Background/Purpose: Predictors of seizures and epilepsy in systemic lupus erythematosus (SLE) patients are well described but descriptions of status epilepticus (SE) have thus far been limited to isolated case reports. The purpose of this study is to determine the electro-clinical characteristics as potential predictors of long-term outcome of SE in SLE. 

Methods: This is an observational study of consecutive SLE patients with SE, enrolled out of the Maryland Lupus cohort that consists of 1138 SLE patients. Demographic, clinical features, laboratory data, brain magnetic resonance imaging (MRI) studies and video electroencephalography (EEG) were examined during SE event. Outcome was determined at 12 months following SE event. Seizure not related to SLE were excluded.

Results:

In this SLE population, 78 (6.9%) of all patients had seizures related to SLE, and of these 7 (9.0%) had SE.  At the SE events, patients [mean age 21.4 years, 72 % women, 72 % African American, and a mean duration of SLE of 6.5 years] presented with cutaneous vasculitis (57%), arthritis (43 %), psychosis ( 29 %), nephritis (14 %) and ischemic stroke (14 %). Convulsive SE was observed in 6 patients (3 generalized, 3 complex partial with secondary generalized) and non-convulsive SE in 1. Prolonged electrographic seizure activity (1-3 days) with continuous slowing and multifocal spikes in the bifornatal and temporal regions, associated with refractory complex partial and generalized seizures were observed in 5 patients. EEG seizures resolved abruptly in 2 patients. In addition to immunotherapy, multiple anti-epileptic drugs were needed to control SE in all patients.

All SE patients had a symptomatic structural lesion with probable etiologic significance with increased T2/FLAIR hyperintensity in the front- temporal and posterior cortex, and hypointense lesions in the regions of the amygdyla –hippocampal complex, thalamus and basal ganglia areas. Restricted weighted sequence images demonstrated restricted diffusion in the corresponding cortical regions of the frontal and temporal lobes in two patients. Two patients had normal initial brain MRI, but abnormal lesions were identified using functional FDG-PET scanning.

A favorable clinical and EEG response was recognized in all SLE patients with SE, with improvement in consciousness and survival. Recurrent seizure events were observed in 3 patients following the SE event, while recurrent SE events were observed in one patient who developed mesial temporal sclerosis overtime. Poor functional and cognitive outcome observed in 3 (43 %) patients, was associated with prolonged SE event, presence of cortical and subcortical structural lesions on brain MRI that correlated with the recorded EEG abnormality. No mortality among the SE patients was observed. 

Conclusion: Overall, SLE patients with SE seem to have a favorable clinical outcome. Patients with prolonged SE events with a greater cortical-subcortical lesion burden on brain MRI have poor physical and cognitive function on long-term outcome. Identifying the pathogenic role of specific SLE related autoantibodies/cytokines in relation to brain structural lesions of SLE patients with SE is needed.

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ACR Meeting Abstracts - https://acrabstracts.org/abstract/electroclinical-correlates-and-outcome-of-status-epilepticus-in-systemic-lupus-erythematosus/