Freiesleben W, Söylemezoglu F, Lowe J, Janzer R C, Kleihues P
Department of Pathology, University Hospital, Zürich, Switzerland.
Neuropathol Appl Neurobiol. 1997 Feb;23(1):36-42. doi: 10.1111/j.1365-2990.1997.tb01183.x.
Two cases of Wernicke's encephalopathy with the rare phenomenon of ballooned neurons in the mamillary bodies are reported. Both patients suffered from acute Wernicke's symptoms starting approximately two weeks before death. The mamillary bodies contained grossly enlarged, ballooned neurons, in one case associated with focal necrosis. The affected neurons were immunoreactive for phosphorylated neurofilament (160 and 200 kDa), and synaptophysin. Ubiquitin and alpha beta-crystallin expression were not detected. The mamillo-thalamic tract appeared normal in both cases. There was a marked associated microglial reaction, as shown by the antibody Ki-MIP. It is concluded that the ballooning of mamillary neurons reflects an acute retrograde reaction to primarily axonal damage. Rather than being a rare manifestation of the disease, these cases may constitute a typical intermediate early stage (10-15 days) in the development of Wernicke's encephalopathy).
报告了两例韦尼克脑病,其乳头体中出现了罕见的气球样神经元现象。两名患者均在死亡前约两周开始出现急性韦尼克症状。乳头体中含有明显肿大的气球样神经元,其中一例伴有局灶性坏死。受影响的神经元对磷酸化神经丝(160和200 kDa)和突触素呈免疫反应性。未检测到泛素和αβ-晶状体蛋白表达。两例患者的乳头丘脑束均正常。如Ki-MIP抗体所示,有明显的相关小胶质细胞反应。结论是,乳头体神经元的气球样变反映了对主要轴突损伤的急性逆行反应。这些病例可能构成韦尼克脑病发展过程中典型的中间早期阶段(10 - 15天),而非该疾病的罕见表现。