Scoliosis in Friedreich’s Ataxia

Abstract

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 RESUME: Les resultats de trois annees d'e'tude sur revolution et le traitement de la scoliose dans I'ataxie de Friedreich sont presentes dans cet article. Trente-deux patients ont ete suivis a I'Hopital Sainte-Justine aux Cliniques des maladies neuromusculaires ou des prises de radiographic de la colonne sont normalisees au moyen du Chariot de la scoliose et du Trone.Pathomecanique -Entre les groupes d'dge I (5 -10 ans) et 11 (10-15 ans), il existe une augmentation substantielle des valeurs de I'angle de Cobb. Associe a ceci, une augmentation est aussi observe'e dans les indices de la surface projete'e. La torsion relative des segments thoracique et lombaire de la colonne vertebrate semble etre associee a une augmentation subite dans les mesures de I'angle de Cobb.A la suite des resultats obtenus relativement a un parametre global de deformation 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 (la surface projetee), une etude de la torsion thoracique a ete effectuee. Par le biais de simulations mathematiques, il a ete revile que la surface projetee varie enfonction de I'angle de torsion pour divers angles de Cobb. En comparaison avec les donnees cliniques obtenues a partir des radiographics normalisees de la colonne, ces simulations ont permis d'evaluer la contribution de la torsion sur Tensemble des deformations associees a la scoliose.Traitement -La prevention de la progression de la scoliose etablie represente notre objectif principal. Une evaluation de la colonne des patients de differents groupes d'dge stimulera un traitement orthopedique precoce de toute courbe de 20° ou plus. Une anxiete persiste lorsque des courbes de 30° ou plus sont observees. Chez I'enfant en croissance, le port du corset qui etait autrefois reco...

Genetic Disorders Producing Compressive Radiculopathy

“…It seems highly probable that disorders producing spinal deformity would eventually cause radiculopathy by either compression of roots in the neural foramina or by dislocation of intervertebral discs. Neurological diseases resulting in scoliosis include CMT, 16,38 spinal muscular atrophy, 38 Friedreich's ataxia, 38,39 familial dysautonomia, 38 and idiopathic torsional dystonia. 40,41 However, although there are several studies examining scoliosis in these patients, there are no reports in the literature of these diseases causing radiculopathy due to spinal deformity.…”

Scoliosis assessment in friedreich's ataxia by means of intrinsic parameters

“…The previous studies (Allard et al, 1980(Allard et al, , 1982 have been expanded to cover a five year period of evaluation where twenty- eight boys and twenty-five girls were seen on the average once a year in the Neuro-muscular Disease Clinic at Sainte-Justine and Marie Enfant hospitals. It was thought necessary to investigate the progression of scoliosis from the onset when the patients were ambulatory to the time when they were wheelchair-bound with severe structural deformation of the spine.…”

“…To minimize the systematic errors due to positioning of the patient and to allow a tri-dimensional reconstruction shape of the spine, two devices, namely the Scoliosis Chariot and the Throne have been utilised to obtain standardized radiographs of ambulatory and non-ambulatory patients. The description and utilisation of these devices have been reported previously in this journal (Allard et al, 1982).…”

“…In a recent study, Allard et al (1982) have investigated the spinal deformity evolution in Friedreich's ataxia with a spatial reconstruction program using cubic spline functions. The program computes tri-dimensional parameters of a geometric curve based on the location of the centroid of each thoracic and lumbar vertebra obtained from standardized bi-planar radiographs according to that of McNeice et al (1975).…”

Scoliosis in patients with Friedreich’s ataxia

“…[4][5][6][7][8][10][11][12] The purpose of this study is to evaluate the demographics, progression of spinal deformity and the effect of bracing and surgical treatment in patients with FA.…”