Clinical and neuropathologic features of progressive supranuclear palsy with severe pallido-nigro-luysial degeneration and axonal dystrophy
Clinical and neuropathologic features of progressive supranuclear palsy with severe pallido-nigro-luysial degeneration and axonal dystrophy
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DOI：
10.1093/brain/awm301
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发表时间：
2008-02-01
期刊：
影响因子：
14.5
通讯作者：
Dickson, Dennis W.
中科院分区：
文献类型：
--
作者：
Ahmed, Zeshan;Josephs, Keith A.;Dickson, Dennis W.
帕利多黑质狼疮萎缩(PNLA)是一种罕见的疾病，在许多情况下具有类似于进行性核上性瘫痪(PSP)的组织病理学特征。在400多例PSP的病理系列中，有8例具有与PNLA相似的特征，包括苍白球、黑质和丘脑底核的严重萎缩和神经元丢失，以及苍白球和黑质中的许多轴突球体。对这8例PSP-PNLA和11例典型PSP患者进行了神经病理学定量指标的比较，并对人口学、临床特征和临床表现的时间进行了评估。PSP-PNLA患者更年轻，病程更长，更多的人最初没有被诊断为PSP；最后，他们在任何主要临床特征上都与PSP没有区别。然而，PSP-PNLA的临床病程与PSP不同，步态异常和书写困难早于PSP，但跌倒、僵硬和吞咽困难晚于PSP。病理上，PSP和PSP-PNLA的病变类型相同，但tau病变的分布和密度不同，PSP-PNLA的运动皮质、纹状体、桥核和小脑的tau病变较少。这些临床和病理结果表明，PSP-PNLA应被认为是PSP的变种。
Pallido-nigro-luysial atrophy ( PNLA) is a rare disorder that in many cases has histopathological features similar to progressive supranuclear palsy ( PSP). In a pathological series of over 400 cases of PSP, eight cases were noted to have features similar to those described in PNLA, including severe atrophy and neuronal loss in the globus pallidus, substantia nigra and subthalamic nucleus, in addition to many axonal spheroids in the globus pallidus and substantia nigra. These eight cases of PSP-PNLA were compared to 11 typical PSP cases with quantitative neuropathologic indices and assessment of demographics, clinical features and the timing of clinical features. PSP-PNLA cases were younger, had longer disease duration and more often were not initially diagnosed with PSP; in the end, they did not differ from PSP with respect to any major clinical feature. The clinical course of PSP-PNLA, however, was different, with earlier gait abnormalities and difficulty with handwriting, but later falls, rigidity and dysphagia than PSP. Pathologically, the same types of lesions were detected in both PSP and PSP-PNLA, but there were differences in the distribution and density of tau-pathology, with less tau-pathology in motor cortex, striatum, pontine nuclei and cerebellum in PSP-PNLA. These clinical and pathological findings suggest that PSP-PNLA should be considered a variant of PSP.