SMART (stroke-like migraine attacks after radiation therapy) syndrome responded to steroid pulse therapy: Report of a case and review of the literature

Abstract

This report presents a case of stroke-like migraine attacks after radiation therapy (SMART) syndrome in a 31-year-old man in whom symptoms and radiological findings resolved with steroid pulsed therapy and reviews the literatures with special emphasis on the use of steroids against SMART syndrome. The patient had a past history of left temporal anaplastic astrocytoma and was treated with surgery followed by local 72 Gy radiation therapy and chemotherapy using Nimustine Hydrochloride. Four years after the surgery, he was suffering from subacute progressing symptoms of headache, right hemianopia, right hemiparesis and aphasia from 2 to 4 days before admission to our hospital. At first he was diagnosed as symptomatic epilepsy but after extensive examination, the final diagnosis was SMART syndrome. His symptoms soon improved with steroid pulse therapy. In the literature, steroid pulse therapy is not necessarily a standard of care for SMART syndrome, but it seemed to decrease the need of biopsy. As the lesions of SMART syndrome require differential diagnosis from recurrences, biopsy was performed in some cases. However, lack of benefit and possible detriment is reported with biopsy of SMART lesions. Through this experience we suggest that steroid pulse therapy may provide speedy recovery from symptoms, and it should be considered before other invasive investigations or treatments.

SMART Syndrome Identification and Successful Treatment

“…Besides carbamazepine and corticosteroids, we administered the calcium channel blocker verapamil. Even though this drug was used previously only in a few case reports [ 9 ], it can have a beneficial effect considering the dysfunction in vasoreactivity involved in SMART pathophysiology [ 5 ]. Posterior reversible encephalopathy syndrome (PRES) is a more frequent entity that shares a similar clinical presentation with SMART syndrome.…”

“…We report the case of a patient with SMART syndrome with typical clinical and imaging features who was treated with corticosteroids and verapamil, showing fast resolution of symptoms. There is no clear consensus regarding effective treatment approaches to SMART; however, there are reports of patients with partial or complete recovery of symptoms after pulse therapy with corticosteroids [ 9 ]. There may also be a role for calcium channel blockers as an adjuvant drug [ 9 ].…”

Stroke-Like Migraine Attacks after Radiation Therapy Syndrome: Clinical and Imaging Characteristics

“…SMART syndrome is a rare neurologic complication that appears months to years after radiotherapy of intracranial neoplasms with unilateral headache and possibly along with other symptoms such as nausea, vomiting, hemiparesis, and seizures. [6] Although the pathophysiology of SMART syndrome is not clearly understood, radiation-induced damage of endothelial cells is proposed to be the initial insult,[7] with some considering the pathogenesis similar to posterior reversible encephalopathy syndrome (PRES) due to the insult leading to blood–brain barrier malfunction seen with some vasculopathies. [8] SMART syndrome is usually self-limited; therefore, invasive testing such as angiography or biopsy is not recommended and may even be harmful.…”

“…[8] SMART syndrome is usually self-limited; therefore, invasive testing such as angiography or biopsy is not recommended and may even be harmful. [6]…”

Incremental Utility of Tc-99m Glucohepatonate Single-Photon Emission Computed Tomography over 18F-Flourodeoxyglucose Positron Emission Tomography in Diagnosis of Brain Tumor Recurrence – Old is Gold

“…[ 3 ] The patients with SMART syndrome present with features of cortical dysfunction with clinical symptoms manifesting as seizures, migraine-like headache, hemiparesis, confusion, visuospatial defects, etc. [ 3 4 ] Among the various pathophysiological mechanisms, the role of radiation-induced endothelial dysfunction, direct injury to the neurons, and other inflammatory, metabolic, and genetic factors has been suggested. [ 5 ] On magnetic resonance imaging (MRI), unilateral temporoparietal cortical involvement with postcontrast enhancement is described in the literature.…”