中国医学科学院学报2024，Vol.46Issue(4)：546-553,8.DOI:10.3881/j.issn.1000-503X.15885
17例经脑活检确诊的原发性中枢神经系统血管炎患者的各临床亚型特点分析
Clinical Features of 17 Patients With Primary Angiitis of the Central Nervous System Confirmed by Brain Biopsy
摘要
Abstract
Objective To analyze the clinical features of 17 patients with primary angiitis of the central nervous system(PACNS)and thus facilitate the early diagnosis and treatment,reduce the recurrence and mortal-ity,and improve the prognoses of this disease.Methods We collected the data of patients with PACNS diag-nosed by brain biopsy from January 2009 to June 2023 and analyzed their clinical presentations,laboratory and imaging manifestations,electrophysiological and pathological changes,and treatment regimens and prognosis.Results The 17 patients diagnosed with PACNS via brain biopsy included one child and 16 adults.The subtyp-ing results showed that 10,2,3,2,1,and 1 patients had tumorous,spinal cord-involved,angiography-posi-tive,rapidly progressive,hemorrhagic,and amyloid β-related PACNS,respectively.Eleven(64.7％)of the patients were complicated with secondary epilepsy.All the patients exhibited abnormal manifestations in head MRI,with 94.1％showing lesions with uneven enhancement around the lesions or in the leptomeninges.Mag-netic resonance angiography revealed large vessel abnormalities in 3 patients,and spinal cord involvement was observed in 2 patients.Histopathological typing revealed 7(43.7％)patients with lymphocytic vasculitis and 5(31.2％)patients with necrotizing vasculitis.Eleven patients were treated with glucocorticoids and cyclophospha-mide,which resulted in partial lesion disappearance and symptom amelioration in 6 patients upon reevaluation with head MRI after 3 months of maintenance therapy.Two,1,and 3 patients experienced rapid disease progres-sion,death,and recurrence within 1 year,respectively.Three patients showed insensitivity to hormonotherapy and residual disabilities.Two patients received rituximab after relapse and remained clinically stable during a fol-low-up period of 0.5-1 year.Conclusions Tumorous PACNS was more prone to epilepsy,mainly occurring in males.The most common histopathological type was necrotizing vasculitis,which responded to hormonotherapy and had favorable outcomes.Therefore,for the young patients with epilepsy and intracranial tumorous lesions,the possibility of PACNS should be considered.Spinal cord involvement in PACNS was often located in the thorac-ic and cervical cords,suggesting a poorer prognosis.Electromyography commonly revealed neural conduction ab-normalities in the anterior horn or roots,providing clues for differential diagnosis.For suspected spinal cord in-volvement,comprehensive electromyography is recommended.Rapidly progressive PACNS often presented infrat-entorial lesions,such as lesions in the pons and medulla,with a higher mortality rate.Hemorrhagic PACNS was rare,and a multifocal hemorrhagic lesion with enhancement in the intracranial region,particularly in young pa-tients,should raise suspicion.For the patients with recurrent or progressive disease,rituximab is a recommended therapeutic option.关键词
原发性中枢神经系统血管炎/临床亚型/肌电图/MRI/利妥昔单抗Key words
primary angiitis of the central nervous system/clinical subtype/electromyography/MRI/rituximab分类
医药卫生引用本文复制引用
张莉,孙慧,张世敏,高赛,武雷,黄德晖..17例经脑活检确诊的原发性中枢神经系统血管炎患者的各临床亚型特点分析[J].中国医学科学院学报,2024,46(4):546-553,8.基金项目
中国研究型医院学会科研课题(Y2023FH-5JK09) （Y2023FH-5JK09）
