A clinicopathological study of vascular progressive supranuclear palsy - A multi-infarct disorder presenting as progressive supranuclear palsy
A clinicopathological study of vascular progressive supranuclear palsy - A multi-infarct disorder presenting as progressive supranuclear palsy
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DOI：
10.1001/archneur.59.10.1597
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发表时间：
2002-10-01
影响因子：
--
通讯作者：
Dickson, DW
中科院分区：
文献类型：
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作者：
Josephs, KA;Ishizawa, T;Dickson, DW
工作背景：提示诊断为进行性核上性麻痹（PSP）的临床特征包括早期福尔斯、轴性强直、垂直核上性眼肌麻痹和左旋多巴反应迟钝。当存在这些临床特征时，诊断几乎总是PSP，但血管疾病有时也有类似的表现，称为血管PSP。目的：评估提交给PSP脑库的血管PSP病例的临床和病理特征。设计：审查大体和显微神经病理学特征，确定T单倍型，回顾性分析4例生前诊断为PSP但不符合PSP病理诊断标准的患者的临床资料，并对其中4例患者进行了血管病理学检查。所有患者均有垂直性核上性眼肌麻痹、福尔斯病史和逐渐进展的病程。福尔斯在症状发作后1年开始出现，所有患者的神经系统检查结果均不对称。磁共振成像扫描显示一名患者的腔隙性基底节梗死，另一名患者的放射冠和半卵圆中心T2加权信号增加。大体和显微镜神经病理学研究证实了脑梗死;在大脑皮层（n=4）、丘脑（n=4）、基底节（n=3）和小脑（n=4）中。1例脑干受累，但丘脑底核或黑质未见梗死。4例患者中，3进行H2 tau单倍型，在一般population.Conclusions罕见：不对称的迹象，福尔斯症状发作后1年，血管病变的磁共振成像扫描，和H2 tau单倍型可能有助于区分血管PSP从PSP。丘脑和基底节梗死;在血管性PSP患者中很常见，当存在时，可能导致误诊。
Background: Clinical features suggesting a diagnosis of progressive supranuclear palsy (PSP) include early falls, axial rigidity, vertical supranuclear ophthalmoplegia, and levodopa unresponsiveness. When these clinical features are present, the diagnosis is almost always PSP, yet vascular disease sometimes has a similar presentation, referred to as vascular PSP.Objective: To evaluate clinical and pathologic features of cases of vascular PSP submitted to a PSP brain bank.Design: Review of gross and microscopic neuropathological features, determination Of T haplotype, and medical record review of 4 patients with an antemortem diagnosis of PSP who did not meet the pathologic criteria for PSP and instead had vascular pathologic abnormalities.Results: All patients had vertical supranuclear ophthalmoplegia, a history of falls, and a gradually progressive disease course. Falls began 1 year after symptom onset, and all patients had asymmetric findings on a neurological examination. A magnetic resonance imaging scan revealed lacunar basal ganglia infarcts in one patient and an increased T2-weighted signal in the corona radiata and centrum semiovale in another. Gross and microscopic neuropathological studies demonstrated infarcts; in the cerebral cortex (n=4), thalamus (n=4), basal ganglia (n=3), and cerebellum (n=4). The brainstem was affected in one patient, but no infarcts, were detected in the subthalamic nucleus or substantia nigra. Of the 4 patients, 3 carried an H2 tau haplotype, a rare occurrence in the general population.Conclusions: Asymmetric signs, falls after 1 year of symptom onset, vascular lesions on a magnetic resonance imaging scan, and an H2 tau haplotype may help differentiate vascular PSP from PSP. Thalamic and basal ganglia infarcts; are common in patients with vascular PSP and, when present, may contribute to misdiagnosis.