Uncommon and Non-atherosclerotic Vasculopathies, Venous Disease, and Spinal Vascular Disorders
Core concepts
Non-atherosclerotic vasculopathies require pattern recognition. Cervical artery dissection causes neck pain, headache, Horner syndrome, and posterior circulation ischemia; antithrombotic choice is individualized. Reversible cerebral vasoconstriction syndrome produces recurrent thunderclap headaches with segmental arterial narrowing that resolves; avoid glucocorticoids and provoking drugs. Primary angiitis of the CNS is rare and requires cautious clinicopathologic correlation because angiography is nonspecific. Moyamoya disease causes progressive distal ICA stenosis with collateral vessels; revascularization is considered in ischemic or fragile states. CADASIL causes recurrent lacunes, migraine, and cognitive decline with anterior temporal pole involvement. Sickle cell disease, Fabry disease, and fibromuscular dysplasia complete the differential. Cerebral venous sinus thrombosis presents with headache, papilledema, seizures, or focal deficits; CT venography or MR venography confirms it. Anticoagulation is standard even with hemorrhagic venous infarction. Spinal dural arteriovenous fistula causes progressive myelopathy, often with venous congestion and elevated CSF protein; urgent angiography and treatment prevent irreversible deficits. Spinal cord infarction is usually anterior and presents with abrupt pain, paraplegia, and sphincter dysfunction.
Traps
A normal initial angiogram does not exclude reversible vasoconstriction; repeat imaging may be needed. Do not treat primary angiitis without excluding mimics. Do not miss spinal dural fistula because symptoms are attributed to degenerative disease. Do not withhold anticoagulation for venous thrombosis solely because of hemorrhage.
Worked example
A young woman with recurrent thunderclap headaches, normal initial CT, and segmental narrowing on CTA: suspect reversible cerebral vasoconstriction syndrome, stop triggers, use nimodipine if appropriate, and arrange follow-up vascular imaging.