Shuai Chen1, Xin-Liang Liang1, Shuang He1, Jie-Wen Zhang2, Shu-Jian Li3. 1. Department of Neurology, Zhengzhou University People's Hospital (Henan Provincial People's Hospital), Zhengzhou, 450003, Henan, China. 2. Department of Neurology, Zhengzhou University People's Hospital (Henan Provincial People's Hospital), Zhengzhou, 450003, Henan, China. zhangjw9900@126.com. 3. Department of Neurology, Zhengzhou University People's Hospital (Henan Provincial People's Hospital), Zhengzhou, 450003, Henan, China. lsjdoctor@126.com.
Abstract
INTRODUCTION: Histiocytic necrotizing lymphadenitis, also known as Kikuchi-Fujimoto disease, is a rare benign self-limiting inflammatory disease often seen in young adults. The main clinical features are fever with cervical lymphadenopathy. Neurological complications of Kikuchi-Fujimoto disease were occasionally reported although the specific pathogenesis was not clear. The condition could be severe when encephalitis coexists. METHODS: Here we reported a young case of Kikuchi-Fujimoto disease with subsequent severe autoimmune encephalitis. RESULTS: The symmetric striatal and limbic MRI lesions combined with psycho-cognitive, epileptic symptoms supported encephalitis. Tissue-based immunofluorescence revealed widely cytoplasmic fluorescence in rat cerebellar and hippocampal neurons, which provide evidence for immune-mediated encephalitis. The clinical outcome was satisfactory after immunosuppressive therapy with MRI lesions largely disappeared. CONCLUSION: The encephalitis complication of Kikuchi disease may be autoimmune and mediated by cytotoxic T cells.
INTRODUCTION: Histiocytic necrotizing lymphadenitis, also known as Kikuchi-Fujimoto disease, is a rare benign self-limiting inflammatory disease often seen in young adults. The main clinical features are fever with cervical lymphadenopathy. Neurological complications of Kikuchi-Fujimoto disease were occasionally reported although the specific pathogenesis was not clear. The condition could be severe when encephalitis coexists. METHODS: Here we reported a young case of Kikuchi-Fujimoto disease with subsequent severe autoimmune encephalitis. RESULTS: The symmetric striatal and limbic MRI lesions combined with psycho-cognitive, epileptic symptoms supported encephalitis. Tissue-based immunofluorescence revealed widely cytoplasmic fluorescence in rat cerebellar and hippocampal neurons, which provide evidence for immune-mediated encephalitis. The clinical outcome was satisfactory after immunosuppressive therapy with MRI lesions largely disappeared. CONCLUSION: The encephalitis complication of Kikuchi disease may be autoimmune and mediated by cytotoxic T cells.
Authors: Joung-Hee Byun; Su Eun Park; Sang Ook Nam; Young A Kim; Young Mi Kim; Gyu Min Yeon; Yun-Jin Lee Journal: Brain Dev Date: 2017-10-16 Impact factor: 1.961