Neuroimmunology Clinic, Concord Hospital, University of Sydney.
Brain & Mind Centre, University of Sydney, New South Wales, Australia.
Curr Opin Neurol. 2019 Jun;32(3):467-474. doi: 10.1097/WCO.0000000000000683.
To review the clinical findings, differential diagnosis, treatment and outcome of pseudotumoral demyelinating lesions including tumefactive demyelination and Baló's concentric sclerosis.
MRI findings, such as dynamic restricted diffusion changes at the edge of pseudotumoral lesions help to discriminate atypical demyelination from key differential diagnoses, and together with histopathological data, indicate that tissue hypoxia may be important aetiologically. CT-PET imaging can help to distinguish pseudotumoral lesions from high-grade tumours. Although most patients with pseudotumoral lesions have or later develop multiple sclerosis, a proportion will experience a monophasic course or be diagnosed with neuromyelitis optica spectrum disorders (NMOSD), myelin oligodendrocyte glycoprotein (MOG) antibody-associated demyelination or acute disseminated encephalomyelitis (ADEM). Many patients with pseudotumoral demyelinating lesions have a favourable prognosis.
Not all patients with pseudotumoral lesions require a brain biopsy but close follow-up of biopsied and nonbiopsied lesions is indicated once a diagnosis is established. Testing for AQP4-IgG and MOG-IgG is recommended when a pseudotumoral demyelinating lesion is identified. In the absence of large, prospective studies, it seems reasonable that patients with pseudotumoral lesions who fulfil multiple sclerosis diagnostic criteria are treated with multiple sclerosis therapies.
综述假瘤样脱髓鞘病变（包括肿块样脱髓鞘和 Baló 同心圆硬化）的临床特征、鉴别诊断、治疗和预后。
假瘤样病变 MRI 表现为病变边缘弥散受限的动态变化，有助于将非典型脱髓鞘与关键鉴别诊断区分开来，结合组织病理学数据表明组织缺氧可能具有重要的病因学意义。CT-PET 成像有助于区分假瘤样病变和高级别肿瘤。虽然大多数假瘤样病变患者存在或随后发展为多发性硬化症，但一部分患者表现为单相病程或被诊断为视神经脊髓炎谱系疾病（NMOSD）、髓鞘少突胶质细胞糖蛋白（MOG）抗体相关脱髓鞘疾病或急性播散性脑脊髓炎（ADEM）。许多假瘤样脱髓鞘病变患者预后良好。
并非所有假瘤样病变患者都需要进行脑活检，但一旦确诊，应密切随访活检和非活检病变。当诊断为假瘤样脱髓鞘病变时，建议检测水通道蛋白 4 抗体（AQP4-IgG）和髓鞘少突胶质细胞糖蛋白抗体（MOG-IgG）。在缺乏大型前瞻性研究的情况下，对于符合多发性硬化症诊断标准的假瘤样病变患者，采用多发性硬化症治疗似乎是合理的。
