• Hideyuki Iwayama, Norimitsu Wakao, Hirokazu Kurahashi, Norika Kubota, Ayako Hattori, Toshiyuki Kumagai, and Akihisa Okumura.
• Department of Pediatrics, Aichi Medical University, Aichi, Japan. Electronic address: iwahide1976@gmail.com.
• Brain Dev. 2021 Jan 1; 43 (1): 121-126.

ObjectiveTo assess the success rate, procedure time, and adverse events of intrathecal administration of nusinersen via the paramedian approach in adolescents and adults with spinal muscular atrophy (SMA) associated with scoliosis.MethodsSeven patients with genetically confirmed SMA (age, 12-40 years) were included. Intrathecal administration of nusinersen was performed via paramedian approach using fluoroscopy after determination of the largest interlaminal foramen among L2-L3, L3-L4, or L4-L5 by three-dimensional computed tomography. We measured the times for preparation, positioning, and puncture, and the total time of stay. Adverse effects of intrathecal administration were noted.ResultsIntrathecal administration via paramedian approach was successful for all 38 opportunities. The median total time of stay was 44.0 min (interquartile range, 37.3-50.0 min). The total time of stay was significantly longer in patients with SMA type 1 than in those with SMA type 2, but was not different according to the severity of scoliosis. Adverse effects included oxygen supplementation, headache, and back pain. Sedation was correlated with oxygen supplementation and headache.ConclusionsIntrathecal administration of nusinersen via the paramedian approach had the advantages of a high success rate and short procedure time with fewer adverse events in SMA patients associated with scoliosis.Copyright © 2020 The Japanese Society of Child Neurology. Published by Elsevier B.V. All rights reserved.

22 keywords...

hide…