For Families and Carers : Neck Instability

Neck instability is an uncommon condition, which occurs in the rest of the population as well. Though it is still very uncommon, it is more common in people with Down’s syndrome.

“The reported incidence of asymptomatic craniovertebral instability in people with Down syndrome is between 10 and 24%, however, the incidence of symptomatic instability is much less and is around 1%.” Source: Down Syndrome – Current Perspectives, Edited by Richard W. Newton et al, Mac Keith Press, 2015.

What this means is that although between 10 and 24% of people with Down’s syndrome have the unstable neck joint, only around 1% of these people go on to develop the symptoms of neck instability (they are symptomatic).

The joints at the top of the spine and at the base of the skull allow us to shake and nod our heads. In people with Down’s syndrome, the ligaments (tissue that connects one bone to another bone thus holding a joint together) are stretchier. Therefore, joints may be looser and more flexible which can lead to slippage of the vertebrae.

Problems can develop if a vertebra slips too far and puts pressure on the nerves in the spinal cord. It can, in extreme cases, lead to sudden neck dislocation though this is rare. Slippage of the vertebrae can happen very gradually due to day-to-day wear and tear or it can happen suddenly as a result of a severe jolt or high impact.

In people with Down’s syndrome, the most common place for this slippage to happen is at the first and second vertebrae of the neck (known as ‘atlanto-axial instability’).

Similar problems can also occur between the base of the skull and the atlas vertebrae (known as ‘atlanto-occipital instability’) although this is not as common as atlanto-axial instability. These two conditions are collectively known as craniovertebral instability.

Evidence suggests most people with Down’s syndrome develop certain mild symptoms /warning signs before long-term damage takes place. These are:

  • Pain at a spot near the hard bump behind the ear
  • A stiff neck, which does not get better quickly
  • Unusual head posture
  • Changes in the way a person walks so that they may look unsteady on their feet
  • Change in a person’s ability to manipulate things with their hands

If a person is showing any of these symptoms, get them checked out by a doctor as soon as possible. If the symptoms come on suddenly, get the person an emergency appointment.

If a person does not have any of the above symptoms, there is no reason why they should not take part in normal sporting activities [1] with appropriate supervision. You should be aware that some sports (e.g. trampolining, gymnastics, boxing, kick boxing, diving, rugby, judo and horse riding) carry with them more of a risk because of increased likelihood of impact. You should contact the relevant sport national governing bodies to find out about their protocols for people who have Down’s syndrome.

Thankfully, as we know people with Down’s syndrome can have this issue, it means that measures can be put in place to optimise safety and to ensure maximum inclusion and participation in sporting activities and exercise. Our DSActive Team provide advice to coaches who are part of their programme about how their sessions/activities can be adapted to manage the risk of neck impact/injury.

Because of wider awareness of the potential issue of neck instability in people with Down’s syndrome, knowledge of the condition is included in the curriculum that all anaesthetists in training follow in the UK. Questions specifically referencing Down’s syndrome frequently appear in the examinations that all anaesthetists have to pass in order to become consultants so they may ensure the neck is properly supported during an operation.

For more information on the diagnosis and treatment of neck instability, this literature review may be of interest.

Information for GPs on cervical spine issues can be found here.

[1] Basic Medical Surveillance Essentials for people with Down syndrome CERVICAL SPINE DISORDERS: CRANIOVERTEBRAL INSTABILITY REVISED 2012 (One of a set of guidelines drawn up by the Down Syndrome Medical Interest Group (DSMIG UK)

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