title: Reversible basal ganglia lesions in neuropsychiatric lupus: a report of three pediatric cases.
abstract: OBJECTIVE
  Central nervous system involvement represents a serious and common complication of systemiclupus erythematosus (SLE). We describe the characteristics of patients with neuropsychiatric (NP) SLE complicated with reversible basal ganglia lesions.
  
  
  METHODS
  We describe the cases of three NPSLE patients.
  
  
  RESULTS
  They presented with NP manifestations such as headache, cognitive dysfunction, tremors, seizures, and mood disorder. The levels of autoantibodies to NMDA (N-methyl-d-aspartate) receptor antibodies and antiribosomal-P antibodies were elevated, indicating the presence of an acute phase. Marked elevation of interleukin-6 in cerebrospinal fluid was noted when these patients showed NP symptoms. Moreover, the patients presented with high-intensity lesions in the basal ganglia on T2-weighted images, fluid-attenuated inversion recovery (FLAIR) images, diffusion-weighted images (DWI) and apparent diffusion coefficient (ADC) maps. Following immunosuppressive treatment, almost complete improvement of the lesions was noted.
  
  
  CONCLUSIONS
  The reported cases indicate that reversible vasculopathies represent vasogenic edema localized in basal ganglia lesions and that activation of the autoimmune system and inflammation could lead to NP manifestations in SLE.
authors.affiliation: Department of Pediatrics, Tokyo Medical University, Tokyo, Japan.
authors.forename: Satoshi
authors.initials: S
authors.lastname: Sato
authors.forename: Junya
authors.initials: J
authors.lastname: Nakajima
authors.forename: Masaru
authors.initials: M
authors.lastname: Shimura
authors.forename: Hisashi
authors.initials: H
authors.lastname: Kawashima
authors.forename: Taku
authors.initials: T
authors.lastname: Yoshio
authors.forename: Yuko
authors.initials: Y
authors.lastname: Hara
mesh_terms: D000293:Adolescent; D001323:Autoantibodies / Q000097:blood; D001480:Basal Ganglia Diseases / Q000175:diagnosis* / Q000188:drug therapy / Q000209:etiology*; D002648:Child; D004487:Edema / Q000175:diagnosis* / Q000188:drug therapy / Q000209:etiology*; D005260:Female; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D015850:Interleukin-6 / Q000134:cerebrospinal fluid; D020945:Lupus Vasculitis, Central Nervous System / Q000150:complications* / Q000276:immunology / Q000378:metabolism; D008279:Magnetic Resonance Imaging; D016194:Receptors, N-Methyl-D-Aspartate / Q000276:immunology; D016896:Treatment Outcome
chemical_list: D001323:Autoantibodies; D007166:Immunosuppressive Agents; D015850:Interleukin-6; D016194:Receptors, N-Methyl-D-Aspartate
keywords: apparent diffusion coefficient; diffusion-weighted images; lupus; neuropsychiatric systemic lupus erythematosus; normal range 2; posterior reversible encephalopathy syndrome