中枢神经系统的初级血管炎:亚型之间的特征差异和印度队列的结果
Naveen K Paramasivan1, Dev P Sharma1, S M Krishna Mohan1
1From the Department of Neurology (N.K.P., S.M.K.M., S.S., S.E.S., P.S.); Imaging Sciences and Interventional Radiology (D.P.S.); Comprehensive Stroke Care Program (S.E.S., P.S.); and Achutha Menon Centre for Health Science Studies (P.S.S.), Sree Chitra Tirunal Institute for Medical Sciences and Technology, Trivandrum, India.
Neurology(R) neuroimmunology & neuroinflammation
|June 10, 2024
概括
中枢神经系统 (PACNS) 亚型的原发性血管炎存在显著差异. 在PACNS中,小血管血管炎带来了独特的挑战,导致诊断延迟,长期结果较差,复发增加.
科学领域:
- 神经学 神经学
- 免疫学 免疫学 免疫学
- 内部医学 内部医学
背景情况:
- 中枢神经系统初级血管炎 (PACNS) 是一种罕见的疾病,患病率和死亡率很高.
- PACNS亚型表现出多样化的表现,使诊断复杂化.
- 诊断复杂性增加,因为可能会错过某些模式的呈现.
研究的目的:
- 在印度群体中区分PACNS亚型.
- 描述PACNS亚型的临床和放射性特征.
- 为了比较PACNS亚型之间的治疗和结果.
主要方法:
- 成年PACNS患者 (2000-2019年) 的回顾性单中心队列研究.
- 诊断证实使用卡拉布雷斯和马莱克标准.
- 基于临床,放射学,治疗和结果数据的中小血管血管炎亚型的比较.
主要成果:
- 小血管血管炎亚型的发病年龄较小,中风发病频率较低,诊断/治疗延迟显著 (620天对118天).
- 小血管血管炎组在1年和最后的随访时 (72%对34%) 经历了较差的结果,复发率更高 (89%对30%).
- 总体而言,69%和69.8%的患者在6个月和1年后分别有良好的结果;49%的患者出现复发.
结论:
- 小血管血管炎是一个独特的PACNS实体,与诊断/治疗延迟和不利的长期结果有关.
- 增加复发是小血管血管炎亚型的特征.
- 识别PACNS亚型对于及时诊断和有效的治疗计划至关重要.
更多相关视频
相关概念视频
01:21Aneurysm II: Clinical Manifestations and Diagnostic Studies
592
Thoracic, aortic arch and abdominal aneurysms are significant vascular conditions that can present with various clinical manifestations and lead to serious complications. Understanding these manifestations and the appropriate diagnostic studies is essential for effective management and treatment.Thoracic Aortic AneurysmsThoracic aortic aneurysms often remain asymptomatic until they reach a size that impinges on adjacent structures. They typically cause deep, diffuse chest pain that radiates to...
592
01:26Bacterial Meningitis II: Pathophysiology
45
Bacterial meningitis typically begins when pathogens such as Neisseria meningitidis and Streptococcus pneumoniae colonize the nasopharynx and invade the bloodstream. This process is facilitated by bacterial virulence factors, such as polysaccharide capsules, which resist phagocytosis and complement-mediated killing. Less commonly, bacteria reach the central nervous system via contiguous spread from infections like otitis media or sinusitis, through congenital or acquired dural defects, or...
45
01:26Brain Abscess l: Introduction
47
A brain abscess is a focal, intracerebral infection characterized by a localized collection of pus within the brain parenchyma, resulting from microbial invasion and the body’s inflammatory response. It progresses through stages: early and late cerebritis, followed by early and late capsule formation, reflecting tissue destruction, immune response, and eventual encapsulation.Etiology and PathogenesisCausative organisms vary with source and host factors, often involving polymicrobial...
47