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Abstract Details

Awareness for SMART Syndrome (Stroke-Like Migraine Attacks After Radiation Therapy), a Rare and Delayed Complication of Cranial Irradiation: A Case Report with Literature Review
Headache
P11 - Poster Session 11 (8:00 AM-9:00 AM)
12-005
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SMART syndrome is a rare, delayed complication of cranial radiation therapy, often presenting years later with triad of migraines, stroke-like deficits, and seizures. The diagnosis is challenging, as symptoms may mimic tumor recurrence, radiation necrosis, or stroke. Here, we report a case that developed decades after cranial irradiation.
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69-year-old woman with history of Astrocytoma resection and radiation therapy 35 years ago presented with a fall, left-hemianopsia, and worsening of left-hemiparesis. She eventually developed focal seizure arising from the Left temporoparietal region in video-EEG and intermittent migrainous-headache with episodic worsening of hemiparesis, initial workup including MRI brain, whole spine, and CSF analysis was negative for infectious/autoimmune/paraneoplastic causes. She was empirically treated with antibiotics/antiviral and IVIG for autoimmune-encephalitis in the interval without adequate response.

A repeat brain MRI after four days of presentation, performed due to worsening seizures and new migraine, revealed FLAIR/T2 hyperintensity in the right parieto-temporo-occipital region, with cortical gyri-form enhancement and diffusion restriction in the previously irradiated area which had fully reversed on two weeks follow up imaging. Her symptoms resolved with conservative management (analgesics & levetiracetam), and she was discharged with consideration of verapamil.


Our case demonstrates a rare occurrence of SMART-syndrome initially thought to be todd’s paresis, tumor recurrence, or autoimmune encephalitis. SMART-syndrome, though rare, should be included in the differential diagnosis with history of cerebral irradiation, even decades later (range from 1 to 37 years, average 20-years post-radiation). The underlying mechanism unknown but believed to be due to radiation-induced neuronal, mitochondrial, or Blood-brain-barrier/endothelial injury causing neuronal hyper-excitability and cortical-spreading-depression manifesting as seizures, migraine, and paroxysmal focal deficits/stroke-like symptoms (aphasia, hemianopsia, hemi-sensory loss, hemiparesis, or neglect), with cortical gyri-form enhancement on MR imaging.

Awareness and early recognition of SMART-syndrome can help prevent unnecessarily extensive evaluations and invasive procedures such as brain biopsies for suspected tumor recurrence.


Authors/Disclosures
Shiv K. Jha, MBBS
PRESENTER
Dr. Jha has nothing to disclose.
Monika Pandit, MBBS Dr. Pandit has nothing to disclose.
Binod Mehta, MD Dr. Mehta has nothing to disclose.
Jeremy Wong, MD (Montefiore Medical Center) Dr. Wong has nothing to disclose.
Eric Mariuma, MD (Montefiore Medical Center) Dr. Mariuma has nothing to disclose.