The Journal of Bone and Joint Surgery, Vol 58, Issue 1 13-24, Copyright © 1976 by Journal of Bone and Joint Surgery, Inc
Spinal deformity associated with heritable neurological conditions: spinal muscular atrophy, Friedreich's ataxia, familial dysautonomia, and Charcot-Marie-Tooth disease
RN Hensinger and GD MacEwen
Children with progressive neurological conditions such as spinal muscular
atrophy, Friedreich's ataxia, familial dysautonomia (Riley-Day syndrome),
and Charcot-Marie-Tooth disease have a significant risk of acquiring a
serious spinal deformity. As with paralytic scoliosis following
poliomyelitis, the curves are difficult to control with bracing, and
progression does not cease with maturation. An increasing spinal curvature
may lead to loss of ambulation or, for the wheelchair-bound patient, loss
of sitting balance. The curvature may further compromise pulmonary function
that may already be compromised by the neurological deficit. Twenty
patients are reported with an average follow-up of six years (range, one to
fourteen years). For the properly selected patient, surgical stabilization
of the spine arrested the progress of the curve and improved function.
Complications were few; however, pseudarthrosis was more common than in
patients with non-neurological problems.
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