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In male children with Duchenne muscular dystrophy (DMD), the increase in motor skills around their expected peak age suggests that they will reach a later age before losing their ability to stand and walk. walk, according to the results of a study recently published in Developmental medicine and child neurology.
In children born with DMD who have never received corticosteroid therapy, a progressive weakening of muscle, pulmonary and cardiac functions progressing to loss of walking ability (LOA) occurs at the age of 10 years. However, patients who have received long-term treatment with corticosteroids will experience LOA 2 to 4 years later. The North Star Ambulatory Assessment (NSAA) shows that the functional abilities of healthy men peak at 4 years of age and those with DMD typically between 6 and 7 years of age.
The objective of the present study was to assess the association between functional test scores (NSAA and timed rise from the floor [TRF]) recorded at the peak age for male children with DMD, and the age of these children at LOA.
Researchers conducted a retrospective study of 293 male DMD patients enrolled in the UK North Star Network database of 13 centers with an NSAA score taken between 6 years and 7 years 6 months, at least one visit over 8 years , and a follow-up beyond 3 years. At baseline (6 years 10 months ± 6 months), the highest median NSAA score was 27 (IQR: 22-31) and the mean TRF was 4.9 seconds (± 2.9). Baseline corticosteroid use data were available for 224 patients. An additional 30 patients had never been treated with steroids, although 23 of these patients subsequently received corticosteroid treatment.
For the entire cohort (first visit at 5 years 6 months ± 1 year 2 months; last visit 12 years 8 months ± 2 years 11 months; median follow-up 7 years 4 months) a higher initial NSAA and a low associated TRF at an advanced age at LOA (P <.001 nsaa scores of to had a probability versus with remaining ambulant beyond age the patients in study lost their ability walk median at loa years months month researchers noted multivariate analysis daily trf and corticosteroid regimens intermittent were all independently associated outcome>P =.01). »
Limitations of the study included: missing data in the NSAA database, a high percentage of patients switching steroid type or regimen, and review of data from only one standard of care country.
The researchers concluded that “NSAAs and TRFs recorded at expected peak age are significant determinants of age at LOA in male children with DMD, the latter being more important even when recorded early in the course of the disease. This could be useful knowledge for early stage family counseling regarding prognosis.
Zambon AA, Ayyar Gupta V, Ridout D, et al. Maximum functional capacity and age at loss of walking in Duchenne muscular dystrophy. Dev Med Child Neurol. Published online February 14, 2022. doi:10.1111/dmcn.15176