Status epilepticus and histopathological findings in a case of stroke-like migraine attacks after radiation therapy (SMART) syndrome
Abstract number :
2.072
Submission category :
4. Clinical Epilepsy / 4A. Classification and Syndromes
Year :
2016
Submission ID :
199439
Source :
www.aesnet.org
Presentation date :
12/4/2016 12:00:00 AM
Published date :
Nov 21, 2016, 18:00 PM
Authors :
P. Emanuela Voinescu, Brigham and Women's Hospital; Jeffrey Helgager, Brigham and Women's Hospital; Christopher Parres, Brigham and Women's Hospital; Maya Graham, Brigham and Women's Hospital; Henrikas Vaitkevicius, Brigham and Women's Hospital; Umberto D
Rationale: Stroke-like migraine attacks after radiation therapy (SMART) is a syndrome manifested by transient neurological symptoms , usually including seizures, thought to be the expression of a focal neuronal dysfunction in an area exposed to radiation several years prior. MRI typically shows cortical thickening and gadolinium enhancement, and perfusion studies have suggested impaired cerebrovascular autoregulation as a causative factor, though the pathophysiologic underpinnings of this syndrome are still poorly understood. Methods: Here we report the case of a 72 year-old man with a history of right frontal atypical meningioma status post resection and XRT, presenting 14 years later with recurrent seizures which, in the setting of gastroenteritis, led to refractory focal status epilepticus, ultimately cured with right frontal lobectomy. Results: Even prior to the episode of focal status epilepticus, his MRI brain with contrast revealed increasing gyriform enhancement and cortical thickening within the right frontal lobe, raising suspicion for SMART syndrome. Long-term video EEG monitoring revealed medically refractory electroclinical focal status epilepticus arising from the right fronto-temporal region; intraoperative electrocorticography captured electrographic seizures arising from the right inferior/middle frontal gyrus. Pathology revealed reactive changes in white and gray matter which were likely the sequelae of previous radiation treatment, seizures, and surgery. Conclusions: This is the first reported case of SMART manifesting as super refractory focal status epilepticus requiring surgical treatment, and one which will provide valuable neuropathological information regarding this still mysterious syndrome. Funding: None
Clinical Epilepsy