Abstract
Stroke-like migraine attacks after radiation therapy (SMART) syndrome is a rare, delayed complication of cranial radiation therapy that consists of migraine-like headaches and focal neurologic deficits such as visual loss, aphasia, hemiparesis, hemisensory loss, and unconsciousness. SMART syndrome may be mistaken for tumor recurrence, radiation necrosis, and stroke. Timely recognition of SMART syndrome prevents unnecessary brain biopsies and enables appropriate anticipatory guidance. We present a 38 year-old right handed male with new headaches, vertigo, visual symptoms, and left-sided paresthesias. Neuroimaging revealed a heterogeneously enhancing mass with invasion into the transverse sinus, diagnosed as an epithelioid hemangioendothelioma by surgical pathology. After resection, the patient underwent proton beam radiation for maximal tissue-sparing. Six months later, he developed radiation necrosis. After another year, he developed recurrent headaches with transient language difficulties and blurry vision during each headache. Neuroimaging was consistent with SMART syndrome, and the patient was started on valproate. Verapamil was added after a second attack. The patient's headaches improved, but he remains dyslexic. Subsequent imaging shows resolution of gyriform contrast enhancement and continued left temporo-occipital T2/FLAIR hyperintensity. We present a case of early SMART syndrome following proton beam radiotherapy, as well as the dual occurrence of radiation necrosis and SMART syndrome in this individual. Radiation necrosis and SMART syndrome are known complications of radiotherapy, with the latter less well-described. We discuss a possible shared pathophysiology involving endothelial cell dysfunction and impaired cerebrovascular autoregulation, and we question whether proton RT increases risk of early SMART syndrome development.