2023 American Academy of Neurology Abstract Website

Objective:

To present a case of systemic lupus erythematosus (SLE) that manifested as refractory seizures with a positive response to immunosuppressive therapy.

Background:

SLE is a chronic autoimmune disease characterized by the production of autoantibodies resulting in tissue injury across multiple organs; up to 50% of patients develop neurologic involvement, collectively referred to as neuropsychiatric systemic lupus erythematosus. Autoimmune encephalitis remains a rare presentation of SLE.

Design/Methods:

N/A

Results:

A previously healthy 22-year-old-female presented with fatigue, worsening cognitive impairment, multiple pre-syncopal episodes that began a few months ago. There was concern for seizures; video-EEG monitoring captured electrographic seizures originating in the right temporal region. Levetiracetam was initiated but focal epileptic seizures in right temporal, frontal regions with secondary generalization continued to occur. Lacosamide and valproic acid were added with some improvement. MRI brain was unremarkable.

Given the milieu of the patient's symptoms, autoimmune encephalitis was suspected. CSF studies showed an elevated opening pressure (30mL), but were negative for Mayo clinic autoimmune encephalitis panel. The patient was started on plasmapheresis concurrently with a 5-day course of IV methylprednisolone with steroid taper. The patient achieved seizure freedom on day 2 of plasmapheresis and day 4 of steroids. A pan-CT scan was essentially unremarkable. Rheumatologic workup revealed low C3, C4, complement levels, positive ANA, and positive double-stranded DNA antibodies, confirming systemic lupus erythematosus. The patient was transitioned to mycophenolic acid. Follow up 8 weeks later showed increased C3, C4 levels while patient was maintained on immunosuppressants and she continued to be seizure free.

Conclusions:

Antibody-mediated diseases can occur anywhere in the body where the immune system is able to gain access to a target antigen. We believe that CNS involvement of lupus may represent an under-recognized entity in the general category of NPSLE with features of autoimmune encephalitis, which include a similar therapeutic response to early plasmapheresis.