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经旁正中入路给予nusinersen 治疗脊髓性肌萎缩症。

Administration of nusinersen via paramedian approach for spinal muscular atrophy.

机构信息

Department of Pediatrics, Aichi Medical University, Aichi, Japan.

Department of Orthopedics, Aichi Medical University, Aichi, Japan.

出版信息

Brain Dev. 2021 Jan;43(1):121-126. doi: 10.1016/j.braindev.2020.07.014. Epub 2020 Aug 6.

DOI:10.1016/j.braindev.2020.07.014
PMID:32773161
Abstract

OBJECTIVE

To 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.

METHODS

Seven 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.

RESULTS

Intrathecal 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.

CONCLUSIONS

Intrathecal 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.

摘要

目的

评估鞘内注射依那西普通过旁正中入路在伴有脊柱侧凸的脊髓性肌萎缩症(SMA)青少年和成人中的成功率、手术时间和不良事件。

方法

纳入 7 名经基因证实的 SMA 患者(年龄 12-40 岁)。通过三维 CT 确定 L2-L3、L3-L4 或 L4-L5 之间最大的椎间孔后,使用透视法通过旁正中入路进行鞘内注射依那西普。我们测量了准备、定位和穿刺的时间以及总停留时间。记录鞘内给药的不良反应。

结果

旁正中入路鞘内给药在所有 38 次机会中均成功。中位总停留时间为 44.0 分钟(四分位间距,37.3-50.0 分钟)。SMA 1 型患者的总停留时间明显长于 SMA 2 型患者,但与脊柱侧凸的严重程度无关。不良事件包括氧疗、头痛和背痛。镇静与氧疗和头痛有关。

结论

鞘内注射依那西普通过旁正中入路在伴有脊柱侧凸的 SMA 患者中具有成功率高、手术时间短、不良事件少的优点。

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