ABSTRACT
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.