Isolated Central Nervous System Vasculitis as a Manifestation of Neurosarcoidosis

J Stroke Cerebrovasc Dis. 2016 Jun;25(6):e89-92. doi: 10.1016/j.jstrokecerebrovasdis.2016.02.036. Epub 2016 Apr 12.

Abstract

A 62-year-old male presented to our clinic with recurrent fever, skin lesions (petechiae), scleral wounds, and hilar adenomegaly. A diagnosis of sarcoidosis was established, which resolved with corticosteroid treatment. After a few months, the patient developed confusion and behavioral changes, with few objective neurological deficits. Brain magnetic resonance imaging showed slight focal meningeal enhancement (prepontine region). The level of angiotensin-converting enzymes was normal in the serum and increased in the cerebrospinal fluid. The patient was diagnosed with neurosarcoidosis, and corticosteroid treatment was prescribed, yielding good clinical response. Nine months later, the patient presented with multiple ischemic strokes, and arteriography demonstrated multiple distal irregularities in all arterial territories, suggesting cerebral vasculitis. Even with corticosteroids, cyclophosphamide, and intravenous immunoglobulin, the patient died. Vasculitis is rarely seen in association with sarcoidosis, and in this case, no systemic manifestation was observed at the time that the patient developed vasculitis.

Keywords: Sarcoidosis; angiitis; neurosarcoidosis; vasculitis.

Publication types

  • Case Reports

MeSH terms

  • Adrenal Cortex Hormones / therapeutic use
  • Brain Ischemia / etiology
  • Central Nervous System Diseases / complications*
  • Central Nervous System Diseases / diagnosis
  • Central Nervous System Diseases / drug therapy
  • Cerebral Angiography
  • Cyclophosphamide / therapeutic use
  • Disease Progression
  • Fatal Outcome
  • Humans
  • Immunoglobulins, Intravenous
  • Immunosuppressive Agents / therapeutic use
  • Magnetic Resonance Imaging
  • Male
  • Middle Aged
  • Sarcoidosis / complications*
  • Sarcoidosis / diagnosis
  • Sarcoidosis / drug therapy
  • Stroke / etiology
  • Time Factors
  • Tomography, X-Ray Computed
  • Treatment Outcome
  • Vasculitis, Central Nervous System / diagnosis
  • Vasculitis, Central Nervous System / drug therapy
  • Vasculitis, Central Nervous System / etiology*

Substances

  • Adrenal Cortex Hormones
  • Immunoglobulins, Intravenous
  • Immunosuppressive Agents
  • Cyclophosphamide

Supplementary concepts

  • Neurosarcoidosis