中枢神经系统原发性血管炎的疑难病例表现

Challenging presentation of primary vasculitis of the central nervous system.

作者信息

Sánchez-Román Edgar, Monternach-Aguilar Felipe, Reyes-Vaca Jorge Guillermo, Rodríguez Leyva Ildefonso

机构信息

Neurology Service, Hospital Central "Dr. Ignacio Morones Prieto", Avenida Venustiano Carranza 2405 L, San Luis Potosi 78210, Mexico.

Biochemistry laboratory, Facultad de Medicina de la Universidad Autónoma de San Luis Potosí, Mexico.

出版信息

Cereb Circ Cogn Behav. 2021 Sep 8;2:100027. doi: 10.1016/j.cccb.2021.100027. eCollection 2021.

DOI:10.1016/j.cccb.2021.100027

PMID:36324727

原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC9616383/

Abstract

OBJECTIVE

To show a patient with a diagnosis of primary vasculitis of the nervous system and review this entity's literature.

CLINICAL CASE

Male 32 years, with two events; first, with a transient monoparesis of the right upper extremity, improve with steroids in days. Now, with a motor Jacksonian progression from the upper to the lower right extremities and generalized seizures. After; aphasia, right hemiparesis, and delirium. In the antiresonance, the finding of multiple arterial cerebral "beading." With an in-depth study, the diagnosis of primary cerebral vasculitis was made. The management with levetiracetam, steroids, and Azathioprine offers a satisfactory evolution.

DISCUSSION

The primary CNS vasculitis is an exclusion diagnosis, with angio-MRI is possible to suspect it, but it is always obligate to discard a secondary etiology by infection, systemic diseases, neoplasia, and drugs.

CONCLUSION

Although this problem is infrequent, we must consider this possibility. Opportune treatment can restore the quality of life.

摘要

目的

展示一例诊断为原发性神经系统血管炎的患者，并回顾该疾病的相关文献。

临床病例

32岁男性，有两次发病情况；第一次，出现右上肢短暂性单瘫，数天内使用类固醇后症状改善。现在，出现从右上肢到右下肢的杰克逊运动性进展及全身性癫痫发作。之后，出现失语、右侧偏瘫和谵妄。在磁共振血管造影中，发现多条脑动脉呈“串珠样”改变。经过深入检查，确诊为原发性脑血管炎。使用左乙拉西坦、类固醇和硫唑嘌呤进行治疗，病情进展令人满意。