CLIPPERS综合征一例并文献复习
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摘要
类固醇激素反应性慢性淋巴细胞性炎性反应伴脑桥血管周围强化症(CLIPPERS)综合征是一种对类固醇激素治疗敏感的慢性中枢神经系统炎性疾病,主要累及脑桥,部分患者也可同时累及多个部位。该病比较罕见,其诊断主要依据头部MR增强扫描示"胡椒盐"样强化这一特殊的影像学改变及结合临床中其他检查手段进行诊断,需与临床表现及影像学改变与CLIPPERS综合征类似的疾病进行鉴别,给予激素治疗后可有效缓解临床症状。作者报道1例主要累及小脑和脊髓的CLIPPERS综合征,以加强对该病的认识。
Chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids( CLIPPERS syndrome) is a chronic central nervous system inflammatory disease with sensitivity to steroid therapy.It mainly involves the pons and sometimes other sites.It is relatively rare,and diagnosed by the special sign of‘pepper-salt-like enhancement'in the head magnetic resonance imaging combining other clinical examinations.It should be differentiated from diseases with similar imaging changes.Steroid therapy can effectively relieve the clinical symptoms.The authors report a case of CLIPPERS syndrome that mainly involving cerebellum and spinal cord,and that will be helpful for the understanding of this disease.
Chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids( CLIPPERS syndrome) is a chronic central nervous system inflammatory disease with sensitivity to steroid therapy.It mainly involves the pons and sometimes other sites.It is relatively rare,and diagnosed by the special sign of‘pepper-salt-like enhancement'in the head magnetic resonance imaging combining other clinical examinations.It should be differentiated from diseases with similar imaging changes.Steroid therapy can effectively relieve the clinical symptoms.The authors report a case of CLIPPERS syndrome that mainly involving cerebellum and spinal cord,and that will be helpful for the understanding of this disease.
引文
[1]Pittock SJ,Debruyne J,Krecke KN,et al.Chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids(CLIPPERS)[J].Brain,2010,133(9):2626-2634.
[2]Veerapandiyan A,Chaudhari A,Deo P,et al.Chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids(CLIPPERS):a pediatric case report with six year follow-up[J].Mult Scler Relat Disord,2017,17:95-98.
[3]甘信涛,潘星辰,何昕,等.类固醇激素反应性慢性淋巴细胞性炎症伴脑桥血管周围强化症1例[J].中国医学影像技术,2017,33(1):159-160.
[4]周立霞,全冠民,袁涛,等.主要累及全脊髓和脑干的CLIPPERS一例[J].脑与神经疾病杂志,2016,24(11):701-704.
[5]张维雄,黄丽娜,高秀菊,等.类固醇激素反应性慢性淋巴细胞性炎性反应伴脑桥血管周围强化症1例并文献复习[J].中国神经免疫学和神经病学杂志,2015,22(5):347-351.
[6]谢萌,邹丽萍,孟岩,等.儿童类固醇激素反应性慢性淋巴细胞性炎症伴脑桥周围血管强化症1例报告及文献综述[J].临床儿科杂志,2015,33(11):933-936.
[7]Gabilondo I,Saiz A,Graus F,et al.Response to immunotherapy in CLIPPERS syndrome[J].J Neurol,2011,258(11):2090-2092.
[8]Tan BL,Agzarian M,Schultz DW.CLIPPERS:induction and maintenance of remission using hydroxychloroquine[J].Neurol Neuroimmunol Neuroinflamm,2015,2(1):e56.
[9]Mele N,Guiraud V,Labauge P,et al.Effective antituberculous therapy in a patient with CLIPPERS:new insights into CLIPPERS pathogenesis[J].Neurol Neuroimmunol Neuroinflamm,2014,1(1):e6.
[10]刘昭.原发性中枢神经系统血管炎[J].心脑血管病防治,2009,9(4):296-298.
[11]郑奎宏,马林,史丽静,等.肿块样原发性中枢神经系统血管炎的磁共振表现[J].中国临床医学影像杂志,2010,21(11):761-764.
[12]王金涛,隋世华,董丽华,等.CLIPPERS综合征1例报告并文献复习[J].中国神经精神疾病杂志,2016,42(2):121-123.
[2]Veerapandiyan A,Chaudhari A,Deo P,et al.Chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids(CLIPPERS):a pediatric case report with six year follow-up[J].Mult Scler Relat Disord,2017,17:95-98.
[3]甘信涛,潘星辰,何昕,等.类固醇激素反应性慢性淋巴细胞性炎症伴脑桥血管周围强化症1例[J].中国医学影像技术,2017,33(1):159-160.
[4]周立霞,全冠民,袁涛,等.主要累及全脊髓和脑干的CLIPPERS一例[J].脑与神经疾病杂志,2016,24(11):701-704.
[5]张维雄,黄丽娜,高秀菊,等.类固醇激素反应性慢性淋巴细胞性炎性反应伴脑桥血管周围强化症1例并文献复习[J].中国神经免疫学和神经病学杂志,2015,22(5):347-351.
[6]谢萌,邹丽萍,孟岩,等.儿童类固醇激素反应性慢性淋巴细胞性炎症伴脑桥周围血管强化症1例报告及文献综述[J].临床儿科杂志,2015,33(11):933-936.
[7]Gabilondo I,Saiz A,Graus F,et al.Response to immunotherapy in CLIPPERS syndrome[J].J Neurol,2011,258(11):2090-2092.
[8]Tan BL,Agzarian M,Schultz DW.CLIPPERS:induction and maintenance of remission using hydroxychloroquine[J].Neurol Neuroimmunol Neuroinflamm,2015,2(1):e56.
[9]Mele N,Guiraud V,Labauge P,et al.Effective antituberculous therapy in a patient with CLIPPERS:new insights into CLIPPERS pathogenesis[J].Neurol Neuroimmunol Neuroinflamm,2014,1(1):e6.
[10]刘昭.原发性中枢神经系统血管炎[J].心脑血管病防治,2009,9(4):296-298.
[11]郑奎宏,马林,史丽静,等.肿块样原发性中枢神经系统血管炎的磁共振表现[J].中国临床医学影像杂志,2010,21(11):761-764.
[12]王金涛,隋世华,董丽华,等.CLIPPERS综合征1例报告并文献复习[J].中国神经精神疾病杂志,2016,42(2):121-123.