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Título

Smart Syndrome – A case report

RESUMO

Case presentation: P.S.P is a 57 years-old male, with a past history of resection of an ependymoblastoma in the thalamic region requiring adjuvant radiotherapy in 1989. In 2005, he initiated a decline in functionality manifested by cognitive complaints, loss of balance and gait disturbances, with slow and progressive worsening over time. In the past 4 years, he began to experience throbbing headaches with moderate to severe intensity and preceded in 2 to 3 days by auto-limited episodes of aphasia and gait instability. In 2021, he was admitted to our hospital presenting a migraine-like episode, accompanied by aphasia and right-sided hemiparesis, with prolonged duration. Brain MRI showed T2/FLAIR hyperintensity involving the cortex of the temporal and occipital lobes of the left hemisphere, with gyriform gadolinium enhancement, without water diffusion restriction. Videoencephalogram showed rare epileptiform discharges in the left temporal region and left-sided bursts of frontal rhythmic delta waves. Resonance angiography and blood analysis was unremarkable. The patient was diagnosed with Stroke-like migraine attacks after radiation therapy syndrome (SMART syndrome) and treatment with dexamethasone and analgesics was initiated, with complete resolution of the symptoms in 3 weeks.
Discussion: SMART syndrome is a rare delayed neurological complication of cranial radiotherapy, manifesting years after the initial treatment. The syndrome usually presents and is diagnosticted with recurrent and often reversible episodes of migraine-like attacks, seizures and/or focal neurological deficits, representing transitory dysfunction of the irradiated cortical areas, associated with reversible, transient and unilateral cortical gadolinium enhancement associated with abnormal T2/FLAIR signal changes of the affected brain region.
Image control after two months can be useful to assess the resolution of the alterations. Differential diagnosis can be mainly tissue necrosis. Treatment consists of managing symptoms with analgesics, anti-seizure medications and corticosteroids. Typically, the course of the disease is self-limiting.
Final comments: The case reported illustrates an extremely rare delayed neurological complication of cranial radiotherapy. SMART syndrome is a radiologic and clinical entity of importance, since its recognition as a self-limited disease prevents potentially iatrogenic diagnostic tests pursuing tumor recurrence and also potentially harmfull treatments.