Fellows’ Journal Club
December 2013
(1 of 3)
Clinical and imaging findings in 11 patients with SMART syndrome were reviewed. All patients became symptomatic on average 20 years postirradiation and all showed unilateral gyriform cerebral enhancement that resolved spontaneously in 2–5 weeks though 45% had residual neurologic deficits. Twenty-seven percent of patients developed laminar necrosis and brain biopsies of 4 patients showed nonspecific findings.
Summary
We retrospectively reviewed clinical and imaging findings in 11 patients with stroke-like migraine attacks after radiation therapy (SMART) syndrome to better understand this disorder previously thought to be reversible. Six men and 5 women had complex bouts of neurologic impairment beginning, on average, 20 years after cerebral irradiation. All had characteristic, unilateral gyriform enhancement on MR imaging that developed within 2–7 days and typically resolved in 2–5 weeks. Unlike prior reports, 45% had incomplete neurologic recovery manifesting as dysphasia, cognitive impairment, or hemiparesis. The remaining 55% recovered completely over an average of 2 months. Three of 11 patients developed cortical laminar necrosis. Brain biopsies in 4 of 11 did not demonstrate a specific pathologic substrate. These additional 11 patients contribute to the understanding of variability in stroke-like migraine attacks after radiation therapy syndrome, which often but not uniformly manifests with headaches and seizures, demonstrates a typical evolution of imaging findings, and may result in permanent neurologic and imaging sequelae.
