INTRODUCTION Systemic lupus erythematosus (SLE) is a chronic, relapsingremitting autoimmune disease which primarily affects the skin, joints, and kidneys but may involve any organ system, including peripheral, autonomic, or central nervous system (CNS). 1 The CNS involvement may be considered primary if directly related to SLE activity or secondary when related to treatment complications, infections, or metabolic abnormalities such as uremia. 2 The neuropsychiatric involvement in SLE (NPSLE), first mentioned by Kaposi more than 100 years ago, remains one of the main challenges facing the rheumatologist and other physicians. 2 NPSLE can precede the onset of lupus or occur at any time during its course, most frequently within the first three years. 3-6 The prevalence of CNS involvement in SLE ranges from 14% to 80%, depending on the diagnostic criteria. 7 SLE commonly involves the meninges, cranial nerves, cerebrum, spinal cord, and rarely involves the hindbrain, causing rhombencephalitis (RE), which is a syndrome of multiple causes and variable outcomes. The term "rhombencephalitis" refers to an inflammatory disease of the rhombencephalon or the hindbrain, which is composed of the pons, cerebellum, and the medulla oblongata. The term is derived from the Greek word, "rhombos" meaning a lozenge-shaped figure, plus "enkephalos", meaning the brain. 8-10 No cases of SLE and rhombencephalitis were identified in a search of the medical literature.
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