Table 2.

"Gold standard" arteriopathy diagnosis in 355 childhood arterial ischemic stroke cases

		n	(%)
Source of "gold standard" arteriopathy classification
Vascular imaging review (normal, no arteriopathy review)		116	(32.7)
Arteriopathy diagnosis review		239	(67.3)
Baseline with clinical findings (Step 2)		94	(26.5)
Follow-up (Step 3)		89	(25.1)
Adjudication		56	(15.8)
Definite arteriopathy		127	(35.8)
Secondary diagnosis classified with high certainty*		109	(30.7)
Arterial dissection		26	(7.3)
Transient cerebral arteriopathy		25	(7.0)
Primary moyamoya		17	(4.8)
Secondary moyamoya		17	(4.8)
PHACES		2	(0.6)
Genetic arteriopathy		4	(1.1)
Primary vasculitis		0	(0)
Secondary vasculitis (including meningitis)		15	(4.2)
Iatrogenic		1	(0.3)
Fibromuscular dysplasia		2	(0.6)
Secondary diagnosis not further classified		18	(5.1)
Differential diagnosis includes:**
Transient cerebral arteriopathy		9	(2.5)
Arterial dissection		11	(3.1)
Primary moyamoya		4	(1.1)
Secondary moyamoya		0	(0)
Genetic arteriopathy		1	(0.3)
Primary vasculitis		2	(0.6)
Secondary vasculitis (including meningitis)		4	(1.1)
Iatrogenic		0	(0)
Possible arteriopathy		34	(9.6)
Differential diagnosis includes:**
Transient cerebral arteriopathy		9	(2.5)
Arterial dissection		27	(7.6)
Primary moyamoya		1	(0.3)
Secondary moyamoya		0	(0)
Genetic arteriopathy		0	(0)
Primary vasculitis		1	(0.3)
Secondary vasculitis (including meningitis)		7	(2.0)
Iatrogenic		0	(0)
Embolic (not arteriopathy)		33	(9.3)
No arteriopathy		194	(54.6)
No abnormalities on vascular imaging (no arteriopathy review)		116	(32.7)
Arteriopathy review		78	(22.0)
Classified as no arteriopathy, no embolus		1	(0.3)
Classified as no arteriopathy, low likelihood embolic		2	(0.6)
Classified as isolated occlusion, high likelihood embolic		75	(21.1)

^*Sub-categories are mutually exclusive

^**Sub-categories are not mutually exclusive