Cerebrovascular pathology and misdiagnosis of multiple system atrophy: An autopsy study.
Cerebrovascular pathology and misdiagnosis of multiple system atrophy: An autopsy study.
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DOI：
10.1016/j.parkreldis.2020.05.018
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发表时间：
2020-06
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.;Tipton, Philip W.;Low, Phillip A.;Josephs, Keith A.;Dickson, Dennis W.
多系统萎缩症（MSA）是一种进行性神经退行性疾病，其特征是自主神经功能障碍、帕金森病和小脑性共济失调的组合。其他疾病可以模仿 MSA，但尚不清楚脑血管病理学（所谓的“血管性帕金森病”）是否可以模仿 MSA。本研究旨在确定伪装成 MSA 的血管性帕金森病的临床病理学特征和危险信号。我们利用脑库数据库筛选了 270 名生前诊断为 MSA 的患者，他们没有 MSA 的病理证据，但有脑血管病理，包括白质脑病、腔隙性梗塞和微梗塞。对新皮质、基底神经节、丘脑、脑干和小脑的组织学切片进行了审查。审查医疗记录以表征临床特征。 MSA 临床诊断的概率是在当前共识标准的指导下进行回顾性分配的。四名患者有脑血管病理，但没有神经退行性过程。所有患者的基底节均检测到脑室周围白质慢性缺血性改变、皮质下白质脑病、腔隙性梗塞或微梗塞。尚未发现可能导致自主神经衰竭的脑血管病理学。临床上，两名患者被诊断为可能的 MSA-帕金森病，一名患者可能患有 MSA-帕金森病，一名可能患有 MSA-小脑型；然而，他们还具有一种或多种不支持 MSA 的特征（例如，发病年龄 >75 岁、痴呆）、血管危险因素和其他可能导致自主神经衰竭的病因（例如自主神经病变）。当与脑血管危险因素和合并症相结合时，脑血管病理可能会伪装成 MSA。这项研究的重要教训是，MSA 的诊断需要排除其他原因，包括脑血管疾病。
Multiple system atrophy (MSA) is a progressive neurodegenerative disease characterized by a combination of dysautonomia, parkinsonism, and cerebellar ataxia. Other disorders can mimic MSA, but it is unknown whether cerebrovascular pathology, so-called “vascular parkinsonism,” can mimic MSA. This study aimed to determine the clinicopathological features and red flags for vascular parkinsonism masquerading as MSA. Using a brain bank database, we screened 270 patients with an antemortem diagnosis of MSA, who did not have pathologic evidence of MSA, but rather cerebrovascular pathology, including leukoencephalopathy, lacunar infarcts, and microinfarcts. Histologic sections from the neocortex, basal ganglia, thalamus, brainstem, and cerebellum were reviewed. Medical records were reviewed to characterize the clinical features. The probability of a clinical diagnosis of MSA was assigned retrospectively, guided by current consensus criteria. Four patients had cerebrovascular pathology without neurodegenerative processes. Chronic ischemic changes in periventricular white matter, subcortical leukoencephalopathy, lacunar infarcts, or microinfarcts were detected in basal ganglia of all patients. Cerebrovascular pathology that might contribute to autonomic failure was not identified. Clinically, two patients were diagnosed with possible MSA-parkinsonism, one with probable MSA-parkinsonism, and one with possible MSA-cerebellar type; however, they also had one or more non-supporting features of MSA (e.g., onset >75-year-old, dementia), vascular risk factors, and other etiologies (e.g., autonomic neuropathy) that could cause autonomic failure. When combined with cerebrovascular risk factors and comorbidities, cerebrovascular pathology may masquerade as MSA. The important lesson from this study is that the diagnosis of MSA requires exclusion of other causes, including cerebrovascular disease.
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影响因子：
8.6
作者：
Stankovic, Iva;Quinn, Niall;Wenning, Gregor K.
通讯作者：
Wenning, Gregor K.
影响因子：
4.1
作者：
Koga, Shunsuke;Roemer, Shanu F.;Dickson, Dennis W.
通讯作者：
Dickson, Dennis W.
影响因子：
4.4
作者：
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通讯作者：
LANTOS, PL
影响因子：
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作者：
Benarroch, EE;Schmeichel, AM;Parisi, JE
通讯作者：
Parisi, JE
影响因子：
--
作者：
Josephs, KA;Ishizawa, T;Dickson, DW
通讯作者：
Dickson, DW