Case Report

Overwhelming Leukoencephalopathy as the Only Sign of Neuropsychiatric Lupus

SHYAM PRABHAKARAN, MATTHEW BRAMLAGE, MARK A. EDGAR, BETTY DIAMOND, JOHN A. HARDIN, and BRUCE T. VOLPE

ABSTRACT.

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. (J Rheumatol 2005;32:1843-5)

Key Indexing Terms:

SYSTEMIC LUPUS ERYTHEMATOSUS
PATHOLOGY
AUTOANTIBODIES
IMMUNOLOGY
NERVOUS SYSTEM

---

From the Department of Neurology and Neuroscience, Weill Medical College of Cornell University, New York; Department of Pathology, New York Presbyterian Hospital Weill-Cornell Medical Center, New York; Department of Microbiology and Immunology and Medicine, Albert Einstein College of Medicine, Bronx, New York, USA.

Supported by National Institutes of Health (NIAMS/AR RO149126), the Lupus Research Institute, and the Burke Medical Research Institute.

S. Prabhakaran, MD, Department of Neurology, Columbia University Neurologic Institute, New York, NY; M. Bramlage, MD; M.A. Edgar, MD, Department of Pathology, New York Presbyterian Hospital Weill-Cornell Medical Center; B. Diamond, MD, Department of Medicine, Columbia University College of Physicians and Surgeon, New York, NY; J.A. Hardin, MD, Department of Medicine, Albert Einstein College of Medicine; B.T. Volpe, MD, Department of Neurology and Neuroscience, Weill Medical College of Cornell University.

Address reprint requests to Dr. B. Volpe, Department of Neurology and Neuroscience, Weill Medical College of Cornell University, 525 East 68th Street, New York, NY 10021. E-mail: bvolpe@burke.org

Accepted for publication May 9, 2005.

---