B48-25 Breaking the Anchor: A Case of Npsle Presenting as Refractory Status Epilepticus

Approximately 33-50% of systemic lupus erythematosus (SLE) patients have neurologic or neuropsychiatric symptomatology. While no diagnostic criteria of neuropsychiatric lupus erythematous (NPSLE) exists, patients may be diagnosed with SLE by serology and also have neuropsychiatric symptoms defined by the American College of Rheumatology. These syndromes include seizure disorder, aseptic meningitis, demyelinating syndromes, myelopathy, headache, cerebrovascular disease, movement disorders, autonomic disorders, myasthenia gravis, polyneuropathy, cranial neuropathy, Guillain Barre syndrome, mononeuropathy, plexopathy. The patient was an otherwise healthy 42-year-old male brought by ambulance after he was found on the ground and confused. He was evaluated by his primary care provider (PCP) approximately a week prior to admission for fatigue, malaise, and rash over his face and extremities. Infectious etiology was suspected, and he was started on doxycycline as an outpatient. Upon arrival at the emergency department, he began to have tonic-clonic movements which continued despite sequential administration of intravenous lorazepam. He required intubation for airway protection and was placed on a propofol infusion for status epilepticus. The patient was started on broad spectrum antibiotics and cultures were drawn, including from the cerebrospinal fluid (CSF). CSF studies were only significant for protein. Cultures remained negative. MRI brain with and without contrast were unremarkable. The patient continued to have intermittent seizures despite various antiepileptic regimens and propofol infusion, so the patient was started on midazolam infusion with improvement to his seizure activity. Autoimmune serology was pending for several days but eventually showed positive ANA and anti-double stranded DNA antibodies. Additional seropositivity included anti-smith antibodies, and anti-beta-2-glycoprotein-1 IgG. He was eventually started on hydroxychloroquine 400 mg daily and a three-day course of 1000 mg IV methylprednisolone daily. After initiation of pulse dose steroids, the patient was able to wean off midazolam infusion for status epilepticus. He was extubated and discharged with close rheumatology follow up. Upon initial encounter in the emergency department, it was reported that the patient had recently visited Cambodia thus parasitic or tropical disease was high on the differential. However, the history of presenting events was independently reviewed by the critical care team, and it was later discovered that anchoring bias had skewed the work-up. This case exemplifies the importance of maintaining broad differentials and obtaining independent history. High suspicion, early diagnosis, and subsequent treatment can result in positive outcomes for patients afflicted with neurological manifestations of SLE. This abstract is funded by: None