A German team conducted a retrospective cross-sectional study of 75 patients to assess the course of neuromuscular scoliosis associated with SMA.
- a total of 75 patients were included in the analysis; 36.5% had type I SMA, 40.5% had type II SMA and 23% had type III SMA.
- all were treated with Spinraza®, Zolgensma® and Evrysdi® as monotherapy or in combination, with first treatment received at a mean age of 4.8 years.
- during the course of the study, 50 patients were diagnosed with scoliosis, 24 of whom required surgery.
- the mean age at diagnosis of scoliosis was 2 years for type I SMA, 4.6 years for type II SMA and 14 years for type III SMA.
- the mean Cobb angles at the time of detection of scoliosis were 30.1°, 38.7° and 49.4° respectively.
- the mean age at the time of scoliosis surgery was 5 years for type I SMA, 7.4 years for type II SMA and 10.3 years for type III SMA.
- the mean Cobb angles at the time of surgery were 70.1°, 55.3° and 69.8° respectively.
- a positive correlation between pelvic obliquity and scoliosis was found, with a higher prevalence in types I and II.
- no correlation was found between the CHOP-INTEND functional motor scale and Cobb angles, but a negative correlation was found with the HFMSE score, suggesting that the higher the score, the less severe the development of scoliosis.
These results suggest an increase in the number of children with SMA type I and II being treated who will develop early-onset scoliosis, hence the need for complementary multidisciplinary management and early surgical intervention.
