Prion and Dementia Research Unit, Institute of Neuropathology, University Medical Center, Göttingen, Germany.
Department of Neurodegenerative Diseases, Hertie Institute for Clinical Brain Research, University Hospital Tübingen, Tübingen, Germany.
Acta Neuropathol Commun. 2021 Nov 24;9(1):187. doi: 10.1186/s40478-021-01286-4.
In sporadic Creutzfeldt-Jakob disease (sCJD), the pathological changes appear to be restricted to the central nervous system. Only involvement of the trigeminal ganglion is widely accepted. The present study systematically examined the involvement of peripheral ganglia in sCJD utilizing the currently most sensitive technique for detecting prions in tissue morphologically. The trigeminal, nodose, stellate, and celiac ganglia, as well as ganglia of the cervical, thoracic and lumbar sympathetic trunk of 40 patients were analyzed with the paraffin-embedded tissue (PET)-blot method. Apart from the trigeminal ganglion, which contained protein aggregates in five of 19 prion type 1 patients, evidence of prion protein aggregation was only found in patients associated with type 2 prions. With the PET-blot, aggregates of prion protein type 2 were found in all trigeminal (17/17), in some nodose (5 of 7) and thoracic (3 of 6) ganglia, as well as in a few celiac (4 of 19) and lumbar (1 of 5) ganglia of sCJD patients. Whereas aggregates of both prion types may spread to dorsal root ganglia, more CNS-distant ganglia seem to be only involved in patients accumulating prion type 2. Whether the prion type association is due to selection by prion type-dependent replication, or due to a prion type-dependent property of axonal spread remains to be resolved in further studies.
在散发性克雅氏病（sCJD）中，病理变化似乎仅限于中枢神经系统。目前广泛接受的是仅累及三叉神经节。本研究利用目前检测组织中朊病毒最敏感的技术，系统地检查了 sCJD 中外周神经节的受累情况。分析了 40 例患者的三叉神经节、结状神经节、星状神经节和腹腔神经节，以及颈、胸、腰交感神经节的神经节，采用石蜡包埋组织（PET）印迹法。除了在 19 例 1 型朊病毒患者中有 5 例含有蛋白聚集物的三叉神经节外，只有在与 2 型朊病毒相关的患者中才发现朊病毒蛋白聚集的证据。使用 PET 印迹法，在所有 17 例 sCJD 患者的三叉神经节（17/17）、部分结状神经节（5/7）和胸神经节（3/6）、以及一些腹腔神经节（4/19）和腰神经节（1/5）中发现了 2 型朊病毒蛋白聚集物。虽然两种朊病毒类型的聚集物可能扩散到背根神经节，但更多的中枢神经系统外神经节似乎仅在积累 2 型朊病毒的患者中受累。这种朊病毒类型的相关性是由于朊病毒类型依赖性复制的选择，还是由于轴突传播的朊病毒类型依赖性特性，仍有待进一步研究解决。
