A Case of Cerebral Large-Vessel Vasculitis Concomitant Fahr Syndrome in Systemic Lupus Erythematosus

Neurologist. 2024 Jan 1;29(1):17-21. doi: 10.1097/NRL.0000000000000520.

Abstract

Introduction: Systemic lupus erythematosus (SLE) is a heterogenous, devastating autoimmune inflammatory disease with multiorgan involvement. A variety of neurological and psychiatric symptoms may be caused by nervous system involvement, termed neuropsychiatric systemic lupus erythematosus.

Case report: We describe a young man newly diagnosed with SLE who had a stroke as an initial symptom and was found to have cerebral large-vessel vasculitis and Fahr syndrome.

Conclusions: The novelties of this report are the extensive cerebral calcification demonstrated on head computerized tomography in a patient with SLE, and the depiction of an underlying vasculitis on high-resolution magnetic resonance vessel wall imaging. It is our aim to describe this atypical form of neuropsychiatric systemic lupus erythematosus onset and to make known the usefulness of the new magnetic resonance imaging techniques for the diagnosis of cerebral large-vessel vasculitis.

Publication types

  • Case Reports

MeSH terms

  • Humans
  • Lupus Erythematosus, Systemic* / complications
  • Lupus Erythematosus, Systemic* / diagnostic imaging
  • Lupus Vasculitis, Central Nervous System* / complications
  • Lupus Vasculitis, Central Nervous System* / diagnostic imaging
  • Magnetic Resonance Imaging
  • Male
  • Vasculitis, Central Nervous System* / complications
  • Vasculitis, Central Nervous System* / diagnostic imaging

Supplementary concepts

  • Fahr's disease