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横贯性脊髓炎的严重模仿者——自发性非创伤性脊髓内出血性脊髓炎:一例报告

Severe Mimic of Transverse Myelitis - Spontaneous Non-Traumatic Intramedullary Hematomyelia: A Case Report.

作者信息

Simon Devin

机构信息

Department of Neurology and Ophthalmology, Michigan State University, Lansing, MI, USA.

出版信息

Neurohospitalist. 2024 Jan;14(1):83-86. doi: 10.1177/19418744231196931. Epub 2023 Aug 17.

Abstract

Spinal cord hemorrhage remains an uncommon vascular pathology with no standard approach to treatment. Intramedullary hematomyelia is the rarest subtype and has only been described in case reports, making it a diagnosis easier to miss. A 55-year-old male with history of polysubstance abuse presented for abrupt onset non-traumatic back pain that progressed over several days evolving into paraplegia, sensory loss up to the T3 dermatome, and incontinence of bowel and bladder. His MRI imaging of the cervical and thoracic spine was concerning for a T1 and T2 hyperintense lesion extending from the internal medulla to the upper thoracic spine causing edema. The initial differential diagnosis, based on history and progressive neurologic decline, included a longitudinally extensive transverse mellitus of unclear etiology. He received high dose steroids and plasma exchange without any clinical improvement. An extensive autoimmune, inflammatory, infectious, and demyelinating workup via serum and CSF analysis was unremarkable. He was transferred to a tertiary facility, where repeat MRI spinal imaging included GRE sequences that was significant for increased susceptibility. MRA imaging of the spine was completed showing no vascular malformation. Overall, the diagnosis of hematomyelia was felt to be most likely. Despite aggressive inpatient rehabilitation, the patient remains paraplegic and requires long term management consistent with severe spinal cord injury. This case highlights the importance of recognizing vascular syndromes as a cause of acute myelopathy, understanding the imaging findings of blood breakdown products on MRI, and the desperate need for successful therapeutic strategies to combat a potentially devastating neurologic process.

摘要

脊髓出血仍然是一种罕见的血管病变,尚无标准的治疗方法。髓内血肿性脊髓炎是最罕见的亚型,仅在病例报告中有描述,因此很容易漏诊。一名有多种药物滥用史的55岁男性因突然发作的非创伤性背痛就诊,疼痛在数天内逐渐加重,发展为截瘫、T3皮节以下感觉丧失以及大小便失禁。他的颈椎和胸椎MRI成像显示,从延髓内部延伸至上胸椎的T1和T2高信号病变导致水肿。根据病史和进行性神经功能衰退,初步鉴别诊断包括病因不明的纵向广泛横贯性脊髓炎。他接受了大剂量类固醇和血浆置换治疗,但临床症状没有改善。通过血清和脑脊液分析进行的广泛自身免疫、炎症、感染和脱髓鞘检查均无异常。他被转至一家三级医疗机构,在那里重复进行的脊柱MRI检查包括GRE序列,显示出明显的磁敏感性增加。脊柱MRA成像显示无血管畸形。总体而言,最有可能的诊断是血肿性脊髓炎。尽管进行了积极的住院康复治疗,患者仍为截瘫,需要长期管理,与严重脊髓损伤一致。该病例强调了认识血管综合征作为急性脊髓病病因的重要性,理解MRI上血液分解产物的影像学表现,以及迫切需要成功的治疗策略来对抗潜在的毁灭性神经过程。

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