Savoldi Filippo, Kaufmann Timothy J, Flanagan Eoin P, Toledano Michel, Weinshenker Brian G
Departments of Neurology (F.S., E.P.F., M.T., B.G.W.), and Neuroradiology (T.J.K.), Mayo Clinic, Rochester, MN; and Neuroimaging Research Unit (F.S.), Department of Neurology, San Raffaele Scientific Institute and University, Milan, Italy.
Neurol Neuroimmunol Neuroinflamm. 2017 May 11;4(4):e355. doi: 10.1212/NXI.0000000000000355. eCollection 2017 Jul.
Elsberg syndrome (ES) is an established but often unrecognized cause of acute lumbosacral radiculitis with myelitis related to recent herpes virus infection. We defined ES, determined its frequency in patients with cauda equina syndrome (CES) with myelitis, and evaluated its clinical, radiologic, and microbiologic features and outcomes.
We searched the Mayo Clinic medical records for ES and subsequently for combinations of index terms to identify patients with suspected CES and myelitis.
Our search yielded 30 patients, 2 diagnosed with ES and an additional 28 with clinical or radiologic evidence of CES retrospectively suspected of having ES. We classified patients in 5 groups according to diagnostic certainty. MRI and EMG confirmed that 2 had only myelitis, 5 only radiculitis, and 16 both. Two had preceding sacral herpes infection and 1 oral herpes simplex. Spinal cord lesions were commonly multiple, discontinuous, not expansile, and centrally or ventrally positioned. Lesions generally spared the distal conus. Nerve root enhancement was occasionally prominent and was smooth rather than nodular. Lymphocytic CSF pleocytosis was common. Thirteen patients (43%) had viral isolation studies, which were commonly delayed; the delay may have accounted for the low rate of viral detection. Acyclovir was administered to 6 patients. Most patients recovered with sequelae; 1 patient experienced encephalomyelitis and died.
ES is a definable condition likely responsible for 10% of patients with combined CES and myelitis. Radiologic findings are not entirely specific but may help in differentiating ES from some competing diagnostic considerations. We propose criteria to facilitate diagnosis.
埃尔施贝格综合征（ES）是急性腰骶神经根炎合并脊髓炎的一种既定病因，但常未被识别，其与近期疱疹病毒感染相关。我们对ES进行了定义，确定其在合并脊髓炎的马尾综合征（CES）患者中的发生率，并评估其临床、影像学和微生物学特征及预后。
我们在梅奥诊所的医疗记录中搜索ES，随后搜索索引词组合以识别疑似CES和脊髓炎的患者。
我们的搜索共找到30例患者，其中2例被诊断为ES，另外28例有CES的临床或影像学证据，回顾性怀疑患有ES。我们根据诊断确定性将患者分为5组。MRI和肌电图证实，2例仅有脊髓炎，5例仅有神经根炎，16例两者皆有。2例有骶部疱疹感染史，1例有口腔单纯疱疹史。脊髓病变通常为多发、不连续、无扩张，位于中央或腹侧。病变一般不累及圆锥末端。神经根强化偶尔明显，呈光滑而非结节状。淋巴细胞性脑脊液细胞增多常见。13例患者（43%）进行了病毒分离研究，通常延迟进行；延迟可能是病毒检测率低的原因。6例患者接受了阿昔洛韦治疗。大多数患者康复但有后遗症；1例患者发生脑脊髓炎并死亡。
ES是一种可定义的疾病，可能导致10%的合并CES和脊髓炎的患者发病。影像学表现并非完全特异，但可能有助于将ES与一些其他竞争性诊断考虑因素区分开来。我们提出了便于诊断的标准。
