Reith W, Kraus C, Harsch N
Klinik für Diagnostische und Interventionelle Neuroradiologie, Universitätsklinikum des Saarlandes, 66421, Homburg/Saar, Deutschland.
Radiologe. 2016 Oct;56(10):890-897. doi: 10.1007/s00117-016-0171-1.
CLINICAL/METHODICAL ISSUE: Vasculitis is a rare cause of diseases of the central nervous system (CNS). Vasculitis can be divided into primary and secondary forms, of which the vast majority can be manifested in various organ systems, including the CNS. Isolated vasculitis of the CNS is limited to the CNS and clinical neurological symptoms as with the other forms of vasculitis, are headaches, encephalopathy, focal deficits and seizures. A criterion of isolated CNS vasculitis is the clinical and laboratory diagnostic exclusion of other forms of vasculitis and the involvement of other organ systems.
Multiple leaps in the caliber of intracranial arteries in cerebral angiography and multiple, small contrast medium-enhanced lesions in magnetic resonance imaging (MRI) of the brain are typical findings, which, however, can also be found in other forms of vasculitis.
The only way of proving meningitis is by a biopsy of the brain meninges and parenchyma. It is necessary to make as accurate a diagnosis as possible, especially in the context of therapeutic options of immunosuppression with steroids and cyclophosphamide.
Cerebral vasculitis is a rare entity but it is an important diagnosis to consider when the appropriate clinical symptoms are present. Thorough laboratory diagnostics and subsequent brain biopsy are necessary to confirm the diagnosis in order to then be able to initiate a specific treatment.
临床/方法学问题:血管炎是中枢神经系统(CNS)疾病的罕见病因。血管炎可分为原发性和继发性,其中绝大多数可在包括中枢神经系统在内的各种器官系统中表现出来。孤立性中枢神经系统血管炎仅限于中枢神经系统,与其他形式的血管炎一样,临床神经症状包括头痛、脑病、局灶性缺损和癫痫发作。孤立性中枢神经系统血管炎的一个标准是通过临床和实验室诊断排除其他形式的血管炎以及其他器官系统的受累情况。
脑血管造影中颅内动脉管径的多处跳跃以及脑部磁共振成像(MRI)中的多处小的对比剂增强病变是典型表现,然而,这些表现也可能在其他形式的血管炎中出现。
证实脑膜炎的唯一方法是进行脑膜和脑实质活检。必须尽可能准确地做出诊断,尤其是在使用类固醇和环磷酰胺进行免疫抑制治疗的背景下。
脑血管炎是一种罕见疾病,但当出现适当的临床症状时,它是一个需要考虑的重要诊断。为了能够启动特定治疗,进行全面的实验室诊断并随后进行脑活检以确诊是必要的。