Jaw Dystonia and Reversible Basal Ganglia Changes as an Initial Presentation of Systemic Lupus Erythematosus. (January 2018)

• Record Type: Journal Article
• Authors: Romba, Meghan; Wang, Yujie; Hu, Shu-Ching; Khot, Sandeep
• Is Part Of: Neurohospitalist. Volume 8:Number 1(2018)
• Page Start: 31
• Page End: 34
• Publication Date: 2018-01
• Subjects: movement disorders -- clinical specialty -- neuroimmunology -- dystonic disorders -- parkinsonian disorders -- imaging techniques; Nervous system -- Diseases -- Periodicals; Neurology -- Periodicals; 616.8
• DOI: 10.1177/1941874417698323
• Languages: English
• ISSNs: 1941-8744

Abstract

Dystonia as a manifestation of neuropsychiatric lupus erythematosus (NPSLE) is uncommon. We report a 25-year-old woman who experienced progressive confusion, reduced speech, and difficulty opening her mouth approximately 2 weeks after development of a facial rash. Brain imaging showed bilateral, symmetric signal abnormalities within the basal ganglia and subcortical white matter. Despite treatment with high-dose steroids, she continued to have difficulty speaking with evidence of jaw dystonia on examination. Jaw dystonia rapidly improved with the initiation of levodopa. Repeat evaluation 3 months later exhibited the absence of jaw dystonia and near resolution of the imaging abnormalities. Our patient demonstrated a unique presentation with jaw dystonia refractory to traditional treatment for NPSLE. Such a presentation likely represents a severe variant of NPSLE requiring both immunosuppressive and symptomatic therapies.