Uncommon Presentation of an Uncommon Disease: Stroke-like Migraine Attacks after Radiation Therapy in a Patient with Sudden Onset Expressive Aphasia and Cryptogenic Fevers (P3-5.029)

Abstract

<h3>Objective:</h3> To present a case of stroke-like migraine attacks after radiation therapy (SMART) syndrome accompanied by fever. <h3>Background:</h3> SMART syndrome is an uncommon condition that presents as migraines with focal neurologic deficits in patients who have undergone brain radiation. This syndrome can present decades after completion of radiation therapy and therefore represents a unique diagnostic challenge. <h3>Design/Methods:</h3> Case report. <h3>Results:</h3> A 26-year-old man with a history of myxopapillary ependymoma status post whole brain radiation therapy 15 years prior presented with 3 days of headaches, sudden onset aphasia, and fevers. Initial exam was significant for severe expressive aphasia with only 1–2 words of speech production. Head CT was unremarkable. CT angiography demonstrated slightly decreased arborization of the distal MCA territory vessels in the left cerebral hemisphere but was otherwise unremarkable. Patient was empirically treated for CNS infection with antimicrobial therapy. CSF analysis was unremarkable, as was the remainder of his infectious workup. Long term EEG monitoring showed focal slowing in the left hemisphere but did not show any epileptiform activity. MRI was completed with arterial spin labeling (ASL) sequence, which showed increased perfusion in the left cerebral hemisphere, consistent with SMART syndrome. Patient’s exam gradually improved over the following week. He followed up in clinic 6 weeks later and his symptoms had significantly improved. He was almost back to baseline. Fevers and headaches had completed resolved. <h3>Conclusions:</h3> This case of SMART syndrome has the typical findings of sudden onset migraines and focal neurologic deficits in a patient who previously underwent brain radiation therapy. Our case was unique in that the patient had fevers of unknown origin; a phenomenon that has only been reported in one prior case. The use of MRI perfusion or ASL can aid in the diagnosis; in this case showing increased perfusion over the affected cerebral hemisphere. <b>Disclosure:</b> Dr. Gill has nothing to disclose. Dr. Gill has nothing to disclose. Dr. Avila has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Alnylam Pharmaceuticals. Dr. Avila has received personal compensation in the range of $10,000-$49,999 for serving on a Speakers Bureau for Alnylam Pharmaceuticals. Dr. Avila has received personal compensation in the range of $500-$4,999 for serving on a Speakers Bureau for Akcea Therapeutics. Gino Mongelluzzo has nothing to disclose.