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Reversible basal ganglia lesions in neuropsychiatric lupus: a report of three pediatric cases
Author(s) -
Sato Satoshi,
Nakajima Junya,
Shimura Masaru,
Kawashima Hisashi,
Yoshio Taku,
Hara Yuko
Publication year - 2014
Publication title -
international journal of rheumatic diseases
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.795
H-Index - 41
eISSN - 1756-185X
pISSN - 1756-1841
DOI - 10.1111/1756-185x.12235
Subject(s) - medicine , basal ganglia , fluid attenuated inversion recovery , pathology , hyperintensity , cerebrospinal fluid , autoantibody , basal (medicine) , magnetic resonance imaging , central nervous system , antibody , radiology , immunology , insulin
Aim 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 T 2‐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. Conclusion 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 .