What is the diagnosis if all three cerebral artery territories (Anterior Cerebral Artery (ACA), Middle Cerebral Artery (MCA), Posterior Cerebral Artery (PCA)) are involved in a stroke?

Involvement of All Three Cerebral Artery Territories (ACA, MCA, PCA) in Stroke

When all three major cerebral artery territories (ACA, MCA, and PCA) are involved in a stroke, the most likely diagnosis is internal carotid artery (ICA) occlusion with poor collateral circulation, or moyamoya disease/syndrome with progressive bilateral stenosis. 1

Primary Diagnostic Considerations

Internal Carotid Artery Occlusion

• ICA occlusion is the leading cause of large territorial infarctions involving multiple vascular distributions, accounting for 41% of large MCA territory infarcts and frequently extending to involve ACA and PCA territories when collateral flow is inadequate 2
• The combination of ACA and MCA territory involvement strongly suggests proximal ICA disease, as the ICA gives rise to both vessels 1
• PCA involvement can occur through compromised posterior communicating artery collaterals or when the PCA has a fetal origin from the ICA (present in approximately 20-30% of individuals) 3

Moyamoya Disease/Syndrome

• Moyamoya is characterized by chronic progressive stenosis of the distal intracranial ICA and proximal ACA and MCA, with less frequent involvement of the basilar artery and posterior cerebral arteries 1
• The Japanese diagnostic criteria require: (1) stenosis at the distal ICA bifurcation (C1) and proximal ACA (A1) and MCA (M1), (2) dilated basal collateral arteries, and (3) bilateral abnormalities 1
• Moyamoya accounts for 6% of childhood strokes and presents with multiple, recurrent ischemic strokes involving predominantly the carotid circulation, often in watershed territories 1, 4
• Infarctions may be superficial or deep and frequently involve multiple vascular territories simultaneously 1

Clinical Presentation Patterns

Severe Neurological Deficits

• Involvement of all three territories produces devastating neurological deficits including hemiplegia affecting face, arm, and leg; hemisensory loss; hemianopia; global aphasia (if dominant hemisphere); and reduced consciousness 2
• The combination of these symptoms has a positive predictive value of 0.73 for large territorial infarction 2
• Reduced consciousness, hemianopia, and complete territorial infarction are independent predictors of death or severe disability 2

Age-Related Patterns

• In children and young adults, consider moyamoya disease, sickle cell disease with moyamoya syndrome, or ICA dissection (12% of large MCA infarcts) 1, 2
• In older adults, consider cardiogenic embolism (54% of large territorial infarcts), ICA atherosclerotic occlusion, or tandem lesions 2, 1

Diagnostic Workup Algorithm

Immediate Imaging (Within 25 Minutes)

• Non-contrast CT to exclude hemorrhage and assess early infarct signs across all three territories 1
• Look for hyperdense MCA sign, loss of gray-white differentiation, and sulcal effacement involving >1/3 of MCA territory plus ACA and PCA regions 1
• Early infarct signs involving multiple territories indicate massive stroke with high risk of hemorrhagic transformation and malignant edema 1

Vascular Imaging (Urgent)

• CT angiography or MR angiography to identify ICA occlusion, moyamoya pattern, or basilar artery involvement 1
• MRA demonstrates absence of flow voids in ICA, MCA, and ACA with abnormally prominent basal ganglia and thalamic collateral vessels in moyamoya 1
• CTA reveals the extent of proximal occlusion and collateral circulation status 1

Advanced Imaging

• Diffusion-weighted MRI confirms acute infarction across all three territories with high sensitivity (88-100%) and specificity (95-100%) 1
• Perfusion imaging (CT or MRI) demonstrates the extent of hypoperfusion and potential for salvageable tissue 1

Etiology-Specific Considerations

Cardiogenic Embolism

• Atrial fibrillation and cardiogenic embolism account for 33-54% of large territorial infarcts 2
• Evaluate with ECG, prolonged cardiac monitoring, and echocardiography 5
• Large emboli can lodge at the ICA terminus (T-occlusion) affecting all downstream territories 1

Sickle Cell Disease

• In patients with sickle cell disease, large infarctions within ACA or PCA territories occur less often than MCA, but progressive vasculopathy can involve all territories 1
• Some individuals develop progressive ICA vasculopathy with distal collaterals (moyamoya syndrome) 1
• TCD showing elevated velocities (≥200 cm/s) predicts 10% annual stroke risk 1

Tandem Lesions

• Tandem strokes (extracranial ICA stenosis/occlusion with ipsilateral large vessel occlusion) account for 20-30% of all ischemic strokes 1
• These present with large clot burden, poor response to thrombolysis (recanalization <10%), and high disability rates (40-69%) 1

Prognostic Implications

Mortality and Morbidity

• Mortality is 17% and severe disability is 50% with large multi-territorial infarctions 2
• Brain edema causes 16 of 35 deaths, occurring earlier (mean 5 days) in younger patients (mean age 57 years) 2
• Patients developing coma on admission day are more likely to die from malignant brain edema 2

Predictors of Poor Outcome

• Coma is an independent predictor of death 2
• Reduced consciousness, hemianopia, and complete territorial infarction independently predict death or severe disability 2
• Peak swelling occurs several days after onset, requiring close monitoring for herniation 1

Critical Management Pitfalls

• Do not delay imaging to obtain extensive cardiac workup—brain imaging must be completed within 25 minutes of arrival 1
• Recognize that involvement of all three territories indicates either proximal ICA occlusion or moyamoya, not isolated branch occlusions 1, 2
• In moyamoya patients, avoid hyperventilation, crying, coughing, or straining as these precipitate ischemic symptoms 1
• Consider decompressive craniectomy early (within 48 hours) for malignant edema in patients <60 years with complete hemispheric infarction 1