原发性中枢神经系统血管炎（附8例经病理确诊病例及文献复习）

摘要/Abstract

摘要：

目的依据国际通用的诊断标新，分析比较原发性中枢神经系统血管炎（primary c entral n ervous system vasculitis，PCNSV）诊断手段。方法收集2012年1月1日-2015年12月31日于首都医科大学附属北京天坛医院神经病学中心神经感染与免疫科住院治疗的PACNS患者共8例，回顾性分析患者临床资料，总结其临床表现、实验室检查、神经影像学及神经病理学特征。结果临床表现：本组8例患者中，居于前五位的首发症状为慢性头痛、痫性发作、类卒中样局灶性神经功能缺损、精神行为异常和锥体外系症状，所有患者均未发现特异性的血生化及脑脊液异常结果，头颅磁共振成像（magnetic resonance imaging，MRI）均可见颅内异常信号，磁共振血管造影（magnetic resonance angiography，MRA）及数字减影血管造影（digital subtraction angiography，DSA）均未见明显异常。患者行脑组织活检，病理结果符合PCNSV表现。结论 PCNSV的诊断要综合考虑患者的临床表现、实验室检查和影像学检查结果，脑组织活检的广泛开展，有助于提高本病的诊断率。

文章导读： 对于缺乏常见危险因素及典型影像学改变的卒中患者，应考虑原发性中枢神经系统血管炎的可能，以期提供新的诊断和治疗措施。

关键词: 原发性中枢神经系统血管炎; 脑组织活检

Abstract:

Objective To analyze and compare the diagnosis method of primary central nervous system vasculitis (PCNSV) according to the international standard. Methods A total of 8 cases who were admitted to Department of Neuro-infection and Immunology of Neurological Disease Center of Beijing Tiantan Hospital, Capital Medical University during Jan.1st, 2012 to Dec.31st, 2015 and diagnosed with PCNSV were reviewed. Their clinical descriptions, laboratory examinations, neuroimaging and pathological characteristic were analyzed. Results The most common clinical symptoms were: chronic headache, seizures, stroke like focal neurological deficits, mental and behavioral abnormalities and symptoms of the external pyramidal system. No specific blood biochemical and cerebral spinal fluid (CSF) abnormalities were found in all patients. Intracranial abnormal signal was seen in magnetic resonance imaging (MRI). Magnetic resonance angiography (MRA) and digital subtraction angiography (DSA) were not significantly abnormal. Brain biopsy was performed in 8 patients and the pathologic results were consistent with PCNSV expression. Conclusion The diagnosis of PCNSV should consider the patient's clinical manifestations, laboratory tests and imaging findings. The extensive development of brain biopsy is helpful to improve the diagnostic rate of the disease.

Key words: Primary central nervous system vasculitis; Brain biopsy

雷爱弟，张星虎，周衡. 原发性中枢神经系统血管炎（附8例经病理确诊病例及文献复习）[J]. 中国卒中杂志, 2016, 11(10): 863-867.

LEI Ai-Di, ZHANG Xing-Hu, ZHOU Heng. Primary Central Nervous System Vasculitis (8 Cases Report and Literature Review)[J]. Chinese Journal of Stroke, 2016, 11(10): 863-867.

参考文献

1 MacLaren K，Gillespie J，Shrestha S，et al. Primary

angiitis of the central nervous system：emerging

variants[J]. QJM，2005，98：643-654.

2 Birnbaum J，Hellmann DB. Primary angiitis of the

central nervous system[J]. Arch Neurol，2009，66：

704-709.3 Calabrese LH，Mallek JA. Primary angiitis of the

central nervous system. Report of 8 new cases，

review of the literature，and proposal for diagnostic

criteria[J]. Medicine (Baltimore)，1988，67：20-39.

4 Ferro JM. Vasculitis of the central nervous system[J].

J Neurology，1998，245：766-776.

5 Scott DG，Bacon PA，Elliott PJ，et al. Systemic

vasculitis in a district general hospital 1972-1980：

clinical and laboratory features，classification and

prognosis of 80 cases[J]. QJM，1982，51：292-311.

6 刘昭. 原发性中枢神经系统血管炎[J]. 心脑血管病防

治，2009，9：296-298.

7 Salvarani C，Brown RD Jr，Christianson T，et al.

An update of the Mayo Clinic cohort of patients

with adult primary central nervous system vasculitis：

description of 163 patients[J]. Medicine (Baltimore)，

2015，94：e738.

8 Ducros A，Bousser MG. Reversible cerebral

vasoconstriction syndrome[J]. Pract Neurol，2009，9：

256-267.

9 Singh S，John S，Joseph TP，et al. Primary angiitis of

the central nervous system：MRI features and clinical

presentation[J]. Australas Radiol，2003，47：127-134.

10 Moritani T，Hiwatashi A，Shier DA，et a1. CNS

vasculitis and vasculopathy：efficacy and usefulness

of diffusion-weighted echoplanar MR imaging[J]. J

Clin Imaging，2004，28：261-270．

11 Moore PM. Central nervous system vasculitis[J]. Curr

Opin Neurol，1998，11：241-246.

12 Zuccoli G，Pipitone N，Haldipur A，et al. Imaging

f indings in pr imary cent ral nervous system

vascul it is[J ]. Cl in Exp Rheumatol，2011，29：

S104-S109.

13 Calabrese LH，Dodick DW，Schwedt TJ，et al.

Narrative review：reversible cerebral vasoconstriction

syndromes[J]. Ann Intern Med，2007，146：34-44.

14 Salvarani C，Brown RD Jr，Hunder GG. Adult

primary central nervous system vasculitis[J]. Lancet，

2012，25：767-777.

15 Greenan TJ，Grossman RI，Goldberg HI. Cerebral

va scul i t i s：MR imag i ng a nd a ng iog r aph i c

correlation[J]. Radiology，1992，182：65-72.

16 Fieschi C，Rasura M，Anzini A，et al. Central

nervous system vasculitis[J]. J Neurol Sci，1998，153：

159-171.

17 Salvarani C，Brown RD Jr，Calamia KT，et a1.

Angiography-negative primary central nervous system

vasculitis：a syndrome involving small cerebral

vessels[J]. Medicine (Baltimore)，2008，87：264-271.

18 Schuster S，Magnus T. Primary central nervous

system vasculitis[J]. Z Rheumatol，2015，74：854-856，

858-860，862.

19 赵贺玲，曹秉振. 原发性中枢神经系统血管炎[J]. 中国

神经免疫学和神经病学杂志，2010，17：74-75.

20 李伟，李少武，王晶，等. 9例原发性中枢神经系统血

管炎临床、影像及病理特点研究[J]. 中国卒中杂志，

2015，10：573-579.

21 韦英秀. 原发性中枢神经系统血管炎12例临床分析[J].

中国老年学杂志，2010，30：2848-2849.

22 庞长绪，李永明，张平. 原发性中枢神经系统血管炎11

例临床疗效分析[J]. 中国现代药物应用，2012，6：99-

100.

23 Yaari R，Anselm IA，Szer IS，et al. Childhood

primary angiitis of the central nervous system：two

biopsy-proven cases[J]. J Pediatr，2004，145：693-697.

24 Elbers J，Halliday W，Hawkins C，et al. Brain biopsy

in children with primary small-vessel central nervous

system vasculitis[J]. Ann Neurol，2010，68：602-610.

25 Salva rani C，Brown R D Jr，Ca lamia KT，e t

al. Primary cent ral nervous system vasculitis：

comparison of patients with and without cerebral

amyloid angiopathy[J]. Rheumatology (Oxford)，2008，

47：1671-1677.

26 Calabrese LH，Mallek JA. Primary angiitis of the

central nervous system. Report of 8 new cases，

review of the literature，and proposal for diagnostic

criteria[J]. Medicine (Baltimore)，1988，67：20-39.

27 Moore PM. Diagnosis and management of isolated

angiitis of the central nervous system[J]. Neurology，

1989，39：167-173.