Abstract This report illustrates the case of a 23-year-old woman with fulminant acute disseminated encephalomyelitis (ADEM) in the setting of hepatic epithelial hemangioendothelioma (EHE) requiring urgent decompressive hemicraniectomy. There were no findings suggestive of infection, angionecrotic vasculitis, or neoplasm. She had no antecedent viral infection and no other signs or symptoms of previously described paraneoplastic processes. While previous cases of ADEM requiring hemicraniectomy have been reported, this is the first without antecedent viral infection or prolonged fever at onset and in the setting of this rare liver malignancy. This case supports the inclusion of ADEM in the differential diagnosis of acute neurologic decline in the absence of malignancies associated with known paraneoplastic syndromes. EHE may be associated with paraneoplastic processes requiring urgent neurosurgical intervention.

中文翻译：

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急性播散性脑脊髓炎与肝上皮血管内皮瘤新的副肿瘤过程相关：病例报告

摘要 本报告描述了一名 23 岁女性暴发性急性播散性脑脊髓炎 (ADEM) 的病例，该病例在肝上皮血管内皮瘤 (EHE) 的情况下需要紧急减压偏侧颅骨切除术。没有发现提示感染、血管坏死性血管炎或肿瘤。她没有先前的病毒感染，也没有先前描述的副肿瘤过程的其他体征或症状。虽然以前有报道过需要进行偏侧颅骨切除术的 ADEM 病例，但这是第一例没有先行病毒感染或在发病时和这种罕见的肝脏恶性肿瘤的情况下长时间发热的病例。该病例支持在不存在与已知副肿瘤综合征相关的恶性肿瘤的情况下，将 ADEM 纳入急性神经功能衰退的鉴别诊断中。