Pyramidal tract degeneration in sporadic Creutzfeldt-Jakob disease
Pyramidal tract degeneration in sporadic Creutzfeldt-Jakob disease
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DOI：
10.1111/j.1440-1789.2007.00812.x
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发表时间：
2007-10-01
期刊：
影响因子：
2.3
通讯作者：
Sobue, Gen
中科院分区：
文献类型：
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作者：
Iwasaki, Yasushi;Yoshida, Mari;Sobue, Gen
我们研究了27例散发性克雅病（sCJD）锥体束变性的神经病理学特征。本研究包括13例亚急性海绵状脑病（SSE），13例全脑病型（PE型）sCJD和1例丘脑型sCJD。病程超过13个月的PE型sCJD病例中观察到锥体束变性。SSE型、病程较短的PE型和丘脑型sCJD均无锥体束变性。所有锥体束变性病例均表现为严重的大脑新皮质受累，伴有广泛的大脑白色变性。在这些锥体束病变中观察到髓鞘和轴突的丢失、巨噬细胞浸润和神经胶质增生，这与大脑新皮质受累引起的继发性锥体束变性一致。有趣的是，一些锥体束变性的病例显示了远端优势的病理学，我们认为这表明锥体束的退行性变性或“回退”现象。我们的研究结果表明，锥体束变性是一个迟发的病理事件sCJD。研究锥体束变性可能有助于阐明sCJD白色病变，特别是PE型sCJD的发病机制。
We investigated the neuropathologic features of pyramidal tract degeneration in 27 cases of sporadic Creutzfeldt-Jakob disease (sCJD). The present study included 13 cases of subacute spongiform encephalopathy (SSE), 13 cases of panencephalopathic-type (PE-type) sCJD and one case of thalamic-type sCJD. Pyramidal tract degeneration was observed in PE-type sCJD cases with disease duration of more than 13 months. Cases of SSE, PE-type sCJD of relatively short duration and thalamic-type sCJD showed no pyramidal tract degeneration. All cases with pyramidal tract degeneration showed severe cerebral neocortical involvement with widespread cerebral white matter degeneration. The loss of myelin and axons and macrophage infiltration and fibrillary gliosis seen in these pyramidal tract lesions is consistent with secondary tract degeneration due to cerebral neocortical involvement. Interestingly, some cases with pyramidal tract degeneration showed a distal-dominant pathology that we believe indicates retrograde degeneration or a "dying-back" phenomenon of the pyramidal tract. Our results indicate that pyramidal tract degeneration is a late pathologic event in sCJD. Investigation of pyramidal tract degeneration may help clarify the mechanisms underlying the development of sCJD white matter pathology, particularly that of PE-type sCJD.