男,10岁。因发热伴咳喘10余天入院。患儿生后1岁起至今反复感染,曾先后患过中耳炎、鼻窦炎、肺炎、肠炎及败血症。5岁时因长期不规则发热伴膝、踝关节肿胀诊为类风湿性关节炎,经阿斯匹林治疗症状缓解,6岁时发现步态不稳并逐渐出现肢体震颤、吐语不清,近4年来症状进行性加重并出现强直性瘫痪及痴呆。未患过麻疹、水痘,家族中亦无类 Male, 10 years old. Due to fever with cough more than 10 days admitted to hospital. Children from 1 year after birth repeatedly infected, has had otitis media, sinusitis, pneumonia, enteritis and sepsis. 5 years old due to long-term irregular fever with knee, ankle swelling diagnosed as rheumatoid arthritis, aspirin treatment of symptoms, 6 instar instability and gradually appear limb tremor, vomiting unclear, In the past 4 years, the symptoms worsen and appear with tonic paralysis and dementia. No measles, chickenpox, nor clan in the family