S for Seizure? A Case of SMART syndrome
Cleo Zarina Reyes1, Erafat Rehim1, Tsneem Mbydeen1, Gary Clauser1, Steven Lewis1
1Lehigh Valley Health Network
Objective:
N/A
Background:

Stroke-like migraine attacks after radiation therapy (SMART) syndrome is a rare condition that has been reported as a delayed consequence of brain irradiation. It presents with recurrent migraines, focal neurologic deficits and seizures. The characteristic MRI findings are gyriform cortical edema and contrast enhancement in irradiated regions. Diffusion restriction is often present. The pathophysiology is not well-understood.

Design/Methods:
NA
Results:
A 46-year-old man with a history of medulloblastoma, status post resection and radiation therapy in 1997, presented with episodic visual hallucinations, confusion and headaches. The hallucinations consisted of Nintendo characters, micropsia and macropsia. Neurological examination revealed left visual field cut. MRI brain with and without contrast showed cortical and leptomeningeal enhancement in right temporo-occipital region with corresponding hyperintensity on T2/FLAIR and restricted diffusion on DWI. CSF studies were unremarkable. Continuous EEG captured two electroclinical seizures stemming from the right occipital region, that progressed to the temporal region then generalized to both occipital regions. He was started on levetiracetam but continued to have seizures. Levetiracetam was increased with the addition of valproic acid and lacosamide. Due to ongoing seizures, he received 5 days of high dose intravenous methylprednisolone. This resulted in improvement in seizure frequency. He was discharged on levetiracetam, lacosamide, and steroid taper. Seizures stopped within 2 weeks and repeat MRI brain 1 month later revealed resolution of the abnormal signals and enhancement. He remains seizure-free 4 months later on levetiracetam and lacosamide.
Conclusions:

There is no clear consensus regarding the treatment of SMART. Here, we report a patient who presented with refractory seizures and achieved full recovery with anti-seizure medications followed by pulse steroid therapy. This exhibits a possible benefit of steroid treatment for seizure control in these patients. Further studies are warranted to delineate the pathophysiology and standardize the management of SMART syndrome.

10.1212/WNL.0000000000202236