Rinsho Shinkeigaku (Clinical Neurology)

Case Report

Case report of a 28-year-old male with the rapid progression of steroid-resistant central nervous system vasculitis diagnosed by a brain biopsy

Keigo Takahashi, M.D.1), Hideki Sato, M.D.1), Hidenori Hattori, M.D., Ph.D.1), Masaki Takao, M.D., Ph.D.2), Shinichi Takahashi, M.D., Ph.D.3) and Norihiro Suzuki, M.D., Ph.D.3)

1)Department of Neurology, Saitama Municipal Hospital
2)Department of Neurology, Saitama Medical University International Medical Center
3)Department of Neurology, Keio University School of Medicine

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 highdose 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.
Full Text of this Article in Japanese PDF (1093K)

(CLINICA NEUROL, 57: 509|514, 2017)
key words: central nervous system vasculitis (CNSV), steroid-resistance, brain biopsy, cyclophosphamide, HHV-7

(Received: 21-Mar-17)