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埃尔斯伯格综合征:一例新型影像学表现的病例报告。

Elsberg Syndrome: A Case Report of a Novel Radiographic Presentation.

作者信息

Lyons Clayton E, Dixit Karan

机构信息

Northwestern University Feinberg School of Medicine, Chicago, IL, USA.

Division of Neuro-Oncology, Department of Neurology, Northwestern University Feinberg School of Medicine, Chicago, IL, USA.

出版信息

Neurohospitalist. 2024 Jul;14(3):347-350. doi: 10.1177/19418744241241107. Epub 2024 Mar 21.

Abstract

Elsberg syndrome (ES) presents with bowel and bladder dysfunction, resembling cauda equina syndrome, and is classified as a clinicoradiographic syndrome most commonly associated with HSV-2 reactivation. Most cases reveal smooth and continuous nerve enhancement on imaging. We present a unique case of ES that presented as several nodular, ring enhancing soft tissue masses along the cauda equina. An 81-year-old woman presented with several weeks of sacral sensory impairment. MRI of the lumbar spine at presentation showed several nodular, ring enhancing soft tissue masses within the thecal sac along the cauda equina, concerning for leptomeningeal carcinomatosis from an unknown primary source. Cerebrospinal fluid (CSF) analysis was notable for lymphocyte predominant pleocytosis and protein elevation, which was nonspecific but suggestive of leptomeningeal carcinomatosis. CSF rapid meningitis panel was positive for HSV2 which was confirmed on HSV2 PCR. The patient was briefly on on acyclovir and was stopped due to lack of meningioencephalitis symptoms. Malignancy workup with cytological analysis and systemic imaging were negative. Given the absence of malignancy and positive HSV2 PCR, the patient was diagnosed with HSV-2 sacral radiculitis and subsequently treated with a full course of intravenous acyclovir with gradual clinical and radiographic improvement. Ring-enhancing lesions along the cauda equina are most suggestive of LC. Our case highlights an as of yet unreported presentation of ES which may be valuable for neurologists to be aware of as to avoid any potential diagnostic dilemma, minimize unnecessary and costly testing, and not delay effective treatment.

摘要

埃尔斯伯格综合征(ES)表现为肠道和膀胱功能障碍,类似于马尾综合征,被归类为一种最常与单纯疱疹病毒2型(HSV - 2)再激活相关的临床影像学综合征。大多数病例在影像学上显示神经强化平滑且连续。我们报告一例独特的ES病例,其表现为沿马尾出现多个结节状、环形强化的软组织肿块。一名81岁女性出现数周的骶部感觉障碍。就诊时腰椎磁共振成像(MRI)显示沿马尾的硬膜囊内有多个结节状、环形强化的软组织肿块,怀疑是不明原发灶的软脑膜癌转移。脑脊液(CSF)分析显示以淋巴细胞为主的细胞增多和蛋白升高,这虽不具特异性但提示软脑膜癌转移。脑脊液快速脑膜炎检测板HSV2呈阳性,HSV2聚合酶链反应(PCR)证实了这一点。患者短期使用阿昔洛韦,后因缺乏脑膜脑炎症状而停药。细胞学分析和全身影像学的恶性肿瘤检查均为阴性。鉴于无恶性肿瘤且HSV2 PCR阳性,该患者被诊断为HSV - 2骶神经根炎,随后接受了全程静脉注射阿昔洛韦治疗,临床和影像学逐渐改善。沿马尾的环形强化病变最提示软脑膜癌转移。我们的病例突出了一种尚未报道的ES表现,神经科医生了解这一点可能很有价值,以避免任何潜在的诊断困境,减少不必要且昂贵的检查,并避免延误有效治疗。

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