Abstract Details

Title S for Seizure? A Case of SMART syndrome

Topic Epilepsy/Clinical Neurophysiology (EEG)

Presentation(s) P3 - Poster Session 3 (5:30 PM-6:30 PM)

Poster/Presentation
Number 9-005

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.

Objective N/A

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.

Authors/Disclosures
Cleo Zarina A. Reyes, MD PRESENTER	Dr. Reyes has nothing to disclose.
Erafat Rehim, MD (LVHN)	Dr. Rehim has nothing to disclose.
Tsneem Mbydeen, MBBS (LVHN)	Dr. Mbydeen has nothing to disclose.
Gary W. Clauser, MD (Lehigh Neurology)	Dr. Clauser has received personal compensation in the range of $5,000-$9,999 for serving as a Consultant for Bigoen . The institution of Dr. Clauser has received personal compensation in the range of $10,000-$49,999 for serving as a Consultant for Genzyme. Dr. Clauser has received personal compensation in the range of $10,000-$49,999 for serving on a Speakers Bureau for Genentech . Dr. Clauser has received personal compensation in the range of $50,000-$99,999 for serving on a Speakers Bureau for Genzyme. Dr. Clauser has received personal compensation in the range of $50,000-$99,999 for serving on a Speakers Bureau for Biogen. Dr. Clauser has received personal compensation in the range of $50,000-$99,999 for serving on a Speakers Bureau for Novartid.
Steven L. Lewis, MD, FAAN (Lehigh Valley Health Network)	Dr. Lewis has received personal compensation in the range of $10,000-$49,999 for serving as an officer or member of the Board of Directors for American Board of Psychiatry and Neurology. Dr. Lewis has received personal compensation in the range of $50,000-$99,999 for serving as an Editor, Associate Editor, or Editorial Advisory Board Member for AAN. Dr. Lewis has received publishing royalties from a publication relating to health care. Dr. Lewis has received publishing royalties from a publication relating to health care. Dr. Lewis has a non-compensated relationship as a Secretary General with World Federation of Neurology that is relevant to AAN interests or activities.