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Oral and Swallowing Abilities Tool (OrSAT) in nusinersen treated patients

Beatrice Berti1, Lavinia Fanelli1, Giulia Stanca1

  • 1Pediatric Neurology Unit and Centro Clinico Nemo, Fondazione Policlinico Universitario Agostino Gemelli IRCCS, Roma, Lazio, Italy.

Archives of Disease in Childhood
|May 16, 2022
PubMed

Insights

Nusinersen treatment in infants with spinal muscular atrophy (SMA) shows promising results for swallowing function. Early intervention and assessment are key to predicting and improving swallowing abilities in these patients.

Area of Science:

  • Neurology
  • Pediatrics
  • Genetics

Background:

  • Type 1 spinal muscular atrophy (SMA) is a severe genetic neuromuscular disorder.
  • Swallowing difficulties are a common and serious complication in SMA patients.
  • Nusinersen is a targeted therapy for SMA.

Purpose of the Study:

  • To longitudinally evaluate the swallowing capabilities of infants with type 1 SMA receiving nusinersen treatment.
  • To identify factors influencing swallowing progression in treated SMA patients.

Main Methods:

  • A cohort of 20 infants with type 1 SMA (mean age at treatment initiation: 3 weeks to 15 months) was studied.
  • The Oral and Swallowing Abilities Tool (OrSAT) was used for swallowing assessment.
  • Follow-up duration ranged from 12 to 62 months post-treatment initiation.

Main Results:

  • 12/20 infants had normal swallowing at treatment start; 10 maintained this throughout follow-up.
  • Two infants initially requiring tube feeding regained some oral intake.
  • Of 8 infants with pre-existing tube feeding, 4 with tracheostomies showed no change, while 4 without tracheostomies demonstrated partial improvement.

Conclusions:

  • The severity of functional impairment at the start of nusinersen treatment correlates with swallowing ability progression.
  • Structured swallowing assessments like OrSAT can detect subtle functional improvements in SMA patients.
Abstract

Keywords:
child developmentneurologypaediatrics

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