title: Early-onset bilateral cerebral arteriopathies: Cohort study of phenotype and disease course.
abstract: OBJECTIVE
  To describe characteristics of young children with arterial ischemic stroke (AIS) and bilateral cerebral arteriopathies.
  
  
  METHODS
  Retrospective review of clinical features, course, and outcome. Neuroimaging was analyzed for infarct pattern, cerebrovascular diagnosis (anatomic/Childhood Arterial Ischemic Stroke Standardized Classification and Diagnostic Evaluation [CASCADE] criteria), and disease progression.
  
  
  RESULTS
  In the 31 children (median age, 18 months), presentations included acute hemiparesis (n=23) and focal seizures (n=12). Seven had systemic arterial disease; 13 had cardiac abnormalities. Twenty had recurrent AIS or transient ischemic attack (after median of 3 months); 16 had >1 recurrence. Median modified Rankin Scale score was 3, with motor impairments in 20, cognitive impairments in 11, and seizures in 7. At presentation, 17 had old and acute infarcts. Twenty-five had high signal in white matter. A total of 13/23 reimaged patients accrued further infarcts over a median of 39 months. Arteriopathy involved the carotid circulation bilaterally in all; 6 had posterior circulation and 11 had extracranial involvement. Arteriopathy distribution was symmetric in 24/31. CASCADE categories were 3A in 19, 3B in 5, 3C in 5, and 7 in 2. After a median of 35 months, 14 had had progression of arteriopathy. Patients categorized as CASCADE 3A (moyamoya) had significantly shorter time to recurrence than other groups.
  
  
  CONCLUSIONS
  Young children with bilateral cerebral arteriopathies (particularly meeting criteria for CASCADE 3A) have a malignant course, with frequent recurrent events, progressive disease, and poor outcomes. Current classifications are limited in characterizing disease in many cases. Symmetric involvement suggests these arteriopathies may be developmentally determined, while systemic involvement suggests potential genetic etiology.
authors: From the Neurosciences Unit (A.A.-Y., V.G.), UCL Institute of Child Health; the Radiology Department (D.E.S.), Great Ormond Street Hospital, NHS Foundation Trust, London, UK; and the Neurology Department (M.T.M.), Royal Children's Hospital, Melbourne, Australia.
authors: Amina
authors: A
authors: Al-Yassin
authors: From the Neurosciences Unit (A.A.-Y., V.G.), UCL Institute of Child Health; the Radiology Department (D.E.S.), Great Ormond Street Hospital, NHS Foundation Trust, London, UK; and the Neurology Department (M.T.M.), Royal Children's Hospital, Melbourne, Australia.
authors: Dawn E
authors: DE
authors: Saunders
authors: From the Neurosciences Unit (A.A.-Y., V.G.), UCL Institute of Child Health; the Radiology Department (D.E.S.), Great Ormond Street Hospital, NHS Foundation Trust, London, UK; and the Neurology Department (M.T.M.), Royal Children's Hospital, Melbourne, Australia.
authors: Mark T
authors: MT
authors: Mackay
authors: From the Neurosciences Unit (A.A.-Y., V.G.), UCL Institute of Child Health; the Radiology Department (D.E.S.), Great Ormond Street Hospital, NHS Foundation Trust, London, UK; and the Neurology Department (M.T.M.), Royal Children's Hospital, Melbourne, Australia. v.ganesan@ucl.ac.uk.
authors: Vijeya
authors: V
authors: Ganesan
mesh_terms: D020520:Brain Infarction / Q000473:pathology / Q000503:physiopathology; D002545:Brain Ischemia / Q000473:pathology* / Q000503:physiopathology; D002539:Cerebral Arterial Diseases / Q000473:pathology* / Q000503:physiopathology; D002675:Child, Preschool; D018450:Disease Progression; D005260:Female; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D008297:Male; D009072:Moyamoya Disease / Q000473:pathology / Q000503:physiopathology; D012008:Recurrence; D020521:Stroke / Q000473:pathology* / Q000503:physiopathology