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SMART syndrome: a case report

RESUMO

CASE PRESENTATION
A 34-year-old man had a progressively developed headache and focal seizures. CT brain scan disclosed a left frontal mass, with gadolinium enhancement. The patient underwent a successful subtotal lesion resection, followed by whole-brain radiotherapy for 3 months (60Gy). Pathological analysis revealed an anaplastic oligoastrocytoma, according to the 3rd WHO classification of central nervous system tumors. Due to great post-radiotherapy brain MRI status, the patient was put under conservative treatment, with control images every six months. The four following brain MRIs revealed important gadolinium enhancement in addition to cortical gyriform enhancement, although the patient remained asymptomatic.
Two years after the initial diagnosis, the patient presented sudden transient right-sided weakness. A new brain MRI showed diffuse gadolinium enhancement. The patient underwent a second resection and tissue histopathology revealed radiation necrosis, with no signs of neoplastic disease. He also started a daily headache of pulsating quality, in association with nausea. Migraine prophylaxis was started, along with steroids therapy, with great symptom control.
Steroid therapy was necessary for the two following years, and the patient presented new clinical recurrences and worsening brain image in every withdrawal attempt. According to Black's criteria, the patient was diagnosed with stroke-like migraine attacks after radiation therapy (SMART) syndrome.
DISCUSSION
SMART syndrome is a rare late complication of radiotherapy and usually presents 12 years after the treatment. Classic symptoms are migraine attacks and spell focal deficits, such as aphasia and hemiplegia, although some patients may experience seizures. Some patients also may recur from clinical deficits.
Risk factors are not clearly defined but higher radiation doses may increase the chances of developing SMART syndrome. Pathophysiology is still poorly understood, but some authors believe that radiation leads to vascular damage, which causes endothelial dysfunction. Brain MRI is not always specific, and distinction from tumor progression may be difficult. No treatment has been established, but some cases report the use of steroids and antiplatelet agents.
CONCLUSION
Our case reports an early development of SMART syndrome. It is a rare complication of growing importance, especially because cancer patients are surviving much longer, and the symptoms can impact on quality of life.

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