Recurrent intracranial hemorrhage and cerebral venous sinus thrombosis: an atypical case of Neuro-Behçet's Syndrome

Clin Neurol Neurosurg. 2020 Apr:191:105680. doi: 10.1016/j.clineuro.2020.105680. Epub 2020 Jan 15.

Abstract

We report the case of a 22-year-old man with an history of headaches, seizures, cognitive impairment associated with recurrent intracranial hemorrhage (ICH), acute ischemic stroke (AIS), worsened over the last eighteen months for a new onset of uveitis and cerebral venous sinus thrombosis (CVST). After excluding common causes of lobar ICH, and some rare ones according to the diagnostic protocol proposed by Beuker et al, in the suspicion of Primary Angiitis of the Central Nervous System (PACNS), the gradual development of a low-grade fever, a cutaneous rash, and a papulopustular manifestation on lower back after rachicentesis (pathergy phenomenon), allowed us to make a diagnosis of Neuro-Behçet's Syndrome (NBS) without oral/genital aphthous ulcerations, according to the International Study Group for Behçet's Disease classification criteria for BD (ICBD).

Keywords: CVST; Neurobehçet; PACNS; anti-TNF-α.

Publication types

  • Case Reports

MeSH terms

  • Behcet Syndrome / diagnosis*
  • Behcet Syndrome / physiopathology
  • Cerebral Angiography
  • Cognitive Dysfunction / physiopathology
  • Headache / physiopathology
  • Humans
  • Intracranial Hemorrhages / diagnostic imaging*
  • Intracranial Hemorrhages / physiopathology
  • Magnetic Resonance Angiography
  • Male
  • Recurrence
  • Seizures / physiopathology
  • Sinus Thrombosis, Intracranial / diagnostic imaging*
  • Sinus Thrombosis, Intracranial / physiopathology
  • Vasculitis, Central Nervous System / diagnosis*
  • Vasculitis, Central Nervous System / physiopathology
  • Young Adult

Supplementary concepts

  • Primary angiitis of the central nervous system