Abstract
BackgroundThis observational study describes our experience delivering nusinersen through lumbar puncture with real-time ultrasound guidance in spinal muscular atrophy (SMA) patients with severe scoliosis.ResultsIntrathecal nusinersen via real-time ultrasound-guided lumbar puncture was given to three patients who had severe thoracic and lumbar scoliosis: a 34-year-old female with type 3a SMA, a 28-year-old male with type 2a SMA, and a 14-year-old girl with type 3a SMA. Lumbar puncture was performed without sedation under ultrasound guidance using a 22G echogenic needle in the interlaminar aspect of the L4–L5 or L5–S1 interspace and a full dose of nusinersen (12 mg/5 mL) was injected after visualizing free cerebrospinal fluid flow. Patients completed their four loading doses and one maintenance dose of nusinersen. All 15 procedures were successful and well tolerated.ConclusionsReal-time ultrasound-guided lumbar puncture is an effective and radiation-free technique to administer intrathecal nusinersen in SMA patients with severe scoliosis when done by practitioners with expertise in this procedure.
Highlights
This observational study describes our experience delivering nusinersen through lumbar puncture with real-time ultrasound guidance in spinal muscular atrophy (SMA) patients with severe scoliosis
The nonradiation, real-time, Zhang et al Orphanet J Rare Dis (2021) 16:274 ultrasound-guided technique, which has been reported to be successfully used by anesthesiologists for central neuraxial block in patients with moderate to severe lumbar scoliosis [8, 9], may be a valuable alternative for nusinersen delivery in this group of patients
We report our clinical experience on a total of 15 repeated nusinersen deliveries through ultrasound-guided real-time lumbar puncture in three SMA patients with severe scoliosis, including detail on the technical essentials of this technique
Summary
This observational study describes our experience delivering nusinersen through lumbar puncture with real-time ultrasound guidance in spinal muscular atrophy (SMA) patients with severe scoliosis. Zhang et al Orphanet J Rare Dis (2021) 16:274 ultrasound-guided technique, which has been reported to be successfully used by anesthesiologists for central neuraxial block in patients with moderate to severe lumbar scoliosis [8, 9], may be a valuable alternative for nusinersen delivery in this group of patients. We report our clinical experience on a total of 15 repeated nusinersen deliveries through ultrasound-guided real-time lumbar puncture in three SMA patients with severe scoliosis, including detail on the technical essentials of this technique
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