Cortical hemichorea-hemiballism
Cortical hemichorea-hemiballism
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DOI：
10.1007/s00415-013-7096-7
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发表时间：
2013-12-01
影响因子：
6
通讯作者：
Kim, Deog Yoon
中科院分区：
文献类型：
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作者：
Hwang, Kyoung Jin;Hong, Il Ki;Kim, Deog Yoon
偏侧舞蹈症-偏侧投掷症（HCHB）很少与皮质病变（如肿瘤或梗死）相关。虽然基底神经节（BG）或丘脑（Th）的功能紊乱的建议，HCHB继发于皮质病变的病理机制仍然不确定。我们招募了继发于脑血管疾病的HCHB患者，排除了其他原因，如高血糖。所有患者均行脑磁共振成像/血管造影（MRI/MRA）和单光子发射计算机断层扫描（SPECT）检查。在神经影像学检查中仅出现皮质异常者被归类为皮质HCHB病例。应用统计参数图（SPM）分析皮质HCHB的病理机制。我们的研究包括10名患者（3名男性和7名女性）。6例患者有急性BG病变伴SPECT异常，1例患者有陈旧性BG病变伴SPECT异常。三名患者被归类为皮质HCHB的病变仅在额叶和顶叶皮质的MRI和SPECT。SPM分析显示额外的低灌注额区，离开BG和Th的任何灌注异常。虽然皮质HCHB由MRI和SPECT严格定义，但皮质HCHB并不少见（30%）。进一步的分析显示，额叶和顶叶之间的交织网络皮质HCHB。皮质功能障碍是重要的皮质HCHB的发病机制，即使没有显着参与BG和Th。
Hemichorea-hemiballism (HCHB) was infrequently related to cortical lesions such as tumor or infarction. Although functional derangement of the basal ganglia (BG) or the thalamus (Th) was suggested, pathomechanism of HCHB secondary to cortical lesions remains uncertain. We recruited the patients with HCHB secondary to cerebrovascular diseases, excluding other causes such as hyperglycemia. All the patients were studied with brain magnetic resonance imaging/angiography (MRI/MRA) and single-photon emission computed tomography (SPECT). Those with only cortical abnormalities in neuroimaging studies were sorted out as the cases of cortical HCHB. Statistical parametric mapping (SPM) analysis of SPECT was performed to investigate the pathomechanism of cortical HCHB. Ten patients (three males and seven females) were included in our study. Six patients had acute BG lesions with SPECT abnormalities, and one had old BG lesions with abnormal SPECT. Three patients were classified as cortical HCHB with lesions only in the frontal and parietal cortices in MRI and SPECT. SPM analysis revealed additional hypoperfusion in frontal areas, leaving BG and Th free of any perfusion abnormalities. Although cortical HCHB was strictly defined by MRI and SPECT, cortical HCHB was not uncommon (30 %). Further analysis showed intertwined networks among the frontal and parietal lobes for cortical HCHB. Cortical dysfunction is important in the pathogenesis of cortical HCHB even without significant involvement of BG and Th.