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本期首先报告了称为福山型先天肌萎缩症的同胞病例。兄为典型的福山型肌萎缩,弟呈Duchenne 型肌萎缩之临床经过,兄15岁5个月,自生后6个月起出现婴儿肌弛缓症状,智能障碍,有痉挛发作,全身关节挛缩,磷酸肌酸激酶中度升高,诊为福山型先天性肌萎缩症。弟8岁5个月,发育尚可。从3岁起走路摇摆,攀登起立,磷酸肌酸激酶极度升高,下肢假肥大,智能发育高度迟延,CT 扫描及脑电图均异常。两者中枢病变无差异,故作者认为弟系良性福山型肌萎缩。木下真男等报告1例先天性多发肌炎。本例最初诊为福山型先天性肌萎缩症,肌活检发现有以细小血管为中心的炎症性肌病改变,投与肾上腺皮质类固醇,血清磷酸肌酸激酶显著下降,临床症状缓慢好转,经4年多观察,确
This issue first reports a case of sibling known as Fukuyama-type congenital muscular atrophy. Brother is a typical Fukuyama type muscle atrophy, brother was Duchenne type atrophy clinical experience, brother 15 years and 5 months, 6 months after birth, baby muscle relaxation symptoms, mental retardation, spasms, systemic contracture, phosphoric acid Moderate creatine kinase increased, diagnosed with Fukuyama-type congenital muscular atrophy. Brother 8 years old 5 months, development is acceptable. From the age of 3 walked swinging, climbing up, extremely elevated phosphocreatine kinase, lower limb false hypertrophy, a high degree of delayed development, CT scan and EEG abnormalities. There is no difference between the two central lesion, so the author believes that Brother Department of Fukuyama Muscular atrophy. Kinoshita Mina and other reports of 1 case of congenital polymyositis. This case was initially diagnosed as Fukuyama-type congenital muscular atrophy, muscle biopsy found in small vessels as the center of inflammatory myopathy change, adrenal corticosteroid administration, serum creatine phosphokinase decreased significantly, clinical symptoms improved slowly, after 4 years More observation, indeed