Rizzuto N, Ferrari G, Piscioli A
Acta Neuropathol. 1980;50(3):237-40. doi: 10.1007/BF00688761.
A 20-year-old woman was admitted to the Hospital because of tetraparesis and respiratory failure, requiring mechanical ventilation. No disturbances of consciousness were associated. She had a residual left lower limb paresis from the age of 14, lasting unchanged until this final episode of bulbar paralysis. She did not recover: death occurred 3 months later. Neuropathological examination revealed Rosenthal fibers in the gray and white matter with typical perivascular and subpial distribution. Areas of mild demyelination were found in the periventricular white matter of the centrum ovale and in the hilum of the nucleus dentatus. In the brain stem the highest density of Rosenthal fibers was found in the medulla where the deposits extended deeply into parenchyma. The relationship of this case with Alexander's disease is discussed.
一名20岁女性因四肢瘫痪和呼吸衰竭入院，需要机械通气。意识无紊乱。她从14岁起就有左下肢残留麻痹，一直未变，直至此次延髓麻痹的最终发作。她未能康复，3个月后死亡。神经病理学检查显示，在灰质和白质中有罗森塔尔纤维，呈典型的血管周围和软膜下分布。在卵圆中心的脑室周围白质和齿状核门发现轻度脱髓鞘区域。在脑干中，延髓的罗森塔尔纤维密度最高，沉积物深入实质。讨论了该病例与亚历山大病的关系。
