Magaña S M, Matiello M, Pittock S J, McKeon A, Lennon V A, Rabinstein A A, Shuster E, Kantarci O H, Lucchinetti C F, Weinshenker B G
Department of Neurology, Mayo Clinic College of Medicine, Rochester, MN 55905, USA.
Neurology. 2009 Feb 24;72(8):712-7. doi: 10.1212/01.wnl.0000343001.36493.ae.
Posterior reversible encephalopathy syndrome (PRES) is characterized by vasogenic subcortical edema without infarction. It has been associated with hypertensive crises and with immunosuppressive medications but not with neuromyelitis optica (NMO).
We reviewed the clinical and neuroimaging features of five NMO-immunoglobulin G (IgG) seropositive white women who experienced an episode of PRES and had a coexisting NMO spectrum disorder (NMOSD). We also tested for the aquaporin-4 (AQP4) water channel autoantibody (NMO-IgG) in 14 patients from an independently ascertained cohort of individuals with PRES.
All five patients developed abrupt confusion and depressed consciousness consistent with PRES. The encephalopathy resolved completely within 7 days. Comorbid conditions or interventions recognized to be associated with PRES included orthostatic hypotension with supine hypertension, plasma exchange, IV immunoglobulin treatment, and high-dose IV methylprednisolone. Brain MRI studies revealed bilateral T2-weighted (T2W) hyperintense signal abnormalities, primarily in frontal, parieto-occipital, and cerebellar regions. Three patients had highly symmetric lesions and three had gadolinium-enhancing lesions. Follow-up neuroimaging revealed partial or complete disappearance of T2W hyperintensity or gadolinium-enhancing lesions in all five patients. Patients with PRES without NMOSD were uniformly NMO-IgG seronegative.
Brain lesions in some patients with neuromyelitis optica spectrum disorder (NMOSD) may be accompanied by vasogenic edema and manifest as posterior reversible encephalopathy syndrome (PRES). Water flux impairment due to aquaporin-4 autoimmunity may predispose to PRES in patients with NMOSD who experience blood pressure fluctuations or who are treated with therapies that can cause rapid fluid shifts.
后部可逆性脑病综合征（PRES）的特征是血管源性皮质下水肿且无梗死。它与高血压危象和免疫抑制药物有关，但与视神经脊髓炎（NMO）无关。
我们回顾了5名NMO免疫球蛋白G（IgG）血清阳性的白人女性的临床和神经影像学特征，她们经历了一次PRES发作并同时患有NMO谱系障碍（NMOSD）。我们还对来自一个独立确定的PRES患者队列的14名患者进行了水通道蛋白4（AQP4）水通道自身抗体（NMO-IgG）检测。
所有5名患者均出现与PRES一致的急性意识模糊和意识障碍。脑病在7天内完全缓解。已知与PRES相关的合并症或干预措施包括体位性低血压伴仰卧位高血压、血浆置换、静脉注射免疫球蛋白治疗和大剂量静脉注射甲基强的松龙。脑部MRI研究显示双侧T2加权（T2W）高信号异常，主要位于额叶、顶枕叶和小脑区域。3例患者有高度对称的病变，3例有钆增强病变。随访神经影像学显示所有5例患者的T2W高信号或钆增强病变部分或完全消失。无NMOSD的PRES患者均为NMO-IgG血清阴性。
一些视神经脊髓炎谱系障碍（NMOSD）患者的脑病变可能伴有血管源性水肿，并表现为后部可逆性脑病综合征（PRES）。水通道蛋白4自身免疫导致的水通量受损可能使经历血压波动或接受可导致快速液体转移治疗的NMOSD患者易患PRES。
