Guentchev M, Hainfellner J A, Trabattoni G R, Budka H
Austrian Reference Center for Human Prion Diseases, University of Vienna, Austria.
J Neuropathol Exp Neurol. 1997 Oct;56(10):1119-24. doi: 10.1097/00005072-199710000-00005.
There is a distinctive pattern of hippocampal involvement in Creutzfeldt-Jakob disease (CJD) and evidence for selective vulnerability of GABAergic neurons in experimental and human prion disease. We studied hippocampus and temporal cortex from human CJD and control autopsy brains and surgical cryptogenic temporal lobe epilepsy specimens for distribution and density of parvalbumin (PV) and calbindin-D28K (Cal) -positive neurons that are subpopulations of GABAergic neurons. Pathology was evaluated semiquantitatively in 8 regions in 23 CJD brains for severity of spongiform change, astrogliosis and pathological prion protein deposition. In CJD, pathology was severe in pre-parasubiculum and temporal cortex, and little or absent in CA1-4; PV+ neurons were severely reduced or absent in all cases, whereas Cal+ neurons were largely preserved. In controls, the density of PV+ neurons was highest in pre-parasubiculum and temporal cortex, and lowest in CA1-4. In cTLE, loss of PV+ neurons was seen only in CA1-4. The diffuse and severe loss of PV+ neurons in CJD, and the topographical correlation of tissue lesioning in CJD with density of PV+ neurons in controls suggest selective vulnerability and early loss of this subset of inhibitory neurons in CJD. This might relate to characteristic CJD symptoms such as myoclonus and the distinctive EEG pattern.
克雅氏病（CJD）存在独特的海马受累模式，并且在实验性和人类朊病毒病中有证据表明γ-氨基丁酸能神经元具有选择性易损性。我们研究了来自人类CJD、对照尸检脑以及手术切除的隐源性颞叶癫痫标本的海马和颞叶皮质，以了解小白蛋白（PV）和钙结合蛋白-D28K（Cal）阳性神经元（γ-氨基丁酸能神经元的亚群）的分布和密度。对23例CJD脑的8个区域进行海绵状改变、星形胶质细胞增生和病理性朊病毒蛋白沉积严重程度的半定量病理学评估。在CJD中，前副海马和颞叶皮质的病理改变严重，而CA1-4区的病理改变轻微或无；所有病例中PV+神经元严重减少或缺失，而Cal+神经元大部分得以保留。在对照中，PV+神经元密度在前副海马和颞叶皮质最高，在CA1-4区最低。在隐源性颞叶癫痫（cTLE）中，仅在CA1-4区观察到PV+神经元丢失。CJD中PV+神经元的弥漫性严重丢失，以及CJD组织损伤的地形学与对照中PV+神经元密度的相关性，提示CJD中这一抑制性神经元亚群具有选择性易损性和早期丢失。这可能与CJD的特征性症状如肌阵挛和独特的脑电图模式有关。
