Case Diagnosis: SMART (Stroke-like Migraine After Radiation) Syndrome
Case Description: 40 year old with history of childhood medulloblastoma (1983) with resection, whole brain radiation and chemotherapy. He developed a headache with emesis and was taken to the hospital after being confused and answering questions inappropriately. He was aphasic with left sided weakness and a left field cut. Stroke alert was called, but imaging did not show evidence of CVA. It did reveal asymmetric right convexity in the right cerebral hemisphere with dural thickening and leptomeningeal enhancement with small areas of restricted diffusion in several right frontal and occipital sulci. LP showed WBC 31. He developed fevers and was started on antibiotics and acyclovir for presumed meningoencephalitis, however infectious workup was negative. Long term monitoring showed multifocal slowing but no seizures. Based on his remote history of radiation and negative infectious workup he was eventually diagnosed with SMART Syndrome.
Discussions: Patient had a rare complication from radiation therapy years prior. As treatments for brain tumors have improved leading to greater life expectancy, the odds of developing late complications increase. Initially the patient was extremely weak and aphasic. Fortunately this condition usually spontaneously resolves and he made good progress after being transferred to inpatient rehabilitation going from min-mod assist for most ADLs and transfers to overall supervision level.
Conclusions: Given the length of time between his radiation treatments (1983) and the development of SMART Syndrome (2019), the diagnosis could have easily been overlooked. This case highlights the importance of taking a good history as SMART Syndrome may not have been considered if his remote radiation exposure was unknown. Although uncommon, for patients with a known radiation exposure history who develop new neurologic deficits, SMART Syndrome should be considered after ruling out more serious pathology.