中华神经科杂志
中華神經科雜誌
중화신경과잡지
Chinese Journal of Neurology
2015年
11期
980-983
,共4页
李媛%楚兰%张艺凡%蔡刚%贺电%徐竹%朱英武%况时祥%夏聪
李媛%楚蘭%張藝凡%蔡剛%賀電%徐竹%硃英武%況時祥%夏聰
리원%초란%장예범%채강%하전%서죽%주영무%황시상%하총
重症肌无力%LDL受体相关蛋白质类%自身抗体
重癥肌無力%LDL受體相關蛋白質類%自身抗體
중증기무력%LDL수체상관단백질류%자신항체
Myasthenia gravis%LDL-receptor related proteins%Autoantibodies
目的 检测重症肌无力(MG)患者血清低密度脂蛋白受体相关蛋白4抗体(LRP4-ab)含量,并分析该抗体阳性患者的临床特点.方法 共纳入116例MG确诊患者为病例组,40例其他神经免疫疾病患者及40名健康体检者为对照组,采用细胞分析的方法检测LRP4-ab.结果 (1)病例组中共发现LRP4-ab阳性患者2例(1.7%).所有对照组血清中均未发现LRP4-ab.(2)LRP4-ab阳性MG患者均为同一居住地的年轻女性;分型为Osserman Ⅰ型,且伴有胸腺异常;经乙酰胆碱酯酶抑制剂及小剂量糖皮质激素治疗后均获得稳定缓解.结论 发现2例LRP4-ab阳性的MG患者,提示MG可能存在着另一种发病机制.同时,根据该类患者的免疫学特点采用个体化的治疗方案可能对临床诊疗有着重要意义.
目的 檢測重癥肌無力(MG)患者血清低密度脂蛋白受體相關蛋白4抗體(LRP4-ab)含量,併分析該抗體暘性患者的臨床特點.方法 共納入116例MG確診患者為病例組,40例其他神經免疫疾病患者及40名健康體檢者為對照組,採用細胞分析的方法檢測LRP4-ab.結果 (1)病例組中共髮現LRP4-ab暘性患者2例(1.7%).所有對照組血清中均未髮現LRP4-ab.(2)LRP4-ab暘性MG患者均為同一居住地的年輕女性;分型為Osserman Ⅰ型,且伴有胸腺異常;經乙酰膽堿酯酶抑製劑及小劑量糖皮質激素治療後均穫得穩定緩解.結論 髮現2例LRP4-ab暘性的MG患者,提示MG可能存在著另一種髮病機製.同時,根據該類患者的免疫學特點採用箇體化的治療方案可能對臨床診療有著重要意義.
목적 검측중증기무력(MG)환자혈청저밀도지단백수체상관단백4항체(LRP4-ab)함량,병분석해항체양성환자적림상특점.방법 공납입116례MG학진환자위병례조,40례기타신경면역질병환자급40명건강체검자위대조조,채용세포분석적방법검측LRP4-ab.결과 (1)병례조중공발현LRP4-ab양성환자2례(1.7%).소유대조조혈청중균미발현LRP4-ab.(2)LRP4-ab양성MG환자균위동일거주지적년경녀성;분형위Osserman Ⅰ형,차반유흉선이상;경을선담감지매억제제급소제량당피질격소치료후균획득은정완해.결론 발현2례LRP4-ab양성적MG환자,제시MG가능존재착령일충발병궤제.동시,근거해류환자적면역학특점채용개체화적치료방안가능대림상진료유착중요의의.
Objective To test whether myasthenia gravis (MG) patients have low-density lipoprotein receptor-related protein 4 (LRP4) antibodies in serum, and to evaluate its clinical characteristics.Methods This study included 116 patients with MG, 40 patients with other neurological diseases, and 40 healthy control subjects.For the detection of LRP4 antibody, a cell based assay method was used.Results (1) LRP4-ab was detected in 2 of 116 patients (1.7%) with MG.No LRP4 antibodies were identified in sera from other MG subtypes and control subjects.(2)The clinical characteristics of the 2 LRP4-MG patients were studied.Both of them had same ethnic background and presented thymic changes and isolated ocular symptoms (Osserman Ⅰ).They got full remission after treated with acetylchdinest erase inhibitor and small dose prednisone.Conclusions We found 2 LRP4-ab positive in the sera of MG patients in Guizhou, China.These findings may reminder different pathogenesis in MG patients and give advises to special treatments as different subtypes.