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, Hideki Sato, Hidenori Hattori, Masaki Takao, Shinichi Takahashi, Norihiro Suzuki

Research output: Contribution to journalArticle

Abstract

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.

Original languageEnglish
Pages (from-to)509-514
Number of pages6
JournalClinical Neurology
Volume57
Issue number9
DOIs
Publication statusPublished - 2017

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Central Nervous System Vasculitis
Human Herpesvirus 7
Steroids
Biopsy
Brain
Seizures
Herpesviridae Infections
Antineutrophil Cytoplasmic Antibodies
Plasmapheresis
Magnetic Resonance Angiography
Methylprednisolone
Cerebrum
Therapeutics
Vasculitis
Cyclophosphamide
Headache
Inflammation
Technology
Polymerase Chain Reaction
Antibodies

Keywords

  • Brain biopsy
  • Central nervous system vasculitis (CNSV)
  • Cyclophosphamide
  • HHV-7
  • Steroid-resistance

ASJC Scopus subject areas

  • Clinical Neurology

Cite this

Case report of a 28-year-old male with the rapid progression of steroid-resistant central nervous system vasculitis diagnosed by a brain biopsy. / Takahashi, Keigo; Sato, Hideki; Hattori, Hidenori; Takao, Masaki; Takahashi, Shinichi; Suzuki, Norihiro.

In: Clinical Neurology, Vol. 57, No. 9, 2017, p. 509-514.

Research output: Contribution to journalArticle

Takahashi, Keigo ; Sato, Hideki ; Hattori, Hidenori ; Takao, Masaki ; Takahashi, Shinichi ; Suzuki, Norihiro. / Case report of a 28-year-old male with the rapid progression of steroid-resistant central nervous system vasculitis diagnosed by a brain biopsy. In: Clinical Neurology. 2017 ; Vol. 57, No. 9. pp. 509-514.
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