Table 1. Demographic characteristics and risk factors in acute childhood-onset AIS.
Number of subjects affected is given in parentheses.
| Median age (range) | 8 y (1mo–19 yrs) |
| Sex % males | 49% (30/61) |
| CASCADE Subtype | |
| Cardioembolic 1,2 | 21% (13/61) |
| Arteriopathy3 | 41% (25/61) |
| Unilateral focal cerebral arteriopathy (anterior circulation)2 | 20% (12/61) |
| Bilateral cerebral arteriopathy (anterior circulation)2 | 10% (6/61) |
| Aortic/Cervical Arteriopathy2 | 11% (7/61) |
| Multifactorial | 2% (1/61) |
| Other4 | 31% (19/61) |
| Thrombophilia5 | 63% (32/51) |
| Genetic | 17% (6/35) |
| Acquired, acute | 28% (8/28) |
| Acquired, chronic | 6% (2/29) |
| APS | 14% (8/57) |
| Acute plasma D-dimer > 500 ng/mL6 | 31% (13/42) |
| Rheumatologic condition7 | 7% (4/61) |
| Chromosomal abnormality8 | 3% (2/61) |
| Congenital metabolic disorder9 | 3% (2/61) |
| Malignancy10 | 2% (1/61) |
Abbreviations: AIS=arterial ischemic stroke; APA= antiphospholipid antibodies; FVIII=factor VIII activity.
Includes hypoplastic left heart syndrome (n=3), hypoplastic left heart syndrome status-post cardiac transplantation (n=1), Ebstein’s anomaly (n=1), single ventricle (n=1), pulmonary artery stenosis (n=1), pulmonary atresia (n=1), Ivemark syndrome (n=1), idiopathic dilated cardiomyopathy status-post cardiac transplantation (n=1), dilated cardiomyopathy due to myocarditis (n=1), transposition of the great arteries and coarctation of the aorta (n=1), and transposition of the great arteries and ventricular septal defect (n=1).
Classified according to criteria of Bernard et al, 2011.24
Defined as arteriopathy visualized within 1 week of AIS onset. Patients with late- onset arteriopathy were excluded. Patients without acute imaging were not included in analysis.
Includes APA syndrome (n=3), elevated plasma lipoprotein(a) (n=1), factor V Leiden (n=2), familial migraine syndrome (n=2), head/neck trauma (n=2, one in the setting of a genetic protein C deficiency), acute factor VIII elevation (n=1), panhypopituitarism in the setting of factor V Leiden (n=1), pericarditis in the setting of an acute mycoplasma infection (n=1), and extracorporeal membrane oxygenation (ECMO) in the setting of acute respiratory distress syndrome (ARDS) secondary to Influenza Type A (n=1). Five strokes were without any risk factors.
Patients without complete genetic or acquired testing were excluded.
Values of 500 ng/mL from 2006–2008 were determined to be equivalent to values of 1.0 ug/mL from 2009–2011 by local laboratory standards, via cross-validation of more recent versus prior assay method for semiquantitative latex agglutination assay. Acute elevation was defined as a positive lab result obtained within 24 hours of stroke. See also Methods.
Includes systemic lupus erythematosus (n=2) and polyarteritis nodosa (n=2).
Includes trisomy 21 (n=1) and Turner’s syndrome (n=1).
Includes Alagille syndrome (n=2).
Includes juvenile pilocytic astrocytoma of the hypothalmus (n=1).