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Comparison of Nusinersen Monotherapy Versus Combination Therapy With Nusinersen and Onasemnogene Abeparvovec in Spinal Muscular Atrophy Type 1 Patients With Two SMN2 Copies: A Multicenter Study From T

5 hours ago
  • #nusinersen
  • #spinal muscular atrophy
  • #onasemnogene abeparvovec
  • Comparison of nusinersen monotherapy versus combination therapy (nusinersen and onasemnogene abeparvovec) in SMA type 1 patients with two SMN2 copies.
  • Retrospective multicenter study involving 82 patients (42 monotherapy, 40 combination therapy).
  • No significant difference in motor milestones, CHOP-INTEND scores, or respiratory/nutritional status between groups.
  • All deaths (n=8) occurred in the monotherapy group among patients treated after 3 months of age (p=0.005).
  • Early treatment initiation correlated with better feeding outcomes (OR: 0.740, p=0.024).
  • Pre-treatment respiratory and feeding status were predictive of outcomes.
  • Combination therapy showed no clear advantage over monotherapy; early treatment was the main determinant of outcomes.