Neuropathologic characteristics of brainstem lesions in sporadic Creutzfeldt-Jakob disease
Neuropathologic characteristics of brainstem lesions in sporadic Creutzfeldt-Jakob disease
复制标题
DOI：
10.1007/s00401-005-0981-0
复制
发表时间：
2005-06-01
影响因子：
12.7
通讯作者：
Sobue, G
中科院分区：
文献类型：
--
作者：
Iwasaki, Y;Hashizume, Y;Sobue, G
我们调查了33例散发性克雅氏病（sCJD）患者的脑干是否受到病理过程的影响，特别注意脑干萎缩、神经元丢失、锥体束变性和朊蛋白（PrP）沉积。脑干萎缩，特别是在脑桥底部，是相对突出的疾病异常延长的持续时间的患者。神经元丢失和锥体束变性也被确定在一些但不是所有的长期疾病的患者。脑桥核的神经元丢失相对突出，黑质和下橄榄核的神经元丢失较少;脑干被盖的运动核团保存完好。PrP沉积存在于所有患者的脑干，并确定主要是在黑质，四叠体，脑桥核，下橄榄核。PrP沉积在脑桥和延髓的红核和被盖不太突出。PrP沉积发生在锥体束最少或根本没有。在sCJD脑干中的PrP沉积密度与疾病持续时间或神经元变性无关，直到晚期。我们的研究结果表明，萎缩，神经元丢失，锥体束变性发生在sCJD脑干，特别是在一个异常延长病程的患者。这些发现与PrP沉积没有直接关系，可能反映了终末期sCJD。没有VV 1，VV 2，或MV 2的情况下，包括在我们的研究，但是，我们认为，广泛和相对刻板的PrP沉积是一个一致的病理特征sCJD，至少在MM 1 sCJD患者。虽然PrP在脑干中的积累似乎是sCJD的早期病理事件，并且可能保留到疾病的晚期阶段，但脑干对sCJD的病理过程仍然具有相对抵抗力。
We investigated whether the brainstem is affected by the pathologic process of sporadic Creutzfeldt-Jakob disease (sCJD), with particular attention to brainstem atrophy, neuronal loss, pyramidal tract degeneration, and prion protein (PrP) deposition, in 33 patients with sCJD. Brainstem atrophy, particularly in the pontine base, was relatively prominent in patients with disease of unusually prolonged duration. Neuronal loss and pyramidal tract degeneration were also identified in some but not all patients with prolonged disease. Neuronal loss was relatively prominent in the pontine nucleus and less so in the substantia nigra and inferior olivary nucleus; motor nuclei of the brainstem tegmentum were well preserved. PrP deposition was present in the brainstem in all patients, and was identified predominantly in the substantia nigra, quadrigeminal body, pontine nucleus, and inferior olivary nucleus. PrP deposition was less prominent in the red nucleus and tegmentum of the pons and medulla oblongata. PrP deposition occurred least or not at all in the pyramidal tract. The density of PrP deposition in the sCJD brainstem was not associated with disease duration or neuronal degeneration until the late stage. Our results show that atrophy, neuronal loss, and pyramidal tract degeneration occur in the sCJD brainstem, particularly in patients with an unusually prolonged disease course. These findings are not associated directly with PrP deposition and may reflect end-stage sCJD. No VV1, VV2, or MV2 cases were included in our study; however, we suggest that widespread and relatively stereotypic PrP deposition is a consistent pathologic feature of sCJD, at least in MM1 sCJD patients. Although accumulation of PrP in the brainstem appears to be an early pathologic event in sCJD, and may remain into the late disease stage, the brainstem remains relatively resistant to the pathologic process of sCJD.