Takahashi Keigo, Sato Hideki, Hattori Hidenori, Takao Masaki, Takahashi Shinichi, Suzuki Norihiro
Department of Neurology, Saitama Municipal Hospital.
Department of Neurology, Saitama Medical University International Medical Center.
Rinsho Shinkeigaku. 2017 Sep 30;57(9):509-514. doi: 10.5692/clinicalneurol.cn-001034. Epub 2017 Aug 11.
A 28-year-old Japanese male without a significant past medical history presented with new-onset generalized clonic seizure and headache. A brain MRI revealed multiple enhanced lesions on both cerebral hemispheres. Laboratory exams showed no evidence of systemic inflammation or auto-immune antibodies such as ANCAs. Despite four courses of high-dose methylprednisolone pulse therapy and five treatments with plasmapheresis, his symptoms worsened and the MRI lesions progressed rapidly. During these treatments, we performed a targeted brain biopsy, that revealed histological findings consistent with a predominant angiitis of parenchymal and subdural small vessels. He was provided with diagnosis of central nervous system vasculitis (CNSV). Subsequent cyclophosphamide pulse therapy enabled a progressive successful improvement of his symptoms. While diagnostic methods for CNSV remain controversial, histological findings are thought to be more useful in obtaining a more definitive diagnosis than findings in image studies, such as MRI and angiography. We suggest that a brain biopsy should be considered during the early period of cases with suspected CNSV and rapid clinical deterioration. We also detected human herpesvirus 7 (HHV-7) using PCR technology in brain biopsy specimens, however the relationship between CNSV and HHV-7 infection is unknow.
一名28岁无重大既往病史的日本男性，出现新发全身性阵挛性癫痫发作和头痛。脑部MRI显示双侧大脑半球有多个强化病灶。实验室检查未发现全身炎症或自身免疫抗体（如抗中性粒细胞胞浆抗体）的证据。尽管接受了四个疗程的大剂量甲泼尼龙冲击治疗和五次血浆置换治疗，他的症状仍恶化，MRI病灶迅速进展。在这些治疗过程中，我们进行了靶向脑活检，结果显示组织学 findings 与实质性和硬膜下小血管为主的血管炎一致。他被诊断为中枢神经系统血管炎（CNSV）。随后的环磷酰胺冲击治疗使他的症状逐渐得到成功改善。虽然CNSV的诊断方法仍存在争议，但组织学 findings 被认为比MRI和血管造影等影像学检查结果在获得更明确的诊断方面更有用。我们建议，对于疑似CNSV且临床迅速恶化的病例，应在早期考虑进行脑活检。我们还在脑活检标本中使用PCR技术检测到了人类疱疹病毒7（HHV-7），然而CNSV与HHV-7感染之间的关系尚不清楚。
