机构：[a]Department of Neurology, Xuanwu Hospital, Capital Medical University, Beijing, China神经内科首都医科大学宣武医院[b]Neurologische Klinik und Poliklinik, Ludwig-Maximilians-Universitat Munchen, Germany[c]Department of Psychiatry, Psychosomatics and Psychotherapy, Center of Mental Health, University Hospital Würzburg, Germany[d]Departments of Pathology and Radiology, University of Sao Paulo Medical School, Sao Paulo, Brazil[e]Department of Neurology, University of California, San Francisco, USA[f]Praca Amadeu Amaral, 27, cj 31, Bela Vista, Sao Paulo, Brazil[g]Department of Radiology, University of Sao Paulo Medical School, Sao Paulo, Brazil[h]Dr. Senckenbergisches Chronomedizinisches Institut, Goethe-Universitat, Frankfurt/Main, Germany[i]Department of Pathology and Medical Biology, University Medical Center Groningen, University of Groningen, Groningen, The Netherlands[j]Center for Neuropathology and Prion Research, Ludwig-Maximilians-University Munich, Germany[k]Department of Neuroanatomy, Ludwig-Maximilians-Universitat Munchen, Germany[l]Clienia Privatklinik fur Psychiatrie und Psychotherapie, Oetwil am See, Switzerland[m]Department of Psychiatry and Psychotherapy, Ludwig-Maximilians-University Munich, Germany
Introduction: Chorea-acanthocytosis (ChAc) and Huntington's disease (HD) are neurodegenerative conditions that share clinical and neuropathological features, despite their distinct genetic etiologies. Methods: In order to compare these neuropathologies, serial gallocyanin-stained brain sections from three subjects with ChAc were analyzed and compared with our previous studies of eight HD cases, in addition to three hemispheres from two male controls. Results: Astrogliosis was much greater in the ChAc striatum, as compared to that found in HD, with dramatic increase in total striatal glia numbers and the number of glia per striatal neuron. Striatal astrocytes are most likely derived from the striatal subependymal layer in ChAc, which showed massive proliferation. The thalamic centromedian-parafascicular complex is reciprocally connected to the striatum and is more heavily affected in HD than in ChAc. Conclusion: The distinct patterns of selective vulnerability and gliosis observed in HD and ChAc challenge simplistic views on the pathogenesis of these two diseases with rather similar clinical signs. The particular roles played by astroglia in ChAc and in HD clearly need to be elucidated in more detail. (C) 2018 Elsevier Ltd. All rights reserved.
基金：
the ERA-net E-Rare consortium EMINA (European Multidisciplinary Initiative on Neuroacanthocytosis;BMBF 01GM1003)
the National Natural Science Foundation of China (Grant No. 81601099);
the Scientific Research Foundation for the Returned Overseas Chinese Scholars, State Education Ministry ([2015] No.1098);
Beijing Talents Fund (2015000026833ZK06);
Beijing Municipal Administration of Hospitals Youth Program (QML20150801);
Clinical-Basic Medicine Cooperation Fund of Capital Medical University (16JL28).
第一作者机构：[a]Department of Neurology, Xuanwu Hospital, Capital Medical University, Beijing, China[b]Neurologische Klinik und Poliklinik, Ludwig-Maximilians-Universitat Munchen, Germany
通讯作者：
通讯机构：[*1]Department of Psychiatry, Psychosomatics and Psychotherapy, Center of Mental Health, University Hospital Würzburg, Germany
推荐引用方式(GB/T 7714)：
Jia Liu,Helmut Heinsen,Lea T. Grinberg,et al.Subcortical neurodegeneration in chorea: Similarities and differences between chorea-acanthocytosis and Huntington's disease[J].PARKINSONISM & RELATED DISORDERS.2018,49:54-59.doi:10.1016/j.parkreldis.2018.01.009.
APA：
Jia Liu,Helmut Heinsen,Lea T. Grinberg,Eduardo Alho,Edson Amaro Jr....&Adrian Danek.(2018).Subcortical neurodegeneration in chorea: Similarities and differences between chorea-acanthocytosis and Huntington's disease.PARKINSONISM & RELATED DISORDERS,49,
MLA：
Jia Liu,et al."Subcortical neurodegeneration in chorea: Similarities and differences between chorea-acanthocytosis and Huntington's disease".PARKINSONISM & RELATED DISORDERS 49.(2018):54-59
