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23-99.84%. This study indicates that the proposed method has high separation efficiency in analyzing saikosaponins, which provides a new way for the separation and quantitation of saikosaponins in herbal medicines.L-2-hydroxiglutaric aciduria (L2HGA) is a rare, childhood-onset, organic aciduria, with characteristic clinical (cerebellar ataxia) and neuroimaging (subcortical leukodystrophy) features. Movement disorders in this condition are usually of hyperkinetic type. Herein is reported the case of two adult siblings with recent L2HGA d