Rain Silvia, Udding Jan, Broere Daniel
Department of Neurology, Westfriesgasthuis Hospital, Hoorn, The Netherlands.
Department of Radiology, Westfriesgasthuis Hospital, Hoorn, The Netherlands.
Case Rep Neurol. 2016 Nov 14;8(3):234-242. doi: 10.1159/000452830. eCollection 2016 Sep-Dec.
Subdural arteriovenous fistula (SDAVF) is a rare condition characterized by clinical manifestations ranging from mild bilateral sensory deficits to quadriplegia. The diagnosis is often delayed due to unspecific neurological symptoms, initially diagnosed as polyneuropathy or myelopathy. The diagnosis can be delayed for as long as 1-15 years. The following report describes a cervical SDAVF case initially misdiagnosed as myelitis transversa and treated with intravenous steroids. A 56-year-old male presented with sensory deficits and mild leg and right arm weakness. Cervical MRI showed a central medullary hyperintense lesion with contrast enhancement. After metabolic, infectious, and malignant causes were excluded, myelitis transversa was presumed and the patient was treated intravenously with methylprednisolone. Shortly after that, he developed quadriplegia. Cervical MRI imaging showed engorged cervical perimedullary vessels, which were not visible on the initial MRI. The diagnosis was revised and a SDAVF identified. Prompt surgical treatment led to a complete recovery. The effect of intravenous steroids in SDAVF is controversial. Acute clinical worsening after steroid administration is previously reported in several publications; however, due to the paucity of clinical studies on SDAVF, this effect remains mostly overlooked or unknown. The findings in this patient support the causative relation between SDAVF clinical worsening and steroid administration. We propose that acute clinical worsening under steroids in patients initially diagnosed with myelitis should raise suspicion of an SDAVF.
硬脑膜下动静脉瘘(SDAVF)是一种罕见疾病,其临床表现从轻度双侧感觉障碍到四肢瘫痪不等。由于神经症状不具特异性,诊断往往延迟,最初常被诊断为多发性神经病或脊髓病。诊断延迟时间可达1至15年。以下报告描述了一例最初被误诊为横贯性脊髓炎并接受静脉注射类固醇治疗的颈段SDAVF病例。一名56岁男性出现感觉障碍以及轻度腿部和右臂无力。颈椎磁共振成像(MRI)显示中央髓质高信号病变并有强化。在排除代谢、感染和恶性病因后,推测为横贯性脊髓炎,患者接受了甲泼尼龙静脉注射治疗。此后不久,他发展为四肢瘫痪。颈椎MRI成像显示颈髓周围血管充血,这在最初的MRI上未见。诊断被修正,确诊为SDAVF。及时的手术治疗导致完全康复。静脉注射类固醇在SDAVF中的作用存在争议。此前有几篇文献报道了类固醇给药后出现急性临床恶化的情况;然而,由于关于SDAVF的临床研究较少,这种影响大多仍被忽视或不为人知。该患者的发现支持了SDAVF临床恶化与类固醇给药之间的因果关系。我们建议,最初诊断为脊髓炎的患者在使用类固醇时出现急性临床恶化应怀疑为SDAVF。