Table 1.
Arteriopathy subtype, associated medical diagnoses and thrombophilia
| Arteriopathy subtype | N |
|---|---|
| Moyamoya arteriopathy | 14 |
| Idiopathic (9) | |
| Hemoglobinopathy (2) | |
| Fibromuscular dysplasia, systemic (1) | |
| NF1 with cranial radiation (2) | |
| Focal cerebral arteriopathy (MCA, ICA) | 9 |
| Infectious arteritis | 4 |
| Bacterial meningitis (3) | |
| HIV (1) | |
| Focal or multifocal vessel occlusion/stenosis from intracranial extension or embolism from cervical vessel disease | 12 |
| Cervical carotid artery dissection, confirmed (1) | |
| Cervical vertebral artery dissection, confirmed (7) | |
| Cervical vertebral artery dissection, suspected (4) | |
| Other focal or multifocal vessel occlusion/stenosis: | 10 |
| Isolated vessel hypoplasia or stenosis in posterior circulation (4) | |
| Other COW vessel occlusion, suspected embolic or thrombotic (6) | |
| Associated medical diagnoses* | N |
| NF1 | 2 |
| Hemoglobinopathy | 3 |
| Trisomy 21 | 3 |
| Hypertension | 4 |
| Immunologic disorder† | 3 |
| Thrombophilia | N |
| Factor V Leiden mutation heterozygote | 3 |
| Prothrombin gene mutation heterozygote | 1 |
| Elevated lipoprotein a (>30 mg/dL) | 14 |
| Lupus anticoagulant (elevated dRVVT) | 10 |
| Elevated anticardiolipin Ab | 4 |
| Elevated beta-2 glycoprotein Ab | 4 |
| Protein S deficiency | 5 |
| Antithrombin III deficiency | 1 |
| Elevated factor VIII | 1 |
*
Associated medical conditions and thrombophilias are not mutually exclusive and may be multiple in a given patient.
†
Immunologic disorders include HIV, autoimmune lymphoproliferative syndrome, and SAMHD1-mutation-related systemic autoimmune disease.
MCA, middle cerebral artery; ICA, internal carotid artery; COW, circle of Willis; HTN, hypertension; NF1, neurofibromatosis type I; dRVVT, dilute Russell’s viper venom time; Ab, antibody.