Scoliosis in Friedreich's ataxia: longitudinal characterization in a large heterogeneous cohort.
Ann Clin Transl Neurol
; 8(6): 1239-1250, 2021 06.
Article
en En
| MEDLINE
| ID: mdl-33949801
ABSTRACT
OBJECTIVE:
The objective of this study was to characterize the incidence and progression of scoliosis in the natural history of Friedreich's ataxia (FRDA) and document the factors leading to the requirement for corrective surgery.METHODS:
Data on the prevalence of scoliosis and scoliosis surgery from up to 17 years of follow-up collected during a large natural history study in FRDA (1116 patients at 4928 visits) were summarized descriptively and subjected to time to event analyses.RESULTS:
Well over 90% of early or typical FRDA patients (as determined by age of onset) developed intermediate to severe scoliosis, while patients with a later onset (>14 years) had no or much lower prevalence of scoliosis. Diagnosis of scoliosis occurs during the onset of ataxia and in rare cases even prior to that. Major progression follows throughout the growth phase and puberty, leading to the need for surgical intervention in more than 50% of individuals in the most severe subgroup. The youngest patients appear to delay surgery until the end of the growth period, leading to further progression before surgical intervention. Age of onset of FRDA before or after reaching 15 years sharply separated severe and relatively mild incidence and progression of scoliosis.INTERPRETATION:
Scoliosis is an important comorbidity of FRDA. Our comprehensive documentation of scoliosis progression in this natural history study provides a baseline for comparison as novel treatments become available.
Texto completo:
1
Base de datos:
MEDLINE
Asunto principal:
Escoliosis
/
Ataxia de Friedreich
Tipo de estudio:
Clinical_trials
/
Observational_studies
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Prevalence_studies
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Risk_factors_studies
Límite:
Adolescent
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Adult
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Child
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Humans
/
Middle aged
Idioma:
En
Revista:
Ann Clin Transl Neurol
Año:
2021
Tipo del documento:
Article
País de afiliación:
Suiza