Reddy Soma Madhan, Lath Rahul, Swain Meenakshi, Ranjan Alok
Department of Neurosurgery, Apollo Health City, Jubilee Hills, Hyderabad, Andhra Pradesh, India.
Department of Pathology, Apollo Health City, Jubilee Hills, Hyderabad, Andhra Pradesh, India.
Ann Indian Acad Neurol. 2015 Jul-Sep;18(3):345-7. doi: 10.4103/0972-2327.152085.
Chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids (CLIPPERS) is a recently described inflammatory disease of central nervous system with distinct clinical and radiological features. The etiopathogenesis of this rare entity remains to be understood. The histopathological findings closely resemble chronic inflammatory diseases like sarcoidosis and malignancies like lymphoma. With advancements in serology, immunopathology and radiology CLIPPERS is identified as a distinct entity that differs considerably in its clinical presentation, immunopathology, radiological findings and response to steroids. We describe a case that presented to us with progressive quadriparesis and lower cranial nerve deficits whose radiological and pathological findings were consistent with CLIPPERS. The patient had a good outcome with long term immunosuppression.
类固醇反应性桥脑周围血管强化的慢性淋巴细胞性炎症(CLIPPERS)是一种最近描述的中枢神经系统炎性疾病,具有独特的临床和放射学特征。这种罕见疾病的病因发病机制仍有待明确。其组织病理学表现与结节病等慢性炎症性疾病以及淋巴瘤等恶性肿瘤相似。随着血清学、免疫病理学和放射学的发展,CLIPPERS被确定为一种独特的疾病实体,其临床表现、免疫病理学、放射学表现及对类固醇的反应均有很大差异。我们描述了一例以进行性四肢瘫和低位颅神经功能缺损就诊的病例,其放射学和病理学表现与CLIPPERS相符。该患者通过长期免疫抑制治疗取得了良好疗效。