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Scoliosis in Friedreich’s Ataxia

Published online by Cambridge University Press:  18 September 2015

P. Allard ,
J. Dansereau ,
P.S. Thiry ,
G. Geoffroy ,
J.V. Raso  and
M. Duhaime
P. Allard
Affiliation:
Pediatric Research Center, Sainte-Justine Hospital and the Department of Mechanical Engineering, Ecole Polytechnique de Montréal and the Assessment Department, Glenrose Hospital, Alberta
J. Dansereau
Affiliation:
Pediatric Research Center, Sainte-Justine Hospital and the Department of Mechanical Engineering, Ecole Polytechnique de Montréal and the Assessment Department, Glenrose Hospital, Alberta
P.S. Thiry
Affiliation:
Pediatric Research Center, Sainte-Justine Hospital and the Department of Mechanical Engineering, Ecole Polytechnique de Montréal and the Assessment Department, Glenrose Hospital, Alberta
G. Geoffroy
Affiliation:
Pediatric Research Center, Sainte-Justine Hospital and the Department of Mechanical Engineering, Ecole Polytechnique de Montréal and the Assessment Department, Glenrose Hospital, Alberta
J.V. Raso
Affiliation:
Pediatric Research Center, Sainte-Justine Hospital and the Department of Mechanical Engineering, Ecole Polytechnique de Montréal and the Assessment Department, Glenrose Hospital, Alberta
M. Duhaime
Affiliation:
Pediatric Research Center, Sainte-Justine Hospital and the Department of Mechanical Engineering, Ecole Polytechnique de Montréal and the Assessment Department, Glenrose Hospital, Alberta
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Summary:

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The preliminary results based on a three year study on the evolution and management of scoliosis in Friedreich’s ataxia are presented. Thirty-two patients were followed in the Neuromuscular Disease Clinic at Sainte-Justine Hospital where standardized spinal radiographs were taken periodically with the Scoliosis Chariot and the Throne.

Pathomechanics — Between the age groups I (5 – 10 years) and II (10 – 15 years), a substantial increase in the Cobb values occurs. Associated with it, an increase was observed in the thoracic and thoraco-lumbar projected surface area indices. The relative torsion between the thoracic and lumbar segments was associated with a sudden increase in the Cobb measurements.

A computer program which generates a tri-dimensional display of the spine from the coordinates obtained from the standardized radiographs has been adapted to study the importance and influence of the thoracic torsion on the progress of the scoliosis and on the evolution of the total spinal deformity.

Management — Prevention of the progression of established curves was our main objective. Careful examination of the spine, depending on the age of the child, in our preliminary study, stimulated early orthopaedic treatment in any curve of 20° or more. There was always concern for curves of 30° or more. In the growing child, bracing which was recommended is now under a more thorough investigation. The use of spinal pacemakers is being clinically evaluated.

In the older child, the curve was usually stable after sixteen years of age. Surgery was usually attempted in curves over 40° in the growing child. The same curve was usually stable after the growth period.

For the non-ambulatory patients, the present study suggested the prescription of a molded seat with the following characteristics: i) a posterior lumbar support, ii) low thoracic lateral supports and Hi) a slight inclination of the seating system. This was presumed to be beneficial in maintaining stability of the spine.

Type
Research Article
Information
Canadian Journal of Neurological Sciences , Volume 9 , Issue 2 , May 1982 , pp. 105 - 111
Copyright
Copyright © Canadian Neurological Sciences Federation 1982

References

REFERENCES

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