Ultrasound-assisted intrathecal injection of nusinersen in a patient with severe vertebral deformity: a case report

Abstract

Background: Spinal muscular atrophy (SMA) is a mostly autosomal recessive genetic disease characterized by progressive muscle weakness from anterior horn degeneration. Nusinersen has recently been approved as a disease-modifying drug for SMA that needs to be administered intrathecally. Its injection is often associated with extreme difficulty since patients with SMA have severe vertebral deformity and may be with vertebral instrumentation. Case description: A 21-year-old female with type 2 SMA and spinal deformity underwent a series of intrathecal injections of nusinersen. The intrathecal injections have been safely and successfully done by using computed tomography imaging and ultrasonography-assisted technique. Conclusion: This the first report in which ultrasound-assisted technique has been used for the injection of nusinersen through a lumbar puncture in patients with severe spinal deformity. Use of preprocedural ultrasound imaging is highly recommended for treatments that repeatedly require intrathecal access.

Ultrasound‐guided lumbar puncture for nusinersen administration in spinal muscular atrophy patients

“…Nevertheless, cervical approaches are used off label, and little is known about their influence in the pharmacodynamics or efficacy of nusinersen. To the best of our knowledge, the US‐guided lumbar puncture has scarcely been reported to treat SMA patients with complex spines [13]. In this study, all patients younger than 20 years old could be treated and our success rate (96.8%) was similar to previously reported transforaminal approaches [6–9].…”

Ultrasound assisted lumbar intrathecal administration of nusinersen in adult patients with spinal muscular atrophy: A case series

“…Transforaminal approach is effective but presents a higher risk of radicular artery puncture than the interlaminar approach. [20][21][22][23] US or fluoroscopy guided cervical punctures are also described [6][7][8][9][10][11][12][13][14][15][16][17][18][19][20][21][22][23][24] but are used off label and their impacts on nusinersen efficacy are unknown. A recent US guided interlaminar lumbar approach has been proposed and presented as safe and effective: in that paper, 18 SMA patients (children, adolescents, adults) with severe scoliosis and spondylodesis were treated, with a high percentage of success (96.8% of attempts, 77.8% of all patients, 50% of adult patients) and very few AEs.…”

“…We report our experience in US assisted intrathecal nusinersen administration in SMA adults with difficult spines and previous difficulties in successfully undergoing intrathecal treatments, in order to expand the limited evidence so far reported 5‐17 and to encourage the use of US to facilitate intrathecal access.…”

“…[10][11][12] In adults with difficult spines, 13,14 the use of US may decrease the number of attempts 15 and of needle passes necessary for successful spinal anesthesia, 15 subsequently reducing the risk of post dural puncture headache (PDPH), low back pain (LBP) and low patient satisfaction often associated with multiple needle punctures. 16 We report our experience in US assisted intrathecal nusinersen administration in SMA adults with difficult spines and previous difficulties in successfully undergoing intrathecal treatments, in order to expand the limited evidence so far reported [5][6][7][8][9][10][11][12][13][14][15][16][17] and to encourage the use of US to facilitate intrathecal access.…”

Ultrasound‐guided transforaminal approach for nusinersen delivery in adult spinal muscle atrophy patients with challenging access

“…Although ultrasound (US) is recognized as a useful adjunct for lumbar spinal anesthesia in adults, there are only a few papers on US utilization for intrathecal nusinersen delivery in spinal muscle atrophy (SMA) patients, all of which describe intrathecal nusinersen delivery via the translaminar approach 1‐6 . Veiga‐Canuto et al reported that US‐guided translaminar approach was unsuccessful in patients without visible interlaminar space on pre‐procedural computed tomography (CT) 2 .…”