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This study is the first time report on rearrangement sequences in BCKDHA mediated by Alu element, which resulted in MSUD. Our results may also offer new insights into the formation and pathogenicity of MSUD, and may be useful to genetic counseling and genetic testing. Nicotinamide adenine dinucleotide phosphate oxidase 4 (NOX4) plays an important role in the formation of oxidative stress in brain tissues. We intended to investigate relationship between serum NOX4 concentrations and severity, delayed cerebral ischemia (DCI) plus prognosis