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We compared pre\u2010transplant CSF GAG in patients who were treatment na\u00efve (n\u2009=\u200919) versus receiving standard non\u2010BBB penetrating enzyme replacement therapy (ERT, n\u2009=\u200912). In the ERT versus treatment na\u00efve groups, CSF GAG was significantly lower across all content assayed, raising questions about using CSF GAG decrements to show BBB penetration. Future studies should compare GAG reduction in standard versus novel therapies. 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