中枢神经系统非典型炎症性脱髓鞘综合征。
Atypical inflammatory demyelinating syndromes of the CNS.
机构信息
Neuroimmunology Clinic, Concord Hospital, University of Sydney, Sydney, NSW, Australia; Brain and Mind Centre, University of Sydney, Sydney, NSW, Australia.
Neuroimmunology Clinic, Concord Hospital, University of Sydney, Sydney, NSW, Australia; Brain and Mind Centre, University of Sydney, Sydney, NSW, Australia.
出版信息
Lancet Neurol. 2016 Aug;15(9):967-981. doi: 10.1016/S1474-4422(16)30043-6.
Atypical inflammatory demyelinating syndromes are rare disorders that differ from multiple sclerosis owing to unusual clinical or MRI findings or poor response to treatments used for multiple sclerosis. These syndromes include neuromyelitis optica spectrum disorder, acute disseminated encephalomyelitis, tumefactive demyelination, Baló's concentric sclerosis, Schilder's disease, and Marburg's multiple sclerosis. The overlapping features of these syndromes with multiple sclerosis and with each other complicate diagnosis and their categorisation as distinct or related conditions. Recognition of these syndromes is crucial because they differ from multiple sclerosis and other demyelinating and non-demyelinating conditions in their prognosis and treatment. Advances in MRI, pathology, and immunobiology are needed to increase understanding of these syndromes, including the extent to which some of them represent distinct entities, and to assist with improvements in their diagnosis and management.
非典型炎性脱髓鞘综合征是罕见疾病，由于其临床或 MRI 表现异常、对多发性硬化症治疗的反应不佳，与多发性硬化症不同。这些综合征包括视神经脊髓炎谱系疾病、急性播散性脑脊髓炎、肿块样脱髓鞘病变、Balo 同心性硬化症、希尔德病和马尔堡多发性硬化症。这些综合征与多发性硬化症以及彼此之间的重叠特征使诊断和分类变得复杂，难以确定其为独特或相关疾病。认识这些综合征至关重要，因为它们在预后和治疗方面与多发性硬化症以及其他脱髓鞘和非脱髓鞘疾病不同。需要在 MRI、病理学和免疫生物学方面取得进展，以提高对这些综合征的认识，包括其中一些疾病在多大程度上代表独特实体，并协助改善其诊断和管理。
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