From the Maxine Mesinger Multiple Sclerosis Comprehensive Care Center (S.A., G.J.H., F.X.C.) and Departments of Neurology and Neurosurgery (O.T.), Baylor College of Medicine, Houston, TX.
Neurology. 2022 Feb 1;98(5):e555-e560. doi: 10.1212/WNL.0000000000013090. Epub 2021 Nov 19.
We present the case of a 57-year-old man with protein S deficiency and left leg deep vein thrombosis (DVT) 5 years earlier, who developed stepwise progressive bilateral lower limb weakness, numbness/paresthesia, gait imbalance, hesitancy of micturition, and constipation in the setting of recurrent left common femoral DVT treated with apixaban. Symptoms amplified with Valsalva, corticosteroids, and postlumbar puncture, with longitudinally extensive midthoracic T2-hyperintense lesion extending to the conus associated with hazy holocord enhancement on magnetic resonance imaging (MRI), raising suspicion for spinal dural arteriovenous fistula (sDAVF). Initial digital subtraction angiography (DSA) was negative for sDAVF. However, cerebral spinal fluid (CSF) was herpes simplex virus (HSV)-2 positive, and he was treated with antiviral therapy. Unfortunately, he continued to worsen despite treatment. Repeat neuroimaging 12 months after initial presentation demonstrated persistent lower thoracic/conus lesion in addition to cauda equina enhancement and subtle dorsal T2-hypointense flow voids. We raised red flags (e.g., lack of clinical prodrome, no herpetic rash, no CSF pleocytosis, and rostral extent of the lesion) that suggested the HSV2 nucleic acid detection was perhaps unrelated to the neurologic syndrome. Given the high index of suspicion for sDAVF, we repeated spinal vascular imaging. Spinal MRA demonstrated dilated right dorsal perimedullary veins from T10 to T11. Repeat DSA revealed a right T10 sDAVF. Microsurgical treatment rather than embolization of the fistula was successful without complication, with significant improvement in motor, sphincter, and to a lesser extent sensory function, with residual gait imbalance after inpatient rehabilitation 3 weeks postoperatively.
我们报告了一例 57 岁男性，5 年前因蛋白 S 缺乏和左下肢深静脉血栓形成（DVT）接受治疗，此后他反复出现左股总静脉 DVT，并出现逐步进展的双侧下肢无力、麻木/感觉异常、步态失衡、排尿犹豫和便秘。这些症状在 Valsalva 动作、皮质类固醇和腰椎穿刺后加重，磁共振成像（MRI）显示长节段中胸 T2 高信号病变延伸至圆锥，伴有脊髓弥漫强化，提示存在脊髓硬脊膜动静脉瘘（sDAVF）。初始数字减影血管造影（DSA）未发现 sDAVF。然而，脑脊液（CSF）单纯疱疹病毒（HSV）2 阳性，他接受了抗病毒治疗。不幸的是，尽管接受了治疗，他的病情仍继续恶化。初次就诊 12 个月后再次进行神经影像学检查，发现除了马尾增强外，还存在胸下部/圆锥病变以及轻微的背部 T2 低信号流空。我们发现了一些警示信号（例如，缺乏临床前驱症状、无疱疹皮疹、CSF 无细胞增多和病变的颅侧范围），提示 HSV2 核酸检测可能与神经系统综合征无关。鉴于 sDAVF 的高度怀疑，我们重复进行了脊髓血管成像。脊髓 MRA 显示 T10 至 T11 右侧背髓旁静脉扩张。重复 DSA 显示右侧 T10 sDAVF。微血管手术治疗而非瘘管栓塞成功，术后 3 周患者接受住院康复治疗后，运动、括约肌功能明显改善，感觉功能也有所改善，但仍存在步态失衡。
