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Original Investigation
October 2016

Progressive Brain Atrophy in Super-refractory Status Epilepticus

Author Affiliations
  • 1Department of Neurology, Mayo Clinic, Rochester, Minnesota
  • 2Department of Physiology and Biomedical Engineering, Mayo Clinic, Rochester, Minnesota
JAMA Neurol. 2016;73(10):1201-1207. doi:10.1001/jamaneurol.2016.1572
Key Points

Question  Does brain atrophy develop in patients with super-refractory status epilepticus (SRSE) despite seizure control with anesthetic agents?

Findings  In this medical record review of 19 patients undergoing brain magnetic resonance imaging at prespecified points, measurements of ventricular brain ratio (VBR) were taken at disease onset and follow-up, and change in VBR (ΔVBR) was calculated as a percentage of the starting measure. Median ΔVBR was 23.3%, and a significant correlation between duration of anesthetic agent use and ΔVBR was found.

Meaning  Atrophy develops during SRSE despite seizure control, and current management of SRSE may contribute.


Importance  Prolonged seizures in super-refractory status epilepticus (SRSE) have been shown to cause neuronal death and reorganization, and visual inspection in individual case studies has demonstrated progressive cortical and subcortical atrophy. At present, magnetic resonance imaging (MRI) studies that evaluate brain atrophy in SRSE are lacking.

Objectives  To document and quantify the development of atrophy over time in SRSE.

Design, Setting, and Participants  This retrospective medical record review included all patients with SRSE who were admitted to a tertiary referral campus of the Mayo Clinic Hospital with SRSE from January 1, 2001, to December 31, 2013. Patients with (1) an initial MRI scan performed within 2 weeks of SRSE onset, (2) a second MRI scan within 6 months of SRSE resolution, and (3) a minimum duration of 1 week between MRI scans were included. The ventricular brain ratio (VBR) was measured on T2-weighted fluid-attenuated inversion recovery (FLAIR) images at disease onset and during follow-up. Measurements were performed on axial FLAIR images with section thickness of less than 5 mm. The plane immediately superior to the caudate head was chosen for analysis. The hypothesis that atrophy develops during SRSE despite seizure control (electroencephalogram background suppression with anesthetic drugs) was tested. Data were analyzed from June 1 to December 31, 2015.

Main Outcomes and Measures  Change in VBR (ΔVBR) as a percentage of the starting measure.

Results  Nineteen patients met the inclusion criteria; these included 10 men (53%) and 9 women (47%) with a median age of 41 (interquartile range [IQR], 25-68) years. Anesthetic agents were required for a median of 13 (IQR, 5-37) days. Initial MRI was performed a median of 2 (IQR, 1-7.5) days from the onset of SRSE, and the second MRI was performed a median of 11 (IQR, 5-15.5) days from the resolution of SRSE, with a median of 40 (IQR, 15-65) days between MRI scans. Median ΔVBR was 23.3% (IQR, 10.5%-70.3%). A significant correlation between the duration of anesthetic agent use and ΔVBR was found (Spearman r = 0.54; P = .02).

Conclusions and Relevance  Atrophy developed in all patients with SRSE who underwent serial imaging, despite administration of agents for seizure control. The degree of atrophy appears to be related to the duration of SRSE.