Abstracts

Rationale: Although seizures and epilepsy are well established by population studies as common neurological features of systemic lupus erythematosus (SLE) (Mikdashi, 2005), data on status epilepticus (SE) in SLE have thus far been limited to isolated case reports. Characterization of a comprehensive cohort of SE in SLE may help clarify the pathologic role of autoimmune mechanisms in severe and drug resistant seizures. Methods: This observational study included consecutive SLE patients with seizure disorders, enrolled from the Maryland Lupus cohort that consists of 1138 SLE patients diagnosed according to the American College of Rheumatology criteria (Mikdashi, 2005). Patients have been monitored quarterly from 1994 to 2012, over a mean follow-up period of 8 years (range, 1 to 16 years). A standardized protocol was applied at entry to all SLE patients presenting with one or more seizures and those with SE, including history and physical and neurological examination. Demographic, clinical, radiological and laboratory data were documented. Results: In this SLE population, 78 (6.9%) of all patients had one or more seizures related to their SLE, and of these 7 (9.0%) SE. Convulsive SE was observed in 6 patients and non-convulsive SE in 1. In comparing SLE seizure patients with SE (SE+) to those without SE (SE-), seizure disorders occurred earlier in the course of SE+ as compared to SE-. Also high baseline SLE disease activity (as measured by the Systemic Lupus Disease Activity Index [SLEDAI]), median younger age at SLE onset (SE+ 21.4 yrs vs. SE- 30.5 yrs), and shorter duration of SLE (SE+ 6.52 yrs vs. SE- 10.9 yrs) were more frequent among patients with SE. Cutaneous vasculitis (SE+ 57.1% vs. SE- 25.6%) and psychosis (SE= 28.5 vs. SE- 11.5%) were more frequent in patients with SE. There was a trend towards persistent elevation of autoantibodies (dsDNA [SE+ 57.1%, SE- 53.8%], cardiolopin [SE+ 28.6% vs. SE- 24.3%], in patients both with and without SE, though there was no significant statistical difference between the groups. SSA antibodies were less frequent among SE patients [SE+ 28.6% vs. SE- 46.1%). There was a greater cortical-subcortical lesion burden on brain MRI (cortical [SE+ 71%, SE- 21.3%, p value < 0.01], subcortical [SE+ 57.1% vs. SE- 35.5%]). Likewise, abnormal CSF studies (elevated protein levels and WBC) were more frequent in patients with SE (SE+ 14.3% vs. SE- 2.2%). Conclusions: The spectrum of SE in SLE includes preliminary observations of heightened serological activity, including autoantibodies, an increased lesion burden (particularly cortical) on MRI, and abnormal CSF studies in SLE patients with SE compared to those without SE. This suggests that SE patients represent an important subpopulation of SLE seizure patients for whom further study is warranted and may be model that could shed light on the pathologic role of autoimmune mechanisms in severe and drug resistant seizures and epilepsy. Mikdashi J, Krumholz A, Handwerger B. Factors at diagnosis predict subsequent occurrence of seizures in SLE. Neurology 2005;64:2102-2107.