中华内科杂志
中華內科雜誌
중화내과잡지
CHINESE JOURNAL OF INTERNAL MEDICINE
2015年
1期
31-34
,共4页
朱明伟%刘佳%王鲁宁%桂秋萍
硃明偉%劉佳%王魯寧%桂鞦萍
주명위%류가%왕로저%계추평
运动神经元病%TDP-43%泛素蛋白%包涵体%脑%脊髓
運動神經元病%TDP-43%汎素蛋白%包涵體%腦%脊髓
운동신경원병%TDP-43%범소단백%포함체%뇌%척수
Motor neuron disease%TDP-43%Ubiquitin%Inclusion%Brain%Spinal cord
目的 认识TDP-43蛋白在运动神经元病中枢神经系统的病理性表达特征.方法 对4例经尸检病理证实的运动神经元病的临床及病理组织学特征进行总结;并对其脑和脊髓切片进行泛素、磷酸化TDP-43免疫组化染色,观察其阳性表达情况,并与同年龄、无神经疾病病史的3例病例的脑和脊髓组织进行对照.结果 4例病例具有运动神经元病典型的临床与组织学特征,其脑和脊髓组织中,3例存在神经元泛素阳性包涵体结构,主要表现为丝团样包涵体,致密圆形包涵体及路易样包涵体;2例存在明确的TDP-43阳性表达,表现为广泛分布于大脑运动皮层及脊髓前角的各种形态的神经元胞浆包涵体及胶质细胞包涵体,1例存在脊髓前角神经元TDP43弱阳性表达.对照病例无泛素阳性及TDP-43阳性包涵体发现.结论 运动神经元病患者的中枢神经系统存在广泛TDP-43蛋白阳性表达,且其阳性包涵体具有一定特征性,值得进一步研究其形成机制.
目的 認識TDP-43蛋白在運動神經元病中樞神經繫統的病理性錶達特徵.方法 對4例經尸檢病理證實的運動神經元病的臨床及病理組織學特徵進行總結;併對其腦和脊髓切片進行汎素、燐痠化TDP-43免疫組化染色,觀察其暘性錶達情況,併與同年齡、無神經疾病病史的3例病例的腦和脊髓組織進行對照.結果 4例病例具有運動神經元病典型的臨床與組織學特徵,其腦和脊髓組織中,3例存在神經元汎素暘性包涵體結構,主要錶現為絲糰樣包涵體,緻密圓形包涵體及路易樣包涵體;2例存在明確的TDP-43暘性錶達,錶現為廣汎分佈于大腦運動皮層及脊髓前角的各種形態的神經元胞漿包涵體及膠質細胞包涵體,1例存在脊髓前角神經元TDP43弱暘性錶達.對照病例無汎素暘性及TDP-43暘性包涵體髮現.結論 運動神經元病患者的中樞神經繫統存在廣汎TDP-43蛋白暘性錶達,且其暘性包涵體具有一定特徵性,值得進一步研究其形成機製.
목적 인식TDP-43단백재운동신경원병중추신경계통적병이성표체특정.방법 대4례경시검병리증실적운동신경원병적림상급병리조직학특정진행총결;병대기뇌화척수절편진행범소、린산화TDP-43면역조화염색,관찰기양성표체정황,병여동년령、무신경질병병사적3례병례적뇌화척수조직진행대조.결과 4례병례구유운동신경원병전형적림상여조직학특정,기뇌화척수조직중,3례존재신경원범소양성포함체결구,주요표현위사단양포함체,치밀원형포함체급로역양포함체;2례존재명학적TDP-43양성표체,표현위엄범분포우대뇌운동피층급척수전각적각충형태적신경원포장포함체급효질세포포함체,1례존재척수전각신경원TDP43약양성표체.대조병례무범소양성급TDP-43양성포함체발현.결론 운동신경원병환자적중추신경계통존재엄범TDP-43단백양성표체,차기양성포함체구유일정특정성,치득진일보연구기형성궤제.
Objective To understand pathological TDP-43 features in the central nervous systems of patients with clinically and autopsy confirmed motor neuron disease (MND).Methods The clinical and histopathological features of 4 cases with MND confirmed by autopsy were summarized; anti-ubiquitin (Ub) and anti-TDP-43 immunohistochemical staining were carried out on tissue of brains and spinal cords from 4 cases with MND and 3 control cases without history of neurological disorders.Results These 4 cases presented with typical clinical and histologic features of MND.Ub-positive inclusions were observed in brain and spinal cord from 3 cases with the Ub-positive inclusions of skein-round-and lewy body-like structures.Strong TDP-43 pathological staining in brain and spinal cord was identified in 2 cases with MND presented as neuronal and glial cytoplasmic inclusions with various shapes.The TDP-43 positive inclusions were widely distributed in the motor cortex of brain and the anterior horn of spinal cord.TDP-43 weak staining in the spinal cord tissue was observed in 1 case with MND.No Ub-and TDP-43 positive inclusions were found in 3 control cases.Conclusion There is widespread pathological TDP-43 expression in the central nervous system of MND.TDP-43 positive inclusions in MND have relatively high specificity.It is worth further study on their formation mechanism.
