Khot Sandeep, Walker Melanie, Lacy J Matthew, Oakes Patricia, Longstreth W T
Department of Neurology, University of Washington, Seattle, WA 98104, United States.
Neurocrit Care. 2007;7(3):253-6. doi: 10.1007/s12028-007-0044-6.
Delayed posthypoxic demyelination may rarely complicate an episode of severe hypoxia, with or without exposure to carbon monoxide. Following recovery from initial coma, progressive neurologic deterioration ensues with outcomes ranging from death to full recovery. Delayed posthypoxic demyelination is hypothesized to be immunemediated, with support coming from recent animal experiments.
We report a 46-year-old man who developed progressive cognitive deficits with abulia approximately 3 weeks after recovering from coma related to alcohol and morphine intoxication.
Despite treatment with high-dose steroids and plasmapheresis, he continued to deteriorate and remained in a vegetative state until his death under hospice care more than 2 months after his initial hypoxic insult. Serial brain imaging and postmortem examination showed bilateral necrosis of the globi pallidi and extensive demyelination in the centrum semiovale and corona radiata.
Based on an immune-mediated model of disease and given a lack of effective treatments, future use of immunomodulatory therapy may still be worth considering early in the course of this rare and potentially devastating condition.
迟发性缺氧后脱髓鞘可能很少使严重缺氧发作复杂化,无论是否接触一氧化碳。从最初的昏迷中恢复后,会出现进行性神经功能恶化,其结果从死亡到完全恢复不等。迟发性缺氧后脱髓鞘被认为是免疫介导的,这一观点得到了最近动物实验的支持。
我们报告一名46岁男性,在从与酒精和吗啡中毒相关的昏迷中恢复后约3周出现进行性认知缺陷和意志缺失。
尽管接受了高剂量类固醇和血浆置换治疗,但他仍持续恶化,一直处于植物人状态,直到在最初缺氧损伤两个多月后在临终关怀下死亡。系列脑成像和尸检显示双侧苍白球坏死以及半卵圆中心和放射冠广泛脱髓鞘。
基于疾病的免疫介导模型且缺乏有效治疗方法,在这种罕见且可能具有毁灭性的疾病病程早期,未来使用免疫调节疗法可能仍值得考虑。