenic syndrome(LEMS) is a rare autoimmune disease that occurs at the neuromuscular junction,with a low incidence, high clinical misdiagnosis and missed diagnosis rates, and low treatment rates. Its pathogenesis is closely related to the functional P/Q-type voltage-gated calcium channel(VGCC) antibody, whose invasion leads to the limited function of acetylcholine release from the presynaptic membrane, which eventually results in the disorder of end-plate potential, weak muscle contraction, and disuse atrophy. At present, the coded name of the disease in hospital is "paraneoplastic syndrome, associated myasthenia syndrome". Salbutamol is a selective β2-AR agonist that coordinates the T-cell factor balance and inhibits the B-cell activation and reduces the acetylcholine receptor antibody production to exert immunosuppressive effects. Professor Liu Xiaobin believes that if tumor-related LEMS has its main symptoms such as muscle weakness in proximal limbs, muscle atrophy, and weakened deep reflexes, it belongs to the category of "atrophic disease" in Chinese medicine, and the etiology and pathogenesis of this disease is the deficiency of the spleen and stomach, and the imbalance of qi and blood. On the basis of long-term clinical practice, he proposes the theory of tumor-related LEMS with the pathological mechanism of "deficiency of the spleen and stomach,related to the five internal organs". The syndrome differentiation and medications for the disease should focus on the essence of "referring to Yangming alone, strengthening the spleen and kidney;holding the five internal organs to be related, taking into account the whole;coordinating yin and yang, nourishing qi and blood", using Buzhong Yiqi Decoction and Mahuang Fuzi Xixin Decoction as the basis for its treatment, in conjunction with herbs beneficial for fleshes and bones to regulate qi, blood, yin and yang.
关 键 词:LAMBERT-EATON肌无力综合征 补中益气汤 麻黄附子细辛汤 沙丁胺醇 刘小斌
分 类 号:R746.1[医药卫生—神经病学与精神病学]
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