舞蹈病-棘红细胞增多症的纹状体神经生化研究

Neurochemistry of striatum in chorea-acanthocytosis

目的：探讨舞蹈病-棘红细胞增多症的纹状体神经生化改变。方法从尸检脑标本中，选取4例基因确诊的舞蹈病-棘红细胞增多症，2例基因确诊的亨廷顿病，以及4名正常对照。对其石蜡切片分别行脑啡肽、P物质、谷氨酸脱羧酶和Calbindin-D28k免疫组织化学染色。光镜下观察并使用软件行图像分析。结果壳核和尾状核的背侧部脑啡肽、P物质染色和谷氨酸脱羧酶染色在舞蹈病-棘红细胞增多症患者明显减低；Calbindin-D28k染色明显增加。与正常对照相比，舞蹈病-棘红细胞增多症患者苍白球外侧段脑啡肽[16％（4％）、20％（1％），Mann-Whitney U检验，Z＝－2.337， P＝0.029]、内侧段P物质[12％（3％）、22％（1％），Mann-Whitney U检验，Z＝－2.352， P＝0.029）和谷氨酸脱羧酶染色[25％（11％）、33％（4％），Mann-Whitney U检验，Z＝－2.323， P＝0.029]阳性终末像素单位所占百分比显著减低。结论舞蹈病-棘红细胞增多症患者纹状体P物质表达减低程度比脑啡肽更显著，Calbindin-D28k高表达可能对于神经元损伤具有代偿性的保护作用。

Objective To investigate the striatum neurochemistry in chorea-acanthocytosis（ChAc）.Methods The brain samples of autopsy from 4 ChAc patients , 2 Huntington disease patients and 4 normal controls were collected.Immunostainings of enkephalin, substance P, glutamic acid decarboxylase （GAD） and Calbindin-D28k were carried out.Softwares were used to perform image and statistical analyses.Results In ChAc patients , the stainings of enkephalin , substance P and GAD were decreased , while the staining of Calbindin-D28k was increased.In comparison with normal controls , the staining of encephalin in the external segment of globus pallidus （16%（4%） vs 20%（1%）,Mann-Whitney U test,Z=-2.337, P=0.029） and the stainings of substance P （12%（3%） vs 22%（1%）,Mann-Whitney U test,Z=-2.352, P=0.029 ） and GAD in the internal segment of globus pallidus （ 25% （ 11%） vs 33%（ 4%） , Mann-Whitney U test, Z =-2.323, P =0.029 ） were decreased in ChAc patients.Conclusions In ChAc patients, the decrease of substance P was more obvious than encephalin.The increase of Calbindin-D28k may be a protective compensation for the neuron damage.