Scoliosis in Friedreich's ataxia: longitudinal characterization in a large heterogeneous cohort
| العنوان: | Scoliosis in Friedreich's ataxia: longitudinal characterization in a large heterogeneous cohort |
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| المؤلفون: | J. Chad Hoyle, S. H. Subramony, John M. Flynn, Theresa A. Zesiewicz, Louise A. Corben, Christian Rummey, Katherine D. Mathews, George Wilmot, Lauren Seeberger, Antoine Duquette, David A. Lynch, Grace Yoon, Khalaf Bushara, Richard Roxburgh, Martin B. Delatycki, Susan Perlman, Christopher M. Gomez |
| المصدر: | Annals of Clinical and Translational Neurology Annals of Clinical and Translational Neurology, Vol 8, Iss 6, Pp 1239-1250 (2021) |
| بيانات النشر: | John Wiley and Sons Inc., 2021. |
| سنة النشر: | 2021 |
| مصطلحات موضوعية: | 0301 basic medicine, Adult, Pediatrics, medicine.medical_specialty, Ataxia, Adolescent, Neurosciences. Biological psychiatry. Neuropsychiatry, Scoliosis, 03 medical and health sciences, Young Adult, 0302 clinical medicine, medicine, Prevalence, Humans, Longitudinal Studies, Age of Onset, RC346-429, Child, Research Articles, business.industry, General Neuroscience, Incidence (epidemiology), Middle Aged, medicine.disease, Comorbidity, Natural history, 030104 developmental biology, Friedreich Ataxia, Cohort, Disease Progression, Neurology (clinical), Neurology. Diseases of the nervous system, medicine.symptom, Age of onset, business, 030217 neurology & neurosurgery, Natural history study, RC321-571, Research Article |
| الوصف: | 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. |
| اللغة: | English |
| تدمد: | 2328-9503 |
| الوصول الحر: | https://explore.openaire.eu/search/publication?articleId=doi_dedup___::3033a09bc54dc45180d9480c5edd3dd6Test http://europepmc.org/articles/PMC8164850Test |
| حقوق: | OPEN |
| رقم الانضمام: | edsair.doi.dedup.....3033a09bc54dc45180d9480c5edd3dd6 |
| قاعدة البيانات: | OpenAIRE |
| تدمد: | 23289503 |
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