Natural course of scoliosis and lifetime risk of scoliosis surgery in spinal muscular atrophy.
Adolescent
Adult
Aged
Aged, 80 and over
Child
Child, Preschool
Cohort Studies
Cross-Sectional Studies
Disease Progression
Female
Humans
Infant
Infant, Newborn
Longitudinal Studies
Male
Middle Aged
Muscular Atrophy, Spinal
/ complications
Orthopedic Procedures
/ standards
Scoliosis
/ etiology
Young Adult
Journal
Neurology
ISSN: 1526-632X
Titre abrégé: Neurology
Pays: United States
ID NLM: 0401060
Informations de publication
Date de publication:
09 07 2019
09 07 2019
Historique:
received:
01
08
2018
accepted:
20
02
2019
pubmed:
6
6
2019
medline:
7
1
2020
entrez:
6
6
2019
Statut:
ppublish
Résumé
To investigate the natural course of scoliosis and to estimate lifetime probability of scoliosis surgery in spinal muscular atrophy (SMA). We analyzed cross-sectional data from 283 patients from our population-based cohort study. Additional longitudinal data on scoliosis progression and spinal surgery were collected from 36 consecutive patients who received scoliosis surgery at our center. The lifetime probability of receiving scoliosis surgery was ≈80% in SMA types 1c and 2. Patients with type 2 who only learned to sit (type 2a) were significantly younger at time of surgery than those who learned to sit and stand (type 2b). The lifetime risk of surgery was lower in type 3a (40%) and strongly associated with age at loss of ambulation: 71% in patients losing ambulation before 10 years of age vs 22% losing ambulation after the age of 10 years ( The lifetime probability of scoliosis surgery is high in SMA types 1c and 2 and depends on age at loss of ambulation in type 3. Motor milestones such as standing that are not part of the standard classification system are of additional predictive value. Our data may act as a reference to assess long-term effects of new SMA-specific therapies.
Identifiants
pubmed: 31164393
pii: WNL.0000000000007742
doi: 10.1212/WNL.0000000000007742
doi:
Types de publication
Journal Article
Research Support, Non-U.S. Gov't
Langues
eng
Sous-ensembles de citation
IM
Pagination
e149-e158Informations de copyright
© 2019 American Academy of Neurology.