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.
Keywords:
Mucopolysaccharidosis IV; Spinal cord compression; Spinal Cord Diseases
