Two Cases of Malignant Cerebral Edema Related to Systemic Lupus Erythematous (P6.252)

OBJECTIVE: To describe a syndrome of diffuse white matter involvement and malignant cerebral edema in patients with angiography negative systemic lupus erythematous (SLE). BACKGROUND: The neurological manifestations of SLE are incompletely described. Most instances involve the development of a cerebral vasculitis leading to multiple cerebral infarcts. A few case series report a benign idiopathic intracranial hypertension related to SLE responsive to immunotherapy. However there are no reports of patients with malignant cerebral edema, and diffuse white matter changes in the absence of CNS vasculitis. DESIGN/METHODS: Retrospective chart review of two patients admitted to our neurologic intensive care unit. RESULTS: Case one was a 32 year-old woman admitted with nausea and vomiting and cranial nerve findings. An MRI showed bilateral symmetric diffuse T2/FLAIR hyperintensities throughout the white matter. Serology was positive for SLE (ANA, RNP, dsANA, Smith) however cerebral angiogram was negative for vasculitis. The patient developed recalcitrant cerebral edema with intracranial hypertension despite immunospressive therapies. A Licox and ICP monitor was placed to optimize cerebral oxygen delivery and treat intracranial hypertension. Despite maximal aggressive medical management patient neurologically deteriorated and eventually died from sepsis. Case two was a 29 year-old woman with known SLE that presented with loss of consciousness. The CT showed diffuse cerebral edema and white matter involvement. Cerebral angiography was normal. Maximal Immunosuppresive therapy was initiated. A Licox monitor was placed and ICP management included mannitol, hypertonic saline and hypothermia. Again despite maximal medical management the patient subsequently herniated resulting in death by neurological criteria. CONCLUSIONS: Large vessel CNS vasculitis is a known complication of SLE, but these two cases represent a syndrome of white matter changes and diffuse cerebral edema that have yet to be reported in the literature. Further research is needed to better understand the underlying pathology and identify potential treatment options. Disclosure: Dr. Koffman has nothing to disclose. Dr. Asi has nothing to disclose. Dr. Russo has nothing to disclose. Dr. Dani has received personal compensation for activities with General Electric. Dr. Dani holds stock and/or stock options in General Electric, which sponsored research in which Dr. Dani was involved as an investigator. Dr. Manno has nothing to disclose.