Universidade de São Paulo, Faculdade de Medicina, Departamento de Neurologia, São Paulo SP, Brazil.
Universidade de São Paulo, Faculdade de Medicina, Departamento de Anestesiologia, São Paulo SP, Brazil.
Arq Neuropsiquiatr. 2021 Feb;79(2):127-132. doi: 10.1590/0004-282X-ANP-2020-0200.
Spinal muscular atrophy (SMA) is a neurodegenerative disease of lower motor neurons associated with frequent occurrence of spinal deformity. Nusinersen is an antisense oligonucleotide that increases SMN protein level and is administrated by frequent intrathecal lumbar injections. Thus, spinal deformities and previous spinal surgery are important challenges for drug delivery in SMA.
To report imaging methods used for Nusinersen injection in SMA patients.
Nusinersen injection procedures in SMA types 2 and 3 patients who had previous spinal surgery were analyzed retrospectively to describe the imaging and puncture procedures, as well as the occurrence of complications.
Nine SMA patients (14 to 50 years old) underwent 57 lumbar punctures for nusinersen injection. Six patients had no interlaminar space available; in five of them, a transforaminal approach was used, and another one underwent a surgery to open a posterior bone window for the injections. Transforaminal puncture was performed using CT scan in three cases and fluoroscopy in the other two, with a similar success rate. One patient in the transforaminal group had post-procedure radiculitis, and another one had vagal reaction (hypotension). In three cases, with preserved interlaminar space, injections were performed by posterior interlaminar puncture, and only one adverse event was reported (post-puncture headache).
In SMA patients with previous spinal surgery, the use of imaging-guided intervention is necessary for administering intrathecal nusinersen. Transforaminal technique is indicated in patients for whom the interlaminar space is not available, and injections should always be guided by either CT or fluoroscopy.
脊髓性肌萎缩症（SMA）是一种与脊柱畸形频繁发生相关的下运动神经元退行性疾病。Nusinersen 是一种反义寡核苷酸，可提高 SMN 蛋白水平，并通过频繁的鞘内腰椎注射给药。因此，脊柱畸形和先前的脊柱手术是 SMA 药物输送的重要挑战。
报告用于 SMA 患者 Nusinersen 注射的影像学方法。
回顾性分析了有既往脊柱手术史的 SMA 2 型和 3 型患者的 Nusinersen 注射程序，以描述影像学和穿刺程序，以及并发症的发生情况。
9 例 SMA 患者（14-50 岁）接受了 57 次腰椎穿刺以注射 Nusinersen。6 名患者没有可用的椎板间隙；其中 5 名患者采用经椎间孔入路，另 1 名患者接受了后路开骨窗手术以进行注射。经椎间孔穿刺在 3 例中使用 CT 扫描，在另外 2 例中使用透视，成功率相似。经椎间孔组中有 1 例患者出现术后神经根炎，另 1 例患者出现迷走神经反应（低血压）。在 3 例保留椎板间隙的患者中，通过后椎板间穿刺进行注射，仅报告 1 例不良事件（穿刺后头痛）。
对于有既往脊柱手术史的 SMA 患者，鞘内给予 Nusinersen 需要进行影像学引导的介入。对于椎板间隙不可用的患者，经椎间孔技术是指征，注射应始终通过 CT 或透视引导。
