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一例尸检病例的神经病理学发现,该病例显示出与包括他克莫司在内的预处理相关的后部可逆性脑病综合征样神经影像学表现,预处理用于自体外周血干细胞移植。

Neuropathological findings from an autopsied case showing posterior reversible encephalopathy syndrome-like neuroradiological findings associated with premedication including tacrolimus for autologous peripheral blood stem cell transplantation.

作者信息

Hayashi Yuichi, Kimura Akio, Nakamura Hiroshi, Mimuro Maya, Iwasaki Yasushi, Hara Akira, Yoshida Mari, Inuzuka Takashi

机构信息

Department of Neurology and Geriatrics, Gifu University Graduate School of Medicine, Gifu 501-1194, Japan.

Department of Neurology and Geriatrics, Gifu University Graduate School of Medicine, Gifu 501-1194, Japan.

出版信息

J Neurol Sci. 2017 Apr 15;375:382-387. doi: 10.1016/j.jns.2017.02.030. Epub 2017 Feb 14.

Abstract

Posterior reversible encephalopathy syndrome (PRES) is diagnosed based on neuroradiological findings. Typically, PRES is reversible and presents with a good outcome; however, fatal outcomes have been reported. We report an autopsied case showing PRES-like neuroradiological findings associated with premedication including tacrolimus for autologous peripheral blood stem cell transplantation in a 28-year-old woman with a 2-year history of acute myeloid sarcoma/acute myeloid leukemia. Neurological examination revealed disturbed consciousness, muscle weakness in all extremities, and bilaterally diminished tendon reflexes. Brain fluid attenuated inversion recovery MRI showed multiple bilateral hyper-intensity areas in the posterior white matter and left corona radiate. She died of respiratory arrest within 24h after PRES diagnosis. Neuropathological examination revealed diffuse cerebral edema, multiple cerebral hematomas that extended into the lateral ventricles and subarachnoid cavities, and multiple microbleeds predominantly in the inferior surface of the occipital white matter. Microscopic findings revealed paler myelin sheaths, enlargement of the vascular endothelium, leakage of plasma components and red blood cells, and clasmatodendrosis within the occipital white matter. Cerebral herniation and diffuse cerebral edema due to vascular endothelial dysfunction were concluded to be the cause of death. These pathological findings may aid in the pathophysiological recognition of acute-stage PRES.

摘要

后部可逆性脑病综合征(PRES)是根据神经放射学检查结果来诊断的。通常情况下,PRES是可逆的,预后良好;然而,也有致命结局的报道。我们报告一例尸检病例,该病例显示出类似PRES的神经放射学表现,与预处理用药有关,包括一名28岁患有急性髓系肉瘤/急性髓系白血病2年的女性在进行自体外周血干细胞移植时使用了他克莫司。神经系统检查发现意识障碍、四肢肌肉无力以及双侧腱反射减弱。脑脊液衰减反转恢复序列磁共振成像显示双侧后部白质和左侧放射冠有多个高信号区。她在PRES诊断后24小时内死于呼吸骤停。神经病理学检查发现弥漫性脑水肿、多个脑血肿延伸至侧脑室和蛛网膜下腔,以及多个微出血灶,主要位于枕叶白质下表面。显微镜检查发现枕叶白质内髓鞘颜色变淡、血管内皮细胞肿大、血浆成分和红细胞渗漏以及轴索断裂。血管内皮功能障碍导致的脑疝形成和弥漫性脑水肿被认为是死亡原因。这些病理发现可能有助于对急性期PRES的病理生理认识。

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