Abstracts

Rationale: We present a case of emergent epilepsy surgery in a patient with Parry-Romberg syndrome and a greater than 4 week history of drug-resistant status epilepticus.Methods: The patient is a 38 year old female with a history of Parry-Romberg syndrome first presenting around age 9 who has had multiple facial reconstructions on the left in addition to a well established history of epilepsy. The patient was transferred from an outside hospital after progressively worsening seizures resulted in drug-resistant status-epilepticus for over 4 weeks despite 7 conventional AEDs and a Versed drip. Given the emergent situation, an expedited presurgical workup ensued including an MRI and scalp EEG. The scalp EEG revealed diffuse epileptiform abnormalities over the left hemisphere (ipsilateral to her disease) but with apparent seizure onset in the left frontotemporal region. MRI showed several areas of increased T2 signal seen only on FLAIR in the left gyrus rectus, left frontal pole, left middle frontal gyrus, and left parietal-occipital region. Intracranial monitoring was pursued with a left frontotemporal grid and strips to cover areas of interest. Again, diffuse epileptiform abnormalities were seen throughout all areas of the hemisphere. However, seizure onset was located in the area of the grid corresponding to the T2 abnormality over the middle frontal gyrus. Motor mapping was performed. Wada and speech mapping were not able to be performed given the patient's condition. A focal left middle frontal gyrectomy was performed corresponding both to MRI and invasive EEG findings. Additionally, multiple subpial transections (MST) adjacent to the gyrectomy were performed to help reduce seizure spread.Results: Following the middle frontal gyrectomy and adjacent MST, the patient was able to be successfully weaned off her Versed drip, take only po meds, and be discharged home.Conclusions: Parry-Romberg Syndrome is a disease of unknown etiology occasionally associated with partial onset epilepsy. In some cases it has a Rasmussen's encephalitis-like picture (Carreno, et al. Neurology 2007). However, the typical treatment for this entity, hemispherectomy, was not possible in the dominant hemisphere of this adult. However, despite the diffuse abnormalities seen by both MRI and EEG, localization and focal cortical resection of the most active gyrus in seizure generation provides an alternative treatment modality in these emergent situations.