Nusinersen does not prevent hip instability in SMA type II

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Researchers in Hong Kong investigated the extent to which treatment with intrathecal nusinersen influenced the risk of bilateral hip dislocation, a complication very frequently observed in spinal muscular atrophy type II (SMA II) linked to the SMN1 gene :

  • 10 children with SMA type II were included in the study, which ran for three and a half years (2018-2022),
  • the median age of this cohort was 6.8 years,
  • all participants were symptomatic at the time of initiation of nusinersen,
  • although 60% of the children acquired walking ability with treatment, the majority of their hips remained subluxated or at risk of subluxation.

The authors incriminate a hip abductor muscle deficit that persisted despite treatment.

 

Nusinersen Initiation After Onset of Weakness Does Not Prevent Progression of Hip Instability. Kuong EE, Ip HNH, So NLW et al. J Pediatr Orthop. 2024 Apr 29.

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