Iwasaki Yasushi, Hashizume Yoshio, Yoshida Mari, Kitamoto Tetsuyuki, Sobue Gen
Department of Neurology, Nagoya University Graduate School of Medicine, 65 Tsurumai-cho, Showa-ku, 466-8550, Nagoya, Japan.
Acta Neuropathol. 2005 Jun;109(6):557-66. doi: 10.1007/s00401-005-0981-0. Epub 2005 Jun 3.
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.
我们研究了33例散发性克雅氏病（sCJD）患者的脑干是否受该病病理过程影响，尤其关注脑干萎缩、神经元丢失、锥体束变性和朊蛋白（PrP）沉积情况。脑干萎缩，特别是脑桥基底部萎缩，在病程异常延长的患者中相对突出。在部分但并非所有病程延长的患者中也发现了神经元丢失和锥体束变性。神经元丢失在脑桥核相对突出，在黑质和下橄榄核则较轻；脑干被盖部的运动核保存良好。所有患者的脑干均有PrP沉积，主要见于黑质、四叠体、脑桥核和下橄榄核。红核以及脑桥和延髓被盖部的PrP沉积较少。锥体束中PrP沉积最少或无沉积。sCJD脑干中PrP沉积密度在疾病晚期之前与病程或神经元变性无关。我们的结果表明，sCJD患者脑干会出现萎缩、神经元丢失和锥体束变性，特别是在病程异常延长的患者中。这些发现与PrP沉积无直接关联，可能反映了sCJD终末期情况。我们的研究未纳入VV1、VV2或MV2病例；然而，我们认为广泛且相对刻板的PrP沉积是sCJD的一致病理特征，至少在MM1型sCJD患者中如此。虽然PrP在脑干中的积聚似乎是sCJD的早期病理事件，且可能持续至疾病晚期，但脑干对sCJD的病理过程仍具有相对抗性。
