Occlusive radiation cerebral vasculopathy implies medical complexity: a case report

Cranial irradiation is one of the main treatment modalities for central nervous system tumors. It carries many complications, one being occlusive radiation vasculopathy of large vessels. It is an underrecognized etiology for stroke, especially in the younger population. The pathophysiological process is controversial, but there is much literature supporting the theory of its being a secondary form of moyamoya disease.

A 31-year-old Caucasian man with a history of pineal blastoma at the age of 3 years, which was treated with resection, radiotherapy, and chemotherapy, presented to our institution with right M1 stroke. Further assessment by computed tomographic perfusion study with acetazolamide demonstrated steal phenomenon of the right middle cerebral artery territory (type III response) with a small internal region of matched cerebral blood volume defect (that is, infarct core). Coincidentally, he was found to have multiple brain masses consistent with meningiomas. Occlusive radiation vasculopathy was the most likely culprit of the patient’s stroke. The patient was treated medically with “baby” acetylsalicylic acid and clopidogrel for 3 months, then continued only on baby acetylsalicylic acid.

Late-onset occlusive radiation vasculopathy is a potentially severe iatrogenic manifestation of radiotherapy that requires a high index of suspicion as an etiology of stroke in young population, especially those with coexistent meningioma that might be a strong indicator for occlusive radiation vasculopathy as the stroke culprit. We reviewed the available literature to better understand the pathogenesis, clinical presentation, and treatment options of occlusive radiation vasculopathy. Applying perfusion studies with acetazolamide measures the cerebrovascular reserve in patients with occlusive radiation vasculopathy, which could help in determining the appropriate available treatment option.