Clinical and radiological features and details of treatment in patients with cervical ICA stenosis or occlusion
| Patient no. . | Age*/sex . | Clinical events . | Cranial MRI . | Cranial MRA . | Cervical MRA . | Management . |
|---|---|---|---|---|---|---|
| 1 | 16/M | Age 3: TIA (ataxia) Age 16: left hemiparesis Age 20: Recurrent TIAs | Age 3: Normal Age 16: Subsegmental right MCA infarct, DWS left ischemic lesions unchanged at age 20 | No evidence of intracranial vascular stenosis age 3, 16, and 20 | Occlusive lesion proximal right cICA, residual stump at origin of right cICA (Figure 1A), persisting occlusion age 20 | Acute exchange transfusion followed by chronic transfusion program, anticoagulation for 3 mo, aspirin from age 20 |
| 2 | 13/M | Age 13: left hemiparesis, right headache, swallowing difficulties, absent gag Age 14: Recurrent AIS | Age 13 (after AIS): large acute infarct in right MCA territory | No evidence of intracranial vascular stenosis age 13 and 14 | Occlusive lesion proximal right cICA, tapering narrowing of residual stump, persisting occlusion at age 14 | Acute exchange transfusion followed by chronic transfusion program, anticoagulation for 3 mo |
| 3 | 4/F | Age 4: TIA followed by left hemiparesis, left VII, left XII 2 mo later | Extensive left cortical and DWS infarct unchanged at age 5 and age 7 | Minor irregularity of left MCA Normal right-sided vessels | Occlusive lesion proximal left cICA, tapering narrowing of residual stump, persisting occlusion at age 5 and 7 | Acute exchange transfusion followed by chronic transfusion program, anticoagulation for 3 mo |
| 4 | 4/F | Age 4: right hemiparesis, right VII 2 weeks after Varicella, URTI, bilateral cervical lymphadenopathy (recurrent AIS 3 mo) | Extensive left cortical and DWS infarct in left MCA and ACA territories, area-restricted diffusion within previously infarcted area on follow-up scan with recurrent AIS | Attenuated left MCA but not focally stenotic, mild stenosis of right ACA | Severe stenotic lesion proximal left cICA, flame-shaped proximal vessel, and distal string sign (Figure 2), thrombus within cICA at presentation with AIS, partial recanalization of vessel on follow-up age 5 and 6 | Acute exchange transfusion followed by chronic transfusion program |
| 5 | 5/M | Age 2: Probable AIS (acute neurological event in Africa) | Mature infarct left ACA/MCA watershed distribution | No intracranial vasculopathy | Severe stenotic lesion in distal left cICA Tortuous vertebral arteries and prominent tortuous anterior spinal artery (Figure 3) | Chronic transfusion program |
| 6 | 20/M | Age 2 and age 7 transient right hemiplegia, behavioral problems, deteriorating IQ | Age 7-15: annual or biannual scans, bilateral DWS infarcts and left caudate. Age 20: extensive infarction in left DWS and cortex, and right-sided infarction in DWS and focally in occipital cortex | Turbulent flow cranial ICA bilaterally from age 7 to 15, no apparent cerebrovascular disease age 20 | Severe stenotic lesion proximal left cICA, flame-shaped proximal vessel, and distal string sign (Figure 1B) | Chronic transfusion program age 7-14, left-sided EDAS procedure at age 11 Hydroxyurea and venesections at age 15-20 Aspirin and hydroxyurea from age 20 |
| 7 | 34/F | Age 22: Seizure, collapse Age 34: TIA | Age 22 (after AIS): Focal infarct right parietal cortex Age 27: Bilateral cortical ischemia worse on right side, unchanged at age 34 | No stenotic lesions in intracranial vessels | Severe stenotic lesion proximal right cICA, flame-shaped proximal vessel, and distal string sign (Figure 1C) | Regular exchange transfusions at age 22 Aspirin added at age 34 |
| 8 | 12/M | Age 11: left hemiparesis, left VII, left XII | Age 12: right frontal cortical infarct, small lesion in left DWS | Moderate right terminal ICA stenosis | Occlusion of proximal right cICA, flame-shaped appearance of residual stump | Chronic transfusion program at age 11 |
| 9 | 14/F | None | No ischemic change age 10, 14, and 16 | Normal intracranial vessels at age 10, 14, and 16 | Severe stenotic lesion proximal right cICA, attenuation of distal vessel (Figure 1D), uchanged at age 16 | Chronic top-up transfusion program at age 14 |
| 10 | 8/M | None | Right DWS ischemia | Normal intracranial vessels | Moderate stenotic lesion in distal right cICA (Figure 1E) | Regular top-up transfusion program at age 8 |
| Patient no. . | Age*/sex . | Clinical events . | Cranial MRI . | Cranial MRA . | Cervical MRA . | Management . |
|---|---|---|---|---|---|---|
| 1 | 16/M | Age 3: TIA (ataxia) Age 16: left hemiparesis Age 20: Recurrent TIAs | Age 3: Normal Age 16: Subsegmental right MCA infarct, DWS left ischemic lesions unchanged at age 20 | No evidence of intracranial vascular stenosis age 3, 16, and 20 | Occlusive lesion proximal right cICA, residual stump at origin of right cICA (Figure 1A), persisting occlusion age 20 | Acute exchange transfusion followed by chronic transfusion program, anticoagulation for 3 mo, aspirin from age 20 |
| 2 | 13/M | Age 13: left hemiparesis, right headache, swallowing difficulties, absent gag Age 14: Recurrent AIS | Age 13 (after AIS): large acute infarct in right MCA territory | No evidence of intracranial vascular stenosis age 13 and 14 | Occlusive lesion proximal right cICA, tapering narrowing of residual stump, persisting occlusion at age 14 | Acute exchange transfusion followed by chronic transfusion program, anticoagulation for 3 mo |
| 3 | 4/F | Age 4: TIA followed by left hemiparesis, left VII, left XII 2 mo later | Extensive left cortical and DWS infarct unchanged at age 5 and age 7 | Minor irregularity of left MCA Normal right-sided vessels | Occlusive lesion proximal left cICA, tapering narrowing of residual stump, persisting occlusion at age 5 and 7 | Acute exchange transfusion followed by chronic transfusion program, anticoagulation for 3 mo |
| 4 | 4/F | Age 4: right hemiparesis, right VII 2 weeks after Varicella, URTI, bilateral cervical lymphadenopathy (recurrent AIS 3 mo) | Extensive left cortical and DWS infarct in left MCA and ACA territories, area-restricted diffusion within previously infarcted area on follow-up scan with recurrent AIS | Attenuated left MCA but not focally stenotic, mild stenosis of right ACA | Severe stenotic lesion proximal left cICA, flame-shaped proximal vessel, and distal string sign (Figure 2), thrombus within cICA at presentation with AIS, partial recanalization of vessel on follow-up age 5 and 6 | Acute exchange transfusion followed by chronic transfusion program |
| 5 | 5/M | Age 2: Probable AIS (acute neurological event in Africa) | Mature infarct left ACA/MCA watershed distribution | No intracranial vasculopathy | Severe stenotic lesion in distal left cICA Tortuous vertebral arteries and prominent tortuous anterior spinal artery (Figure 3) | Chronic transfusion program |
| 6 | 20/M | Age 2 and age 7 transient right hemiplegia, behavioral problems, deteriorating IQ | Age 7-15: annual or biannual scans, bilateral DWS infarcts and left caudate. Age 20: extensive infarction in left DWS and cortex, and right-sided infarction in DWS and focally in occipital cortex | Turbulent flow cranial ICA bilaterally from age 7 to 15, no apparent cerebrovascular disease age 20 | Severe stenotic lesion proximal left cICA, flame-shaped proximal vessel, and distal string sign (Figure 1B) | Chronic transfusion program age 7-14, left-sided EDAS procedure at age 11 Hydroxyurea and venesections at age 15-20 Aspirin and hydroxyurea from age 20 |
| 7 | 34/F | Age 22: Seizure, collapse Age 34: TIA | Age 22 (after AIS): Focal infarct right parietal cortex Age 27: Bilateral cortical ischemia worse on right side, unchanged at age 34 | No stenotic lesions in intracranial vessels | Severe stenotic lesion proximal right cICA, flame-shaped proximal vessel, and distal string sign (Figure 1C) | Regular exchange transfusions at age 22 Aspirin added at age 34 |
| 8 | 12/M | Age 11: left hemiparesis, left VII, left XII | Age 12: right frontal cortical infarct, small lesion in left DWS | Moderate right terminal ICA stenosis | Occlusion of proximal right cICA, flame-shaped appearance of residual stump | Chronic transfusion program at age 11 |
| 9 | 14/F | None | No ischemic change age 10, 14, and 16 | Normal intracranial vessels at age 10, 14, and 16 | Severe stenotic lesion proximal right cICA, attenuation of distal vessel (Figure 1D), uchanged at age 16 | Chronic top-up transfusion program at age 14 |
| 10 | 8/M | None | Right DWS ischemia | Normal intracranial vessels | Moderate stenotic lesion in distal right cICA (Figure 1E) | Regular top-up transfusion program at age 8 |
TIA indicates transient ischemic attack; VII, 7th nerve palsy; XII, 12th nerve palsy; DWS, deep watershed; and EC-IC, extracranial-intracranial.
Age is at the time of cICA lesion diagnosis.