A Rare Complication of Sarcoidosis

J Stroke Cerebrovasc Dis. 2018 Nov;27(11):2977-2978. doi: 10.1016/j.jstrokecerebrovasdis.2018.06.028. Epub 2018 Aug 2.

Abstract

Background: Cerebral vasculitis is a serious, but uncommon inflammatory condition of the blood vessel walls, with an annual incidence of 1-2 per million. A variety of disorders including encephalopathy, stroke, seizure, acute or subacute focal deficits should be considered as a differential diagnosis.

Clinical case: A 56-year old male with a past history of pulmonary sarcoid presented with a unilateral facial numbness and loss of balance arising during sleep. His computed tomography scan of the brain was normal but brain magnetic resonance imaging with gadolinium demonstrated scattered infarcts in mixture of stroke topography not purely in keeping with embolism nor intrinsic small vessel disease. Further investigations including carotid ultrasound, transthoracic echo and 24-hour electrocardiogram were within normal limits. However, computed tomography angiography showed evidence of a widespread intracranial vasculopathy, as well as evidence of dissection of the left common carotid artery. His elevated calcium was consistence with a sarcoidosis relapse and cerebrospinal fluid analysis was in keeping with a central nervous system inflammatory process. Treatment was commenced with high dose steroids with additional pulsed intravenous cyclophosphamide together with antiplatelet therapy and a statin.

Conclusions: This case illustrates the intracranial vasculopathy as a rare complication of sarcoidosis. Although sarcoid is well recognized to affect the central nervous system, it is unusual in the form of cerebral vasculitis.

Keywords: CNS vasculitis; Stroke; angiitis; sarcoidosis.

Publication types

  • Case Reports

MeSH terms

  • Cerebral Angiography / methods
  • Computed Tomography Angiography
  • Cyclophosphamide / administration & dosage
  • Echocardiography
  • Humans
  • Hydroxymethylglutaryl-CoA Reductase Inhibitors / therapeutic use
  • Immunosuppressive Agents / administration & dosage
  • Magnetic Resonance Imaging
  • Male
  • Middle Aged
  • Platelet Aggregation Inhibitors / therapeutic use
  • Pulse Therapy, Drug
  • Sarcoidosis, Pulmonary / complications*
  • Sarcoidosis, Pulmonary / diagnosis
  • Sarcoidosis, Pulmonary / drug therapy
  • Steroids / administration & dosage
  • Vasculitis, Central Nervous System / diagnostic imaging
  • Vasculitis, Central Nervous System / drug therapy
  • Vasculitis, Central Nervous System / etiology*

Substances

  • Hydroxymethylglutaryl-CoA Reductase Inhibitors
  • Immunosuppressive Agents
  • Platelet Aggregation Inhibitors
  • Steroids
  • Cyclophosphamide