Mizuno Yuri, Shigeto Hiroshi, Yamada Takeshi, Maeda Norihisa, Suzuki Satoshi O, Kira Jun-ichi
Department of Neurology, Neurological Institute, Graduate School of Medical Sciences, Kyushu University.
Rinsho Shinkeigaku. 2016;56(3):186-90. doi: 10.5692/clinicalneurol.cn-000847. Epub 2016 Mar 8.
We report a case of primary central nervous system vasculitis (PCNSV) diagnosed by second brain biopsy. A 53-year-old man initially presented with left lateral gaze diplopia. Brain MRI revealed multiple enhanced lesions in the bilateral frontal lobe, bilateral basal ganglia, left cerebellum and brainstem. An initial brain biopsy of the right frontal lobe suggested immune-related encephalitis with angiocentric accumulation of chronic inflammatory cells, while malignant lymphoma could not be completely ruled out. The patient deteriorated despite being treated with repeated methylprednisolone pulse therapy, cyclophosphamide, and plasmapheresis. A second brain biopsy of the right temporal lobe was then performed. The biopsied specimens showed vascular wall disruption and fibrinoid necrosis with perivascular inflammatory infiltrates, mainly composed of CD8-positive T cells, and PCNSV was diagnosed. He was treated with high dose corticosteroids, in combination with methotrexate (8 mg/week), which reduced the brain lesions. As brain biopsy is an essential investigation for the histological diagnosis of PCNSV; subsequent biopsies may be required when a histopathological diagnosis has not been obtained by the first biopsy, and further aggressive therapy is being considered.
我们报告一例经第二次脑活检确诊的原发性中枢神经系统血管炎（PCNSV）。一名53岁男性最初表现为左侧凝视复视。脑部MRI显示双侧额叶、双侧基底节、左侧小脑和脑干有多个强化病灶。右侧额叶的首次脑活检提示为免疫相关性脑炎，伴有慢性炎性细胞的血管中心性聚集，同时不能完全排除恶性淋巴瘤。尽管接受了反复的甲泼尼龙冲击治疗、环磷酰胺和血浆置换治疗，患者病情仍恶化。随后对右侧颞叶进行了第二次脑活检。活检标本显示血管壁破坏和纤维蛋白样坏死，伴有血管周围炎性浸润，主要由CD8阳性T细胞组成，诊断为PCNSV。他接受了大剂量皮质类固醇联合甲氨蝶呤（8毫克/周）治疗，脑部病灶有所减少。由于脑活检是PCNSV组织学诊断的重要检查；当首次活检未获得组织病理学诊断且考虑进一步积极治疗时，可能需要进行后续活检。
