DiSano Michael A, Cerejo Russell, Mays MaryAnn
Department of Child Neurology, Cleveland Clinic, Cleveland, Ohio, USA.
Cerebrovascular Center, Cleveland Clinic, Cleveland, Ohio, USA.
Spinal Cord Ser Cases. 2017 May 18;3:17025. doi: 10.1038/scsandc.2017.25. eCollection 2017.
The underlying causes of longitudinally extensive transverse myelitis (LETM) are broad and include inflammatory processes, compression and spinal dural arteriovenous fistula (SDAVF). Presenting symptoms of SDAVF are nonspecific and often go misdiagnosed. Acute clinical deterioration from SDAVF has been described following exertion or valsalva. However, deterioration has been recently recognized following steroid administration and may contribute to increased morbidity.
We describe a 63-year-old woman with a 2-year history of intermittent lower extremity numbness and back pain, lumbar stenosis, who presented with subacute worsening of symptoms following a course of oral steroids for an upper respiratory infection. Initial whole-spine imaging was concerning for LETM and lumbar puncture was concerning for an inflammatory process. The patient was treated with intravenous (IV) methylprednisolone, after which she developed acute onset bilateral lower extremity paraparesis with a sensory level. Angiogram confirmed the diagnosis of SDAVF and the patient was treated surgically. Post-operative course was complicated and subsequent clinical improvement has been slow with incomplete recovery to date.
This case illustrates the nonspecific presentation of SDAVF and the difficulty of differentiating it from other causes of LETM. It demonstrates acute clinical deterioration of SDAVF following steroid administration, a recently recognized clinical entity. The most likely mechanism is hydrostatic steroid effect coupled with iatrogenic fluid co-administration causing increased venous congestion. Previous cases have demonstrated this effect to be transient and resolves after discontinuation of steroids. This case highlights a recent association of increased morbidity following steroid administration despite definitive treatment.
长节段横贯性脊髓炎(LETM)的潜在病因广泛,包括炎症过程、压迫和脊髓硬脊膜动静脉瘘(SDAVF)。SDAVF的临床表现不具特异性,常被误诊。已有文献报道SDAVF在用力或瓦尔萨尔瓦动作后会出现急性临床恶化。然而,最近发现类固醇给药后也会出现病情恶化,这可能会导致发病率增加。
我们描述了一名63岁女性,有2年间歇性下肢麻木和背痛病史,患有腰椎管狭窄症,因上呼吸道感染接受口服类固醇治疗后症状亚急性加重。最初的全脊柱成像提示LETM,腰椎穿刺提示存在炎症过程。患者接受了静脉注射甲泼尼龙治疗,之后出现急性双侧下肢轻瘫并伴有感觉平面。血管造影证实了SDAVF的诊断,患者接受了手术治疗。术后病程复杂,目前临床恢复缓慢,至今仍未完全康复。
该病例说明了SDAVF的非特异性表现以及将其与LETM的其他病因区分开来的困难。它展示了类固醇给药后SDAVF的急性临床恶化,这是一种最近才被认识到的临床情况。最可能的机制是类固醇的流体静力效应加上医源性液体联合给药导致静脉充血增加。既往病例表明这种效应是短暂的,停用类固醇后可缓解。该病例强调了尽管进行了明确治疗,但类固醇给药后发病率仍会增加这一最新关联。