Appendix 21 – Atlanto-axial instability
Screening tests for health professionals with form to complete
At each clinic attendance ensure parents are aware of the ‘red flags’ for cervical spine instability, and that they should seek immediate medical help if they are present.
Neck pain
Abnormal head posture
Torticollis
Reduced Neck Movements
Deterioration of gait and/or frequent falls
Increasing fatiguability on walking
Deterioration of Manipulative skills
Other signs of progressive myelopathy
Increase in muscle weakness
Loss of sensation
Onset of incontinence
Alteration in muscle tone
Decreasing co‐ordination
Diminishing kinaesthetic awareness
Pins and needles
For parents and carers:
https://www.downs-syndrome.org.uk/wp-content/uploads/2021/07/20.07.21_Neck-instability-v9-1-l.pdf
For GPs and health professionals:
Sudden dislocation of the neck probably occurs more commonly in people with Down Syndrome than in the rest of the population although it must be stressed that it is still very rare.
If a person with Down Syndrome does not have any of the symptoms already discussed, there is probably no reason for worry about them taking part in everyday routine sporting activities. However, no activity is risk free and so we have to be aware of the risks and make an informed decision about taking part in an activity. It’s a balancing act involving a decision about what is acceptable risk to you and a compromise between a person’s freedom to take part being weighed up against protecting them from possible injury.
There are sports (e.g. trampolining, gymnastics, boxing, diving, rugby and horse riding) that may carry with them more of a risk just as they do for anyone. Prior to taking part in such activities, it is strongly advisable to ask a GP, paediatrician or chartered physiotherapist to screen a person with Down Syndrome using the simple screening test developed by the British Gymnastics Association.
You can download information about the screening test:
https://www.british-gymnastics.org/technical-information/discipline-updates/disabilities
It does not specify how often this screening test needs to be done and the form updated. It is also very upsetting for parents and children if they are invited to for example a trampolining party and the venue refuses to let their child join in, unless they have a medical letter stating this is ok or the screening form above filled in. GPs are sometimes also worried about “signing-off” the form without medical expertise in this area, again causing anxiety for parents and it can be hard to access the community paediatrician with potentially such short notice or physiotherapist. This has been something discussed at the Leading Edge Group (LEG) for DS in Barnet, by the parental representatives. The screening test and form are simple and any health professional who knows the child and can screen with the questions and tests the form and information describes, can sign this form. This could be the physiotherapist, paediatrician, GP, school nurse and so on. Ideally too, every review by the community paediatrician and/or paediatric physiotherapist (although most children are not being seen once they start walking by this team) would not only check about symptoms of atlanto-axial instability but also complete and update the screening form. This is something all health professionals need to address and work towards.
It must be stressed that even if no symptoms are picked up by screening, this just means at the moment in time when screening took place, the evidence did not indicate that a person currently had neck instability. If you are all clear after a screening test you can still go on to develop neck instability.
There are no reliable screening tests to help us to predict those who might subsequently go on to develop a problem with their neck. This is why we all have to be aware of the warning signs that indicate that a person might be at risk of neck dislocation. If there is greater awareness, then people are more likely to consult with a doctor before any permanent damage happens and early treatment can take place.
