A Unique Recurrent Stroke Case due to Bilateral Vertebral Artery Dissection with Familial Hirschsprung Disease

J Stroke Cerebrovasc Dis. 2019 Aug;28(8):e113-e115. doi: 10.1016/j.jstrokecerebrovasdis.2019.04.035. Epub 2019 May 22.

Abstract

Vertebral artery (VA) dissection is one major cause of brain infarction in young and middle-aged adults. Risk factors for VA dissection are hypertension, diabetes mellitus, hyperlipidemia, trauma, and genetic factors. A 32-year-old man with familial Hirschsprung disease at the age of 2 presented cerebellar ischemic stroke due to bilateral VA dissections. A stroke recurred within 17 days despite oral dual antiplatelet therapy. Bilateral VA dissections and recurrent dissections are related to genetic mutations associated with connective tissue diseases. A part of familial Hirschsprung disease has genetic factors in common with cerebrovascular disease. There may be a common genetic background between his VA dissection and Hirschsprung disease.

Keywords: Bilateral vertebral arteries; Dual antiplatelet therapy; Familial Hirschsprung disease; Fibromuscular dysplasia; Vertebral artery dissection.

Publication types

  • Case Reports

MeSH terms

  • Adult
  • Aspirin / therapeutic use
  • Cerebral Angiography / methods
  • Cerebral Infarction / diagnostic imaging
  • Cerebral Infarction / drug therapy
  • Cerebral Infarction / etiology*
  • Cilostazol / therapeutic use
  • Diffusion Magnetic Resonance Imaging
  • Edaravone / therapeutic use
  • Genetic Predisposition to Disease
  • Hirschsprung Disease / complications*
  • Hirschsprung Disease / diagnosis
  • Hirschsprung Disease / genetics
  • Humans
  • Magnetic Resonance Angiography
  • Male
  • Neuroprotective Agents / therapeutic use
  • Phenotype
  • Platelet Aggregation Inhibitors / therapeutic use
  • Recurrence
  • Treatment Outcome
  • Vertebral Artery Dissection / diagnostic imaging
  • Vertebral Artery Dissection / drug therapy
  • Vertebral Artery Dissection / etiology*

Substances

  • Neuroprotective Agents
  • Platelet Aggregation Inhibitors
  • Cilostazol
  • Aspirin
  • Edaravone