Overwhelming leukoencephalopathy as the only sign of neuropsychiatric lupus.
We describe a patient with diffuse leukoencephalopathy, a rare central nervous system complication of systemic lupus erythematosus, who died of brain herniation despite aggressive management. Brain magnetic resonance imaging revealed diffuse white matter hyperintensities consistent with vasogenic edema. Autopsy revealed only widespread cerebral edema. Early recognition and persistent, aggressive treatment will be required to avoid this fatal and rare manifestation of neuropsychiatric lupus.
Prabhakaran
Shyam
S
Department of Neurology, Columbia University Neurologic Institute, New York, NY, USA.
Bramlage
Matthew
M
Edgar
Mark A
MA
Diamond
Betty
B
Hardin
John A
JA
Volpe
Bruce T
BT
D000328:Adult; D001707:Biopsy, Needle; D003131:Combined Modality Therapy; D018450:Disease Progression; D017809:Fatal Outcome; D005260:Female; D006801:Humans; D007150:Immunohistochemistry; D007968:Leukoencephalopathy, Progressive Multifocal / Q000175:diagnosis* / Q000209:etiology / Q000628:therapy; D008180:Lupus Erythematosus, Systemic / Q000150:complications* / Q000175:diagnosis; D020945:Lupus Vasculitis, Central Nervous System / Q000175:diagnosis* / Q000209:etiology / Q000628:therapy; D008279:Magnetic Resonance Imaging / Q000379:methods; D018570:Risk Assessment; D012720:Severity of Illness Index; D013129:Spinal Puncture