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Abstract Details

Stroke-Like Migraine Attacks After Radiation Therapy (SMART) Syndrome: A Case Series
Neuro-oncology
Neuro-oncology Posters (7:00 AM-5:00 PM)
026
To improve clinical recognition, diagnosis, and management of stroke-like migraine attacks after radiation therapy (SMART) syndrome.
SMART syndrome constitutes a rare but serious clinical entity characterized by complex migraine headaches associated with stroke-like focal neurologic symptoms and/or seizures, which may occur years following cranial radiation therapy (RT). The pathophysiology of the condition remains largely unknown. Although often reversible, SMART syndrome can cause debilitating symptoms and result in significant diagnostic and management challenges.
In this case series we present unique clinical and imaging features of three patients diagnosed with SMART syndrome.*
All 3 patients (males, aged 49 – 68 years) had a history of either prior partial or whole brain RT (WBRT) for different indications, including anaplastic astrocytoma (protons; 59.4Gy), CNS lymphoma (WBRT), and small cell lung cancer (prophylactic WBRT; 36Gy). Onset of SMART syndrome post-RT was highly variable, spanning 2, 16, and 28 years, respectively. All patients presented with migraine-like headaches and/or seizures; other concurrent neurologic symptoms included aphasia (n=2), confusion (n=1), visual oscillations (n=1), and hemiparesis (n=1). In all cases, MR imaging identified unilateral cortical gyriform enhancement with associated T2/FLAIR hyperintensities primarily in parieto-occipital cortical regions. In two cases, MR perfusion studies revealed regional elevations in cerebral blood flow (n=2) and volume (n=1), suggestive of abnormal vascular reactivity. All patients eventually recovered neurologically with supportive therapy. Time to clinical and radiographic improvement ranged from 1 (n=2) to 10 months (n=1).

SMART syndrome is a rare but challenging condition that manifests several years to decades following RT. Distinction from tumor progression can be difficult. Cerebral hyperexcitability and impaired cerebral autoregulation constitute potential pathophysiological correlates, suggesting a complex mechanism.

* Portions of a case presented in this series were previously published in Olsen et al. Neurology 2016;86;787-789.

Authors/Disclosures
Sebastian Friedrich Winter (MGH Cancer Center)
PRESENTER
Mr. Winter has nothing to disclose.
Joshua P. Klein, MD, PhD, FANA, FAAN (Brigham and Women's Hospital) Dr. Klein has received personal compensation in the range of $10,000-$49,999 for serving as an Editor, Associate Editor, or Editorial Advisory Board Member for SAGE Publishers. Dr. Klein has received personal compensation in the range of $10,000-$49,999 for serving as an Editor, Associate Editor, or Editorial Advisory Board Member for McGraw-Hill. Dr. Klein has received personal compensation in the range of $5,000-$9,999 for serving as an Editor, Associate Editor, or Editorial Advisory Board Member for Oakstone Publishers. Dr. Klein has received personal compensation in the range of $5,000-$9,999 for serving as an Editor, Associate Editor, or Editorial Advisory Board Member for Wolters Kluwer. Dr. Klein has received personal compensation in the range of $100,000-$499,999 for serving as an Expert Witness for various law firms. Dr. Klein has received publishing royalties from a publication relating to health care.
No disclosure on file
No disclosure on file
Jorg Dietrich, MD, PhD, FAAN Dr. Dietrich has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Amgen. Dr. Dietrich has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Novartis. Dr. Dietrich has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Ono Therapeutics. Dr. Dietrich has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Johnson & Johnson. Dr. Dietrich has received publishing royalties from a publication relating to health care.