(853) Being SMART: Recognizing Etiologies of Stroke-Like Symptoms in Pediatric Patients

Introduction: Strokes in pediatric patients are rare events and very often are associated with congenital vascular abnormalities. Urgent radiological investigation and prompt treatment have significant prognostic implications. However, a patient may occasionally present with definitive stroke-like symptoms, but no radiological abnormality can be detected. Here we present a case of a pediatric patient requiring repeat brain radiation therapy presenting with stroke-like symptoms, which can be attributed to SMART (Stroke-Like Migraine Attacks After Radiation Therapy) Syndrome.

Description: A 14-year-old female had a history of left frontal anaplastic ependymoma requiring surgical resection and radiation with recurrence at 3 years requiring reirradiation resulting in persistent, baseline right-sided weakness. She presented with sudden onset worsened right-sided weakness, dysarthria, right facial droop, and headache. Family had noted a similar episode 3 days prior with spontaneous partial resolution initially attributed to dehydration. CT ruled out acute intracranial hemorrhage, showing post-radiation calcification and gliosis, with a recommendation for MRI to rule out recurrence of the primary neoplasm. MRI was without apparent recurrence, post-radiation encephalomalacia and gliosis with scattered calcifications. MRA/MRV ruled out vascular flow occlusion, stenosis, or aneurysm. She had no evidence of seizure. Despite avoiding the initiation of targeted treatment, she continued to improve. Ultimately, she was transferred for continued care per her primary oncology team before a complete return to baseline.

Discussion: This case represents an important, but rare, long-term complication of childhood CNS radiation. SMART syndrome is rare, though it may be an important emerging long-term complication in patients with a history of brain irradiation. Symptoms are typically transient and episodic and include acute onset headache, seizure, and focal neurologic deficits. New treatments have been proposed, including corticosteroids and calcium channel blockers, though there has been no consistent evidence of efficacy. SMART Syndrome is an emerging differential consideration after stroke, and tumor recurrence have been ruled out. Further recognition will enable early treatment initiation and optimization.