晏显妮 江其龙 刘小斌

〔摘要〕 脊髓延髓性肌萎缩是一种遗传性位于脑干和脊髓的运动神经元神经退行性疾病，为临床罕见疑难病症，需要依靠基因检测确诊，西医目前缺乏特异性治疗方法。刘小斌教授认为本病归属于中医学“痿证”范畴，病机为“禀赋残缺，胎元不足”“脾胃虚损，肝肾亏虚”。刘教授临床治疗本病多以黄芪、五指毛桃、鹿角霜、紫河车及其他补益之药“调理脾胃，培补肝肾，充盈气血”，注重药食同调，灸药结合，中西医并用;将其中医证型分为脾胃气虚证和脾肾亏虚证，疾病早期以补中益气汤为基础方加减治疗，病至后期，以补中益气汤合地黄饮子加减。

〔关键词〕 脊髓延髓性肌萎缩;补中益气汤;临床经验;刘小斌;禀赋

〔中图分类号〕R277.7       〔文献标志码〕B        〔文章编号〕doi：10.3969/j.issn.1674-070X.2021.10.026

Professor Liu Xiaobin's Experience in Treating Spinal and Bulbar Muscular Atrophy

YAN Xianni1， JIANG Qilong1， LIU Xiaobin2*

（1. The First Affiliated Hospital of Guangzhou University of Chinese Medicine， Guangzhou， Guangdong 510405， China;

2. Deng Tietao Institute of Guangzhou University of Chinese Medicine， Guangzhou， Guangdong 510405， China）

〔Abstract〕 Spinal and bulbar muscular atrophy is a hereditary motor neuron neurodegenerative disease located in the brainstem and spinal cord， which is a rare and intractable diseases who depending on genetic testing to diagnose， western medicine lacks specific treatment method at present. Professor Liu Xiaobin thinks that the disease belongs to the category of "flaccidity syndrome” in traditional Chinese medicine （TCM）， the pathogenesis of which is “abnormal of innate， insufficient in endowment”， “deficiency

of spleen and stomach， liver and kidney weakened”. Professor Liu are good at using Huangqi （Astragali Radix）， Wuzhimaotao （Radix Fici Simplicissimae）， Lujiaoshuang （Cervi Cornu Degelatinatum）， Ziheche （Placenta Hominis） and nourishing herbs to “regulating the spleen and stomach， invigorating the liver and kidney， nourishing Qi and blood”， emphasizing on co-ordination of medicine and food， combination of moxibustion and medicine， and combination of Chinese and western medicine. TCM syndrome differentiation of this disease are divided into spleen and stomach Qi deficiency syndrome and spleen and kidney deficiency syndrome. Buzhong Yiqi Decoction is applied to treating this type disease in the early stage， in the later stage， using the Buzhong Yiqi Decoction combined with Dihuang Yinzi.

〔Keywords〕 spinal and bulbar muscular atrophy; Buzhong Yiqi Decoction; clinical experience; Liu Xiaobin; innate constitution

脊髓延髓性肌萎縮（spinal and bulbar muscular atrophy， SBMA）又称肯尼迪病，是一种罕见的X连锁遗传性下运动神经元疾病，其特征是进行性肌无力[1]。SBMA以30～50岁男性多见，临床表现为缓慢进展的肢体近端及球部肌无力、肌萎缩[2]，可伴手部震颤、肌肉痉挛、肌肉萎缩、吞咽困难、构音障碍、四肢无力，最终致轮椅生活[3]。SBMA临床表现以下运动神经元为主，感觉神经和内分泌系统均可受累[4]。编码谷氨酰胺的雄激素受体（androgen receptor， AR）的基因第一外显子CAG重复拷贝（CAG拷贝数>37次），产生突变蛋白，突变蛋白的毒性影响运动神经元和肌肉而致病[5]。疾病发病机制包括下运动神经元和骨骼肌的毒性，其中对转录、细胞内转运和线粒体功能的影响已被记录在案。尽管靶向雄激素作用或促进突变雄激素受体蛋白或编码RNA降解的实验方法正在积极研究中，但治疗SBMA患者的疗法仍然主要是支持性的[6]。刘……