The Journal of Bone and Joint Surgery, Vol 68, Issue 4 564-572, Copyright © 1986 by Journal of Bone and Joint Surgery, Inc
Natural history of scoliosis in Friedreich's ataxia
H Labelle, S Tohme, M Duhaime and P Allard
Does scoliosis associated with Friedreich's ataxia behave like an
idiopathic or a typical neuromuscular scoliosis? Should it be treated like
an idiopathic or a neuromuscular curve? Since no precise information to
answer these questions could be found in the orthopaedic literature, a
retrospective study was undertaken of seventy-eight patients with
Friedreich's ataxia who had been followed at our neuromuscular clinic.
Fifty-six of these patients were found to have typical Friedreich's ataxia
in accordance with the criteria of Geoffroy et al., and their cases were
retained for analysis. Their mean age was twenty years (range, eight to
thirty-three years). The average length of clinical follow-up was nine
years and the average duration of radiographic follow-up of the scoliosis
was 3.5 years. A scoliosis of more than 10 degrees was found in all
patients and was associated with a hyperkyphosis in 66 per cent. Both sexes
were equally affected. Fifty-seven per cent of the curves were double
thoracic and lumbar; 14 per cent were thoracolumbar; 7 per cent, double
thoracic and thoracolumbar; 7 per cent, thoracic; 4 per cent, lumbar; and
11 per cent, multiple small curves. Of the fifty-six patients whose cases
were studied, thirty-six had been followed for at least ten years. Among
these thirty-six, there were twenty whose curves were more than 60 degrees
and progressed (Group I) and sixteen whose curves were 40 degrees or less
and did not progress (Group II).(ABSTRACT TRUNCATED AT 250 WORDS)
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