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Irifaze ICV (idursulfase beta) is a recombinant human iduronate-2-sulfatase (IDS) enzyme replacement therapy developed by GC Biopharma. It is specifically formulated for intracerebroventricular (ICV) administration to treat the severe form of Hunter syndrome (Mucopolysaccharidosis type II, MPS II) characterized by central nervous system (CNS) involvement. By delivering the enzyme directly into the cerebral ventricles via an implanted reservoir, the drug bypasses the blood-brain barrier, which typically limits the efficacy of intravenous enzyme replacement therapies in treating cognitive decline. It was first approved in Japan (as Hunterase ICV) and subsequently in Taiwan (as Irifaze ICV), making it a pioneering treatment for the neurodegenerative aspects of MPS II.
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