Szpak Grazyna M, Lewandowska Eliza, Lechowicz Waldemar, Wierzba-Bobrowicz Teresa, Kulczycki Jerzy, Bertrand Ewa, Pasennik Elzbieta, Dymecki Jerzy
Department of Neuropathology, Institute of Psychiatry and Neurology, Warsaw, Poland.
Folia Neuropathol. 2006;44(3):202-13.
A study of microglial activation and its contribution to the CNS immune response was performed on the brain autopsy material of 40 patients with definite sporadic Creutzfeldt-Jakob disease (sCJD). Spatial patterns of microglial activation and prion protein disease-associated (PrPd) deposition were compared in cerebellar and cerebral cortices using immunohistochemical (IHC) activation markers. Morphological phenotype forms of microglial cells in activation stages were assessed immunohistochemically (IHC). The immune inflammatory response dominated by microglia was found to be a characteristic feature in CJD. Differences in the intensity and patterns of microglial activation corresponded to variable patterns of PrP deposition, whereas the morphological phenotype forms of microglia were specific for activation stages. The presence of activated microglial cells in the various activation stages regardless of illness duration indicates continuous microglial activity and microglial contribution to the spread of infection for the whole symptomatic period of the disease. Remarkable vacuolar degeneration changes of numerous microglial cells in different activation stages including homing stage may suggest dysfunction of microglial immune surveillance in human sCJD that can significantly contribute to transmissible spongiform encephalopathy (TSE) pathogenesis.
对40例确诊的散发性克雅氏病（sCJD）患者的脑尸检材料进行了小胶质细胞激活及其对中枢神经系统免疫反应贡献的研究。使用免疫组织化学（IHC）激活标记物，比较了小脑和大脑皮质中小胶质细胞激活和朊病毒蛋白病相关（PrPd）沉积的空间模式。通过免疫组织化学（IHC）评估激活阶段小胶质细胞的形态表型形式。发现以小胶质细胞为主导的免疫炎症反应是克雅氏病的一个特征。小胶质细胞激活的强度和模式差异与PrP沉积的不同模式相对应，而小胶质细胞的形态表型形式在激活阶段具有特异性。无论病程长短，不同激活阶段均存在活化的小胶质细胞，这表明在疾病的整个症状期，小胶质细胞持续活跃并对感染传播有贡献。在包括归巢阶段在内的不同激活阶段，大量小胶质细胞出现明显的空泡变性变化，这可能提示人类sCJD中小胶质细胞免疫监视功能障碍，这可能对传染性海绵状脑病（TSE）的发病机制有显著影响。
