Learning about Intellectual Disabilities and Health

CERVICAL SPINE INSTABILITY IN PEOPLE WITH DOWN'S SYNDROME
Mary Lindsey (UK)

From CMO's Bulletin 1995:

Exercise is enjoyable and promotes heatlh, but there have been fears that vigorous activity might pose a risk to people with Down's Syndrome. Subluxation of the altanto-axial joint occaisionally leads to compression of the spinal cord 1. Guidance was issued in 1986 2, based on the advice of the Standing Medical Advisory Committee (SMAC), recommending that the neck of a person with Down's Syndrome shoudl be examined radiologically if vigorous sporting activity was envisaged.

More recent research indicates 3 that neck radiology does not reliably predict the risk of compresssion of the spinal cord, which has occurred after radiological investigation showed no abnormality 4, but sporting activity has bot been shown to incresase the risk5. SMAC has concluded that radiological examination is not helpful as a predictor of the risk of atlanto-axial subluxation and accordingly advice given in the 1986 letter 2 is now withdrawn. Instead, clinicians should be alert to clinical features to suggest acute or chronic compression of the spinal cord, and respond promptly. Anaesthetists and all clinicians involved in manipulation of the head should be aware of the increased risk of compression of the spinal cord in all persons with Down's Syndrome.

References

1 Davidson, R.G. (1988) Altanto-axial instability in individuals with Down's Syndrome: a fresh look at the evidence. Pediatrics 1988;81: 857-65.

2 Department of Health and Social Security. (1986) Atlanto-axial instability in people with Down's Syndrome. Heywood (Lancashire): DHSS (Professional letter: PL/CMO(86)9).

3 Selby, K.A., Newton, R.W., Gupta, S. & Hunt, L. (1991) Clinical predictors and radiological reliability in atlanto-axial subluxation in Down's Syndrome. Archive of Diseases in Childhood 1991;66: 876-8.

4 Hungerford, G.D., Akkaraya, V., Rowe, S.E. & Young, G.F. (1981) Atlanto-occipital and atlanto-axial dislocation with spinal cord compression. A case report and review of the literature. British Journal of Radiology 1981;54:758-61.

5 Cremers, M.J.G., Bol, E., de Roos, F. & van Gijn, J. (1993) Risk of sports activity in children with Down's Syndrome and atlanto-axial instability. Lancet 1993;342:511-4.