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.

von Economo neuron (VEN) is a bipolar neuron characterized by a large spindle-shaped soma. VEN is generally distributed in the layer V of anterior insular lobe and anterior cingulate cortex. Fork neuron is another featured bipolar neuron. In recent years,many studies have illustrated that VEN and fork neurons are correlated with complicated cognition such as self-consciousness and social emotion. Studies in the development and morpholigies of these two neurons as well as their pathological changes in various neurological and psychiatric disorders have found that the abnormal number and functions of VEN can cause corresponding dysfunctions in social recognition and emotions both during the neuro-developmental stages of childhood and during the nerve degeneration in old age stage. Therefore, more attentions should be paid on the research of VEN and fork neurons in neuropsychiatric diseases.
Central Nervous System Diseases ; pathology ; Cerebral Cortex ; Humans ; Mental Disorders ; Neurons