Central nervous system lupus presenting as rhombencephalitis responding to aggressive immunotherapy: A unique case. Issue 3 (7th May 2020)

• Record Type: Journal Article
• Authors: Kaur, Harleen; Mittal, Gaurav K.; Singhdev, Jennifer
• Is Part Of: Clinical & experimental neuroimmunology. Volume 11:Issue 3(2020)
• Page Start: 176
• Page End: 180
• Publication Date: 2020-05-07
• Subjects: aggressive immunotherapy -- antinuclear antibody -- CNS lupus -- rhombencephalitis -- SLE; 616.80479
• DOI: 10.1111/cen3.12592
• Languages: English
• ISSNs: 1759-1961

Abstract

Abstract: Background: Systemic lupus erythematosus (SLE) is the prototypic autoimmune connective tissue disorder (CTD) usually affecting young females. Central nervous system lupus is a serious complication encountered in SLE cases. Predominant involvement of rhombencephalon in patients of SLE at the first contact with health care professional has not been seen and described frequently in the literature. It still remains a diagnostic and therapeutic challenge. Case presentation: This paper describes a case of SLE in an adolescent female who was diagnosed as having rhombencephalitis, which is a rare presentation of SLE. Conclusions: Central nervous system lupus should be included in the provisional diagnosis of any young female patient presenting with complicated neurologic manifestations over the background of joint pain or fever or headache or confusion or psychosis, to avoid misdiagnosis and aid in timely institution of aggressive immunotherapy. Abstract : Rhombencephalitis is a rare presentation of SLE. CNS involvement in SLE is generally associated with a poor prognosis. By presenting our case we wish to highlight that keeping a high index of suspicion in appropriate clinical setting is of great importance.