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ABSTRACT  Objective:  Evaluate the results after decompression and stabilization of craniocervical junction in patients with mucopolysaccharidosis (MPS).  Method:  Retrospective study of 10 patients with MPS through the analysis of medical records and additional tests.  Result:  All patients with mid-term and long-term follow-up achieved consolidation of the arthrodesis and 87.5% had neurological improvement of Nurick score.  Conclusion:  Early diagnosis and intervention in cases of stenosis and/or craniocervical instability of patients with MPS provide patients a good recovery of neurological function, despite the great technical difficulty and risk of complications

RESULTS OF SURGICAL TREATMENT IN THE CRANIOCERVICAL JUNCTION IN MUCOPOLYSACCHARIDOSIS