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脊髓硬脊膜动静脉瘘的临床及神经影像学表现:如何避免对此疾病的误诊

Clinical and neuroimaging findings of spinal dural arteriovenous fistulas: How to avoid misdiagnosis of this disease.

作者信息

Takai Keisuke, Taniguchi Makoto

机构信息

Department of Neurosurgery, Tokyo Metropolitan Neurological Hospital, 2-6-1, Musashidai, Fuchu, Tokyo, Japan.

Department of Neurosurgery, Tokyo Metropolitan Neurological Hospital, 2-6-1, Musashidai, Fuchu, Tokyo, Japan.

出版信息

J Orthop Sci. 2019 Nov;24(6):1027-1032. doi: 10.1016/j.jos.2019.07.014. Epub 2019 Aug 13.

Abstract

OBJECTIVE

Spinal dural arteriovenous fistulas (DAVFs) are rare but can cause serious gait and micturition disturbances. Delays in diagnosis and treatment result in poor clinical outcomes; however, the process of misdiagnosis is unknown.

METHODS

Forty consecutive patients were retrospectively analyzed.

RESULTS

Thirty-one patients (78%) were initially misdiagnosed with lumbar spinal stenosis or other diseases, mostly by orthopedic surgeons, even though most patients (85%) had specific symptoms or characteristic neuroimaging findings of spinal DAVFs: they often presented with spastic gait (thoracic myelopathy), progressive ascending numbness that begins in the distal lower extremities (epicous syndrome), and urinary tract symptoms (conus medullaris syndrome); initial lumbar MRI showed T2 signal change in the conus medullaris and vascular flow voids around the cord. The median time from onset to treatment was longer in patients with a misdiagnosis than in those with the correct diagnosis (11 vs 4 months). In all patients, the fistula was completely obliterated by the direct microsurgical procedure; however, patients with a misdiagnosis had developed additional disabilities by the time a correct diagnosis was made (Aminoff-Logue gait grade of 3.6 ± 1.4 vs 2.1 ± 1.5 p = 0.013), and achieved markedly smaller improvements after the treatment (Aminoff-Logue gait grade of 3.0 ± 1.6 vs 1.1 ± 1.5, p = 0.006) than those with the correct diagnosis of spinal DAVFs.

CONCLUSIONS

When common spinal stenosis fail to explain the symptoms such as thoracic myelopathy, epiconus syndrome, and conus medullaris syndrome, the possibility of spinal DAVFs should be considered. If lumbar MRI shows conus medullaris lesions, thoracic MRI should be performed to confirm the diagnosis.

摘要

目的

脊髓硬脊膜动静脉瘘(DAVFs)虽罕见,但可导致严重的步态和排尿障碍。诊断和治疗延误导致临床预后不佳;然而,误诊过程尚不清楚。

方法

对40例连续患者进行回顾性分析。

结果

31例患者(78%)最初被误诊为腰椎管狭窄或其他疾病,大多是由骨科医生误诊,尽管大多数患者(85%)有脊髓DAVFs的特定症状或特征性神经影像学表现:他们常表现为痉挛性步态(胸段脊髓病)、始于下肢远端的进行性上行性麻木(马尾综合征)和尿路症状(圆锥马尾综合征);最初的腰椎MRI显示圆锥马尾T2信号改变及脊髓周围血管流空。误诊患者从发病到治疗的中位时间比正确诊断患者更长(11个月对4个月)。所有患者的瘘均通过直接显微手术完全闭塞;然而,误诊患者在做出正确诊断时已出现额外残疾(阿明诺夫-洛格步态分级为3.6±1.4对2.1±1.5,p = 0.013),且治疗后改善明显小于脊髓DAVFs正确诊断患者(阿明诺夫-洛格步态分级为3.0±1.6对1.1±1.5,p = 0.006)。

结论

当常见的椎管狭窄无法解释胸段脊髓病、马尾圆锥综合征等症状时,应考虑脊髓DAVFs的可能性。如果腰椎MRI显示圆锥马尾病变,应行胸椎MRI以确诊。

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