Spectrum of cerebral arteriopathies in children with arterial ischemic stroke

Neurology. 2020 Jun 9;94(23):e2479-e2490. doi: 10.1212/WNL.0000000000009557. Epub 2020 May 26.

Abstract

Objective: To determine that children with arterial ischemic stroke (AIS) due to an identifiable arteriopathy are distinct from those without arteriopathy and that each arteriopathy subtype has unique and recognizable clinical features.

Methods: We report a large, observational, multicenter cohort of children with AIS, age 1 month to 18 years, enrolled in the International Pediatric Stroke Study from 2003 to 2014. Clinical and demographic differences were compared by use of the Fisher exact test, with linear step-up permutation min-p adjustment for multiple comparisons. Exploratory analyses were conducted to evaluate differences between cases of AIS with and without arteriopathy and between arteriopathy subtypes.

Results: Of 2,127 children with AIS, 725 (34%) had arteriopathy (median age 7.45 years). Arteriopathy subtypes included dissection (27%), moyamoya (24.5%), focal cerebral arteriopathy-inflammatory subtype (FCA-i; 15%), diffuse cerebral vasculitis (15%), and nonspecific arteriopathy (18.5%). Children with arteriopathic AIS were more likely to present between 6 and 9 years of age (odds ratio [OR] 1.93, p = 0.029) with headache (OR 1.55, p = 0.023), multiple infarctions (OR 2.05, p < 0.001), sickle cell anemia (OR 2.9, p = 0.007), and head/neck trauma (OR 1.93, p = 0.018). Antithrombotic use and stroke recurrence were higher in children with arteriopathy. Among arteriopathy subtypes, dissection was associated with male sex, older age, headache, and anticoagulant use; FCA-i was associated with hemiparesis and single infarcts; moyamoya was associated with seizures and recurrent strokes; and vasculitis was associated with bilateral infarctions.

Conclusion: Specific clinical profiles are associated with cerebral arteriopathies in children with AIS. These observations may be helpful indicators in guiding early diagnosis and defining subgroups who may benefit most from future therapeutic trials.

Publication types

  • Comparative Study
  • Multicenter Study
  • Observational Study
  • Research Support, N.I.H., Extramural
  • Research Support, Non-U.S. Gov't
  • Research Support, U.S. Gov't, P.H.S.

MeSH terms

  • Adolescent
  • Age of Onset
  • Anticoagulants / adverse effects
  • Anticoagulants / therapeutic use
  • Aortic Dissection / complications
  • Aortic Dissection / epidemiology
  • Brain Ischemia / epidemiology
  • Brain Ischemia / etiology*
  • Brain Ischemia / therapy
  • Cerebral Arterial Diseases / complications
  • Cerebral Arterial Diseases / epidemiology*
  • Cerebral Infarction / epidemiology
  • Cerebral Infarction / etiology
  • Child
  • Child, Preschool
  • Female
  • Fibrinolytic Agents / therapeutic use
  • Global Health
  • Headache / epidemiology
  • Headache / etiology
  • Humans
  • Infant
  • Infant, Newborn
  • Intracranial Aneurysm / complications
  • Intracranial Aneurysm / epidemiology
  • Male
  • Prospective Studies
  • Recurrence
  • Registries
  • Risk Factors
  • Treatment Outcome
  • Vasculitis, Central Nervous System / complications
  • Vasculitis, Central Nervous System / epidemiology

Substances

  • Anticoagulants
  • Fibrinolytic Agents