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lated with disease activity, and could, therefore, be a potential biomarker of NPSLE for use in future clinical practice. 2020 Annals of Translational Medicine. All rights reserved.Background Autosomal recessive spastic ataxia of Charlevoix-Saguenay (ARSACS) is caused by pathogenic variants in the SACS gene and is characterized by ataxia, peripheral neuropathy, pyramidal impairment and episodic conditions such as epilepsy. Paroxysmal kinesigenic dyskinesia (PKD) had not been previously described in ARSACS. Methods We analyzed clinical ma