Cervical spine instability is a well-known complication of mucopolysaccharidosis IVA (Morquio A disease). If left untreated it nearly always results in spinal cord compression and progressive neurological deterioration. Timely surgery can be not only life-saving but may prevent irreversible damage to the cervical cord.
MRI scan of the cervical spine can detect early cord compression, allowing surgeons to intervene early. It has become an essential part of monitoring MPS IVA.
In a recent paper, Broomfield and colleagues at the Royal Manchester Children’s Hospital have described their experience of cervical spine surgery in MPS IVA over an eighteen-year period. Their findings of course confirm previously reported experience as above. However, they also include six patients who developed clinical signs of cord compression in the absence of any MRI changes. This is an important observation and a timely message. It underlines the importance of careful clinical examination, as well as the danger of over-reliance on MRI scans.
Here is the full reference with the link to the paper (pdf kindly supplied by Dr Broomfield).
