Rovira Álex, Auger Cristina, Rovira Antoni
MR Unit, Department of Radiology, Hospital Universitari Vall d'Hebron, Autonomous University of Barcelona, Barcelona, Spain.
MR Unit, Department of Radiology, Hospital Universitari Vall d'Hebron, Autonomous University of Barcelona, Barcelona, Spain.
Handb Clin Neurol. 2016;135:425-446. doi: 10.1016/B978-0-444-53485-9.00021-0.
Idiopathic inflammatory-demyelinating diseases (IIDDs) represent a broad spectrum of central nervous system (CNS) disorders, including monophasic, multiphasic, and progressive disorders that range from highly localized forms to multifocal or diffuse variants. In addition to the classic multiple sclerosis (MS) phenotypes, several MS variants have been described, which can be differentiated on the basis of severity, clinical course, and lesion distribution. Other forms of IIDD are now recognized as distinct entities and not MS variants, such as acute disseminated encephalomyelitis, and neuromyelitis optica spectrum disorders. The CNS can also be affected by a variety of inflammatory diseases. These include primary angiitis of the CNS (PACNS), a rare disorder specifically targeting the CNS vasculature, and various systemic conditions which, among other organs and systems, can also affect the CNS, such as systemic vasculitis and sarcoidosis. The diagnosis of PACNS is difficult, as this condition may be confused with reversible cerebral vasoconstriction syndrome (RCVS), a term comprising a group of conditions characterized by prolonged but reversible vasoconstriction of the cerebral arteries. Magnetic resonance imaging of the brain and spine is the radiologic technique of choice for diagnosing these disorders, and, together with the clinical and laboratory findings, enables a prompt and accurate diagnosis.
特发性炎性脱髓鞘疾病(IIDDs)是一大类中枢神经系统(CNS)疾病,包括单相、多相和进行性疾病,范围从高度局限性形式到多灶性或弥漫性变体。除了经典的多发性硬化症(MS)表型外,还描述了几种MS变体,可根据严重程度、临床病程和病变分布进行区分。现在,其他形式的IIDDs被认为是不同的实体,而非MS变体,如急性播散性脑脊髓炎和视神经脊髓炎谱系障碍。CNS也可受到多种炎性疾病的影响。这些疾病包括中枢神经系统原发性血管炎(PACNS),这是一种罕见的专门针对CNS血管系统的疾病,以及各种全身性疾病,这些疾病除了其他器官和系统外,也可影响CNS,如系统性血管炎和结节病。PACNS的诊断很困难,因为这种疾病可能与可逆性脑血管收缩综合征(RCVS)相混淆,RCVS是一个术语,包括一组以脑动脉长时间但可逆性血管收缩为特征的疾病。脑和脊柱的磁共振成像是诊断这些疾病的首选放射学技术,结合临床和实验室检查结果,能够实现快速准确的诊断。