Clinical features and outcome in North American adults with idiopathic basal arterial occlusive disease without moyamoya collaterals

Abstract

Objective: To define the clinical characteristics and outcome of patients with idiopathic stenosis or occlusion of the basal arteries, without moyamoya collateral vessel formation.

Methods: We identified patients who presented to our institution from 1996 to 2005 with occlusive disease of the distal internal carotid artery or the proximal middle or anterior cerebral arteries demonstrated by digital subtraction cerebral angiography. We excluded those with evidence of atherosclerotic disease, systemic vasculitis, moyamoya phenomenon, or any other condition that could otherwise explain their arterial occlusive disease. Medical records were reviewed for presenting symptoms and clinical characteristics. Outcome was determined from chart review and phone interviews.

Results: Twelve patients were identified. All presented with transient ischemic attack or stroke. Eleven were women. Age at presentation ranged from 34 to 71 years. Nine had a history of hypertension; 5 had unilateral intracranial disease. Recurrent stroke on medical therapy occurred in none of the 5 during an average follow-up of 29 months. Seven had bilateral disease. Ischemic stroke occurred between 2 and 107 months after the initial event in 5 of 8 medically treated hemispheres. Moyamoya collateral vessels developed in 1 patient as shown on follow-up angiography.

Conclusion: The clinical features and outcome of these patients are similar to those reported in large case series of North American patients with moyamoya phenomenon. These data suggest a common etiology for the basal arterial occlusive process and a variable ability to form moyamoya collateral vessels.

Conflict of interest statement

Conflicts of Interest/Financial Disclosure: None

Figures

Figure 1. Development of moyamoya collaterals in a North American adult

Legend: A 45 year old female patient initially presented with ischemic stroke. Cerebral angiography demonstrated proximal stenosis of the middle cerebral arteries bilaterally (A and V). Figure 1A, AP projection after right common carotid artery injection, demonstrates severe narrowing of the proximal middle cerebral artery (black arrow). Figure 1B, AP projection after left common carotid artery injection, shows similar changes of the distal internal and proximal middle and anterior cerebral arteries (black arrow). She was treated with steroids for presumed vasculitis. On repeat angiography approximately 6 months later, the stenotic lesions markedly progressed with the interval development of moyamoya collaterals (C and D). Figure 1C, AP projection after right common carotid artery injection, shows occlusion of the distal internal carotid artery (asterisk) with moyamoya collaterals (black arrow). Figure 1D, AP projection after left common carotid artery injection, demonstrates similar changes (middle cerebral artery occlusion with moyamoya collateral formation, asterisk) with pial collaterals from the posterior artery branches to the middle cerebral artery territory (black arrow).

Figure 1. Development of moyamoya collaterals in a North American adult

Legend: A 45 year old female patient initially presented with ischemic stroke. Cerebral angiography demonstrated proximal stenosis of the middle cerebral arteries bilaterally (A and V). Figure 1A, AP projection after right common carotid artery injection, demonstrates severe narrowing of the proximal middle cerebral artery (black arrow). Figure 1B, AP projection after left common carotid artery injection, shows similar changes of the distal internal and proximal middle and anterior cerebral arteries (black arrow). She was treated with steroids for presumed vasculitis. On repeat angiography approximately 6 months later, the stenotic lesions markedly progressed with the interval development of moyamoya collaterals (C and D). Figure 1C, AP projection after right common carotid artery injection, shows occlusion of the distal internal carotid artery (asterisk) with moyamoya collaterals (black arrow). Figure 1D, AP projection after left common carotid artery injection, demonstrates similar changes (middle cerebral artery occlusion with moyamoya collateral formation, asterisk) with pial collaterals from the posterior artery branches to the middle cerebral artery territory (black arrow).

Figure 1. Development of moyamoya collaterals in a North American adult

Legend: A 45 year old female patient initially presented with ischemic stroke. Cerebral angiography demonstrated proximal stenosis of the middle cerebral arteries bilaterally (A and V). Figure 1A, AP projection after right common carotid artery injection, demonstrates severe narrowing of the proximal middle cerebral artery (black arrow). Figure 1B, AP projection after left common carotid artery injection, shows similar changes of the distal internal and proximal middle and anterior cerebral arteries (black arrow). She was treated with steroids for presumed vasculitis. On repeat angiography approximately 6 months later, the stenotic lesions markedly progressed with the interval development of moyamoya collaterals (C and D). Figure 1C, AP projection after right common carotid artery injection, shows occlusion of the distal internal carotid artery (asterisk) with moyamoya collaterals (black arrow). Figure 1D, AP projection after left common carotid artery injection, demonstrates similar changes (middle cerebral artery occlusion with moyamoya collateral formation, asterisk) with pial collaterals from the posterior artery branches to the middle cerebral artery territory (black arrow).

Figure 1. Development of moyamoya collaterals in a North American adult

Legend: A 45 year old female patient initially presented with ischemic stroke. Cerebral angiography demonstrated proximal stenosis of the middle cerebral arteries bilaterally (A and V). Figure 1A, AP projection after right common carotid artery injection, demonstrates severe narrowing of the proximal middle cerebral artery (black arrow). Figure 1B, AP projection after left common carotid artery injection, shows similar changes of the distal internal and proximal middle and anterior cerebral arteries (black arrow). She was treated with steroids for presumed vasculitis. On repeat angiography approximately 6 months later, the stenotic lesions markedly progressed with the interval development of moyamoya collaterals (C and D). Figure 1C, AP projection after right common carotid artery injection, shows occlusion of the distal internal carotid artery (asterisk) with moyamoya collaterals (black arrow). Figure 1D, AP projection after left common carotid artery injection, demonstrates similar changes (middle cerebral artery occlusion with moyamoya collateral formation, asterisk) with pial collaterals from the posterior artery branches to the middle cerebral artery territory (black arrow).