Department of Neurology, The Walton Centre NHS Foundation Trust, Liverpool, UK.
Faculty of Health and Life Sciences, The University of Liverpool, Liverpool, UK.
J Neurol. 2021 Dec;268(12):4680-4686. doi: 10.1007/s00415-021-10571-0. Epub 2021 Apr 26.
To describe the diagnostic features of intracranial dural arteriovenous fistulae (DAVF) presenting with cervical cord or brainstem swelling.
Retrospective case note and neuroimaging review of patients with angiographically confirmed DAVF diagnosed during January 2015-June 2020 at a tertiary neuroscience centre (Walton Centre NHS Foundation Trust, Liverpool, UK).
Six intracranial DAVF causing cervical cord or brainstem oedema (all males aged 60-69 years) and 27 spinal DAVF (88% thoracolumbar) were detected over a 5.5-year period. Significantly more patients with intracranial DAVF received steroids for presumed inflammatory myelitis than those with spinal DAVF (5/6 vs 1/27, p = 0.0001, Fisher's exact test). Several factors misled the treating clinicians: atypical rostral location of cord oedema (6/6); acute clinical deterioration (4/6); absence (3/6) or failure to recognise (3/6) subtle dilated perimedullary veins on MRI; intramedullary gadolinium enhancement (2/6); and elevated CSF protein (4/5). Acute deterioration followed steroid treatment in 4/5 patients. The following features may suggest DAVF rather than myelitis: older male patients (6/6), symptomatic progression over 4 or more weeks (6/6) and acellular CSF (5/5).
Intracranial DAVF are uncommon but often misdiagnosed and treated as myelitis, which can cause life-threatening deterioration. Neurologists must recognise suggestive features and consider angiography, especially in older male patients. Dilated perimedullary veins are an important clue to underlying DAVF, but may be invisible or easily missed on routine MRI sequences.
描述以颈髓或脑干肿胀为表现的颅内硬脑膜动静脉瘘（DAVF）的诊断特征。
回顾性分析 2015 年 1 月至 2020 年 6 月在英国利物浦沃尔顿中心 NHS 基金会信托基金会的三级神经科学中心经血管造影确诊的 DAVF 患者的病历和神经影像学资料。
在 5.5 年期间，共发现 6 例颅内 DAVF 引起颈髓或脑干水肿（均为 60-69 岁男性）和 27 例脊髓 DAVF（88%为胸腰椎）。与脊髓 DAVF 相比，颅内 DAVF 患者更有可能因疑似炎性脊髓炎而接受类固醇治疗（5/6 比 1/27，p=0.0001，Fisher 确切检验）。有几个因素误导了治疗医生：脊髓水肿的颅端位置不典型（6/6）；急性临床恶化（4/6）；MRI 上未发现（3/6）或未能识别（3/6）细微扩张的髓周静脉；脊髓内钆增强（2/6）；和 CSF 蛋白升高（4/5）。4/5 例患者在类固醇治疗后病情恶化。以下特征可能提示 DAVF 而非脊髓炎：老年男性患者（6/6），症状进展超过 4 周或更长时间（6/6），CSF 无细胞（5/5）。
颅内 DAVF 虽不常见，但常被误诊和误诊为脊髓炎，这可能导致危及生命的恶化。神经科医生必须识别出提示性特征，并考虑进行血管造影检查，尤其是在老年男性患者中。扩张的髓周静脉是潜在 DAVF 的重要线索，但在常规 MRI 序列上可能看不见或容易漏诊。
