Cerebrovascular pathology presenting as corticobasal syndrome: An autopsy case series of "vascular CBS"
Cerebrovascular pathology presenting as corticobasal syndrome: An autopsy case series of "vascular CBS"
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DOI：
10.1016/j.parkreldis.2019.09.001
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发表时间：
2019-11-01
影响因子：
4.1
通讯作者：
Dickson, Dennis W.
中科院分区：
文献类型：
--
作者：
Koga, Shunsuke;Roemer, Shanu F.;Dickson, Dennis W.
背景：就死后神经病理学而言，皮质基底综合征（CBS）是异质的。虽然之前已经通过死前神经影像学进行了研究，但皮质基底综合征与脑血管病理（血管性CBS）相关的临床病理特征尚未报道。方法：为了识别血管性CBS，我们在CurePSP脑库的数据库中搜索临床诊断为CBS的患者，这些患者不符合皮质基底变性（CBD）或其他神经退行性疾病过程的神经病理学标准，但有明显的脑血管病理。对半脑进行宏观评估，并对其进行组织学评估。回顾了医疗记录，以确定血管性CBS的临床特征。结果：在217例死前诊断为CBS的患者中，我们确定了3例血管性CBS。2例患者在分水岭区（额叶和运动皮层）、脑室周围白质、丘脑和基底节区观察到多发梗死。1例患者无皮质梗死，但有多发白质梗死和皮质脊髓束变性。基于进行性不对称运动症状，包括强直和失用症，以及认知障碍，所有患者在临床上都被认为患有CBS。死前影像学检查显示慢性脑血管疾病，伴有梗死或白质病理。结论：血管性CBS的尸检研究显示，虽然罕见，但涉及额叶、白质束、基底节区、丘脑和皮质脊髓束的脑血管病理可能是提示CBS的临床特征的基础。当神经影像学提示另一种解释，包括关键区域的慢性梗死时，考虑CBD作为潜在病理是值得谨慎的。
Background: The corticobasal syndrome (CBS) is heterogeneous in terms of postmortem neuropathology. While it has been previously studied with antemortem neuroimaging, clinicopathologic features of corticobasal syndrome associated with cerebrovascular pathology (vascular CBS) have yet to be reported.Methods: To identify vascular CBS, we searched the database of the CurePSP Brain Bank for patients with a clinical diagnosis of CBS who failed to meet neuropathologic criteria for corticobasal degeneration (CBD) or other neurodegenerative disease processes, but who had significant cerebrovascular pathology. Hemibrains were assessed macroscopically and processed for histological assessment. Medical records were reviewed to characterize clinical features of vascular CBS.Results: Of 217 patients with an antemortem diagnosis of CBS, we identified three patients with vascular CBS. Multiple infarcts in the watershed regions (frontal lobe and motor cortex), periventricular white matter, thalamus, and basal ganglia were observed in two patients. One patient had no cortical infarcts, but had multiple white matter infarcts and corticospinal tract degeneration. All were clinically thought to have CBS based on progressive asymmetric motor symptoms, including rigidity and apraxia, as well as cognitive impairment. Antemortem imaging studies showed findings of chronic cerebrovascular disease, with infarcts or white matter pathology.Conclusions: This autopsy study of vascular CBS shows that, while rare, cerebrovascular pathology involving the frontal lobe, white matter tracts, basal ganglia, thalamus, and corticospinal tract can underlie clinical features suggestive of CBS. When neuroimaging suggests an alternative explanation, including chronic infarcts in critical regions, caution is merited in considering CBD as the underlying pathology.