刘小斌运用麻附细辛汤合甘草干姜汤加减治疗先天性肌无力综合征经验

Experience of LIU Xiao-Bin in Treating Congenital Myasthenic Syndrome with Modified Mafu Xixin Decoction Combined with Gancao Ganjiang Decoction

先天性肌无力综合征(congenital myasthenia syndrome,CMS)为临床罕见的遗传性肌病。CMS患者虽与重症肌无力患者的症状相似,均有肌无力、易疲劳等症,但CMS为先天禀赋不足疾病,以肾元亏损为本,而重症肌无力为后天失养疾病,故在辨治侧重上有所区别。刘小斌教授认为,CMS的中医病机特点为“先天不足,肾元亏损”“脾胃虚损,五脏相关”,属于中医“痿病”范畴;根据患者不易出汗、舌淡红苔白、脉细的病症表现,可辨为脾肾阳虚兼寒邪束表证(少阴太阴兼太阳证);治疗可采用温阳补肾、健脾益损法,常以麻附细辛汤合甘草干姜汤加减,可酌加桑螵蛸、紫河车等益精养血之品,可获显效。

Congenital myasthenic syndrome(CMS)is a rare hereditary myopathy in clinic.Although the symptoms of CMS patients are similar to those of myasthenia gravis(MS)patients in terms of muscular weakness and fatigue,CMS is a disease of congenital insufficiency with deficiency of kidney,while MS is an acquired disease due to loss of nourishment.Therefore,the focus of the diagnosis and treatment varies in CMS and MS.According to the view of Professor LIU Xiao-Bin,the traditional Chinese medicine(TCM)pathogenesis of CMS is characterized by innate insufficiency and deficiency of kidney,deficiency of spleen and stomach,and five-viscera correlation,and CMS can be classified into the category of atrophy-flaccidity syndrome in TCM.CMS patients usually manifest the symptoms of seldom sweating,light reddish tongue,white coating,and fine pulse,which can be identified as the syndrome of spleen and kidney yang deficiency complicated with pathogenic coldness fettering the exterior(also known as shaoyin taiyin complicated with taiyang syndrome).Therefore,the treatment of CMS can be achieved by the therapy of warming yang and tonifying kidney,strengthening spleen and supplementing deficiency,and by the medication of modified Mafu Xixin Decoction(mainly composed of Ephedrae Herba,Aconiti Lateralis Radix Praeparata,Asari Radix et Rhizoma)plus Gancao Ganjiang Decoction(mainly composed of Glycyrrhizae Radix et Rhizoma and Zingiberis Rhizoma Recens),together with Mantidis Oötheca and Hominis Placenta for replenishing essence and nourishing blood.Significant efficacy will be obtained by following the above approach of syndrome differentiation and treatment.