Takai Keisuke, Endo Toshiki, Seki Toshitaka, Inoue Tomoo
Department of Neurosurgery, Tokyo Metropolitan Neurological Hospital, 2-6-1, Musashidai, Fuchu, Tokyo, 183-0042, Japan.
Department of Neurosurgery, Graduate School of Medicine, Tohoku University, Sendai, Miyagi, Japan.
J Neurol. 2023 Mar;270(3):1745-1753. doi: 10.1007/s00415-022-11536-7. Epub 2022 Dec 19.
The purpose was to clarify diagnostic clues and pitfalls in cranio-cervical junction arteriovenous fistulas (CCJ AVFs) with congestive myelopathy.
In a multicenter observational study by the Neurospinal Society of Japan, we described the demographics, clinical courses, imaging findings, and outcomes of consecutive patients with CCJ AVFs presenting with congestive myelopathy between 2009 and 2019.
Twenty-seven patients were included (mean age, 70 years; male, 96%). Progressive symptoms within one day to one month were more common (63%) than chronic symptoms. Myelopathic symptoms were characterized by ascending paralysis beginning from the legs, involving the trunk and arms, and sometimes ending in the brainstem. Fifteen patients (56%) received a misdiagnosis, including acute transverse myelitis. The most common MRI findings were venous congestive edema of the cervical cord (96%) and the brainstem (63%) and surrounding vascular flow voids (100%). The mean extension of congestive edema was 5.5 ± 2.9 vertebral segments. The most common angiographic findings were a dural AVF (78%) at the C1 level (81%) with descending venous drainage (85%). Seven patients (26%) were administered steroids, which resulted in neurological decline in 3. Neurosurgical obliteration of the AVF led to improvements in MRI findings in 75% and a functional status in 67%; however, 44% remained dependent.
The myelopathy of CCJ AVFs was characterized by acute ascending paralysis in elderly men. A misdiagnosis was common because of the acute presentation due to a longitudinally extensive spinal cord lesion. Dilated vessels on MRI were a key finding for the correct diagnosis. What is already known on this topic? Slowly progressive myelopathy is a well-known symptom that results from impaired spinal venous drainage due to thoracolumbar AVFs. Although cranio-cervical junction arteriovenous fistulas (CCJ AVFs) constitute a treatable cause of congestive myelopathy, detailed information is not currently available due to their rarity. What does this study add? CCJ AVFs often presented with acute ascending myelopathy in elderly men due to a longitudinally extending cervical cord lesion with surrounding flow voids. Steroid pulse therapy was not effective or even harmful to congestive myelopathy, while neurosurgical treatment effectively obliterated AVFs. How might this study affect research, practice or policy? The results obtained revealed diagnostic clues and pitfalls from the largest dataset of patients with CCJ AVFs in a multicenter cohort.
目的是阐明伴有充血性脊髓病的颅颈交界区动静脉瘘（CCJ AVF）的诊断线索和陷阱。
在日本神经脊髓学会的一项多中心观察性研究中，我们描述了2009年至2019年间连续出现充血性脊髓病的CCJ AVF患者的人口统计学特征、临床病程、影像学表现和结局。
纳入27例患者（平均年龄70岁；男性占96%）。1天至1个月内的进行性症状比慢性症状更常见（63%）。脊髓病症状的特点是从腿部开始的上行性麻痹，累及躯干和手臂，有时累及脑干。15例患者（56%）被误诊，包括急性横贯性脊髓炎。最常见的MRI表现是颈髓（96%）和脑干（63%）的静脉充血性水肿以及周围血管流空影（100%）。充血性水肿的平均范围为5.5±2.9个椎体节段。最常见的血管造影表现是C1水平的硬脑膜动静脉瘘（78%），伴有下行性静脉引流（85%）。7例患者（26%）接受了类固醇治疗，其中3例出现神经功能减退。AVF的神经外科闭塞使75%的患者MRI表现改善，67%的患者功能状态改善；然而，44%的患者仍有功能依赖。
CCJ AVF所致脊髓病的特征是老年男性急性上行性麻痹。由于纵向广泛脊髓病变导致的急性表现，误诊很常见。MRI上扩张的血管是正确诊断的关键发现。关于该主题已知的信息有哪些？缓慢进展性脊髓病是胸腰段AVF导致脊髓静脉引流受损引起的一种众所周知的症状。尽管颅颈交界区动静脉瘘（CCJ AVF）是充血性脊髓病的可治疗病因，但由于其罕见性，目前尚无详细信息。本研究补充了哪些内容？CCJ AVF常因纵向延伸的颈髓病变伴周围流空影而在老年男性中表现为急性上行性脊髓病。类固醇脉冲治疗对充血性脊髓病无效甚至有害，而神经外科治疗可有效闭塞AVF。本研究可能如何影响研究、实践或政策？所获得的结果从多中心队列中最大的CCJ AVF患者数据集中揭示了诊断线索和陷阱。
