Graffeo Christopher S, Dawson Elliot T, Murphy Meghan E, Black David F, Parisi Joseph E, Crum Brian A, Meyer Fredric B
Department of Neurologic Surgery, Mayo Clinic, 200 First Street Southeast, Rochester, MN 55905, USA.
Department of Neurology, Mayo Clinic, Rochester, MN, USA.
J Clin Neurosci. 2016 Jan;23:8-13. doi: 10.1016/j.jocn.2015.06.016. Epub 2015 Aug 24.
We present a patient with subacute diencephalic angioencephalopathy (SDAE), a poorly understood syndrome of progressive confusion, disorientation, and dementia that rapidly deteriorates to severe encephalopathy and death. This is the seventh and most thoroughly investigated report of this exceedingly rare diagnosis to date, and we present the first evidence of a potential sentinel syndrome that may allow for earlier diagnosis and therapeutic intervention. We also review the relevant literature, and highlight new evidence that suggests that SDAE and the related disorder, subacute brainstem angioencephalopathy (SBAE), represent severe and terminal variants of posterior reversible encephalopathy syndrome (PRES). Taken together, we suggest a new understanding of SDAE/SBAE and PRES as a spectrum of hypertension-associated non-inflammatory vascular encephalopathies that are mediated by a loss of venous autoregulation, marked by cytotoxic edema, and that ultimately produce subacute-to-acute diencephalic, brainstem, and posterior fossa injuries with devastating neurologic consequences.
我们报告了一例患有亚急性间脑血管性脑病（SDAE）的患者，这是一种鲜为人知的综合征，表现为进行性意识模糊、定向障碍和痴呆，迅速恶化为严重脑病并导致死亡。这是迄今为止关于这一极其罕见诊断的第七份也是研究最深入的报告，我们提供了首个可能的哨兵综合征的证据，这可能有助于早期诊断和治疗干预。我们还回顾了相关文献，并强调了新的证据，这些证据表明SDAE和相关疾病——亚急性脑干血管性脑病（SBAE），代表了后部可逆性脑病综合征（PRES）的严重和终末期变体。综合来看，我们提出了对SDAE/SBAE和PRES的新认识，即它们是一系列与高血压相关的非炎性血管性脑病，由静脉自动调节功能丧失介导，以细胞毒性水肿为特征，最终导致亚急性至急性间脑、脑干和后颅窝损伤，并产生毁灭性的神经后果。
