To showcase an uncommon presentation of lupus cerebritis as incomplete locked-in syndrome.

Systemic lupus erythematosus (SLE) is a multi-system autoimmune disorder that when involving the nervous system, manifests as Neuropsychiatric SLE (NPSLE). NPSLE has a multitude of  mechanisms resulting in the emergence of variable clinical presentations. We describe a peculiar case of NPSLE in a young woman who developed incomplete locked-in syndrome from a pontine lesion.

A 33-year-old female with a history of SLE presented with 2 weeks of fever, headache, and vomiting. On admission, she became obtunded and was emergently intubated. Initial lumbar puncture (LP) revealed pleocytosis (46% neutrophils, 319 corrected nucleated cells/μL), elevated protein (244 mg/dL; normal 15-40 mg/dL), normal glucose (63 mg/dL) and negative cultures. Empiric acyclovir, ampicillin, ceftriaxone, and vancomycin were initiated without any clinical improvement. Neurological examination was notable for limited ability to follow commands, vertical nystagmus, horizontal gaze palsy, diffuse hyperreflexia, and quadriparesis. EEG was consistent with diffuse encephalopathy. Brain magnetic resonance imaging (MRI) demonstrated restricted diffusion and contrast enhancement in the posterior and central pons with edema. Cerebral angiogram showed no signs of vasculitis. Treatment with intravenous (IV) methylprednisolone (MP) 1 gram and IV immunoglobulin 2 g/kg was initiated for five days. Despite these interventions, no discernible clinical improvement was observed, prompting commencement of 500 mg/m2 cyclophosphamide and daily maintenance IV MP 2 mg/kg. Repeat MRI 3-weeks later revealed a marked reduction in the size of the lesion involving the pons. The patient also improved clinically over the month with successful extubation, complete return in mental capabilities, and the ability to ambulate short distances with assistance.

Brainstem involvement in NPSLE is rare and from our review, this is the first documented presentation as a “locked-in syndrome.” The aggressive treatment employed supports the necessity for proactive management strategies to achieve favorable recovery.