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

Veiga‐Canuto, Diana; Cifrián-Pérez, Manuel; Castellano, Inmaculada Pitarch; Vázquez-Costa, J.F.; Aparici, Fernando

doi:10.1111/ene.14586

Cited by 23 publications

(25 citation statements)

References 14 publications

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“…Conversely, some patients with severe scoliosis but less rotation were easily treated without the need for US. So the Cobb's angle alone appears not to influence the success of the procedure, as recently reported 5 . In our patients, the US assisted procedure was always successful, although several attempts were still required in some.…”

Section: Discussionsupporting

confidence: 76%

“…US or fluoroscopy guided cervical punctures are also described 6‐24 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 5 . Despite some differences in the clinical characteristics of our patients (no spondylodesis, no children or adolescents included) and in procedure (assisted versus guided, 25 versus 22 gauge needle) our experience confirms and strengthens the results of the other group.…”

Section: Discussionmentioning

confidence: 99%

“…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.…”

Section: Introductionmentioning

confidence: 99%

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

et al. 2021

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Introduction/Aims Intrathecal nusinersen administration can be challenging in certain adult spinal muscular atrophy (SMA) patients with difficult spinal anatomy who require imaging techniques (fluoroscopy or computed tomography scans) or invasive approaches (catheter placement, laminotomy) to identify the intrathecal space. We used ultrasound (US) assistance to access the lumbar intrathecal space in patients with SMA who experienced previous difficulties or failures with intrathecal dosing. Methods Eighteen adult patients with difficult spines were enrolled. We used US assistance, and we recorded the successful administrations, number of attempts, procedure times, and “patient satisfaction.” Results There were 57 consecutive successful nusinersen spinal administrations in all patients enrolled. In 50% of patients, two or fewer attempts were needed to obtain a successful administration, with four or fewer attempts in 83.3%; only three patients reported more than four attempts because of both severe scoliosis and severe spine rotation (two patients) and obesity (one patient). The mean procedure time was 11.8 min (range, 1.7–28.9). Patient satisfaction was 4.97/5 (range, 4–5; median, 5) on Likert scale at 5 min and at 72 h. No major adverse events were reported, and two post dural puncture headaches were managed with medical therapy and with complete resolution within 72 h. Discussion US assistance seems to be a valid option among treatment choices for intrathecal nusinersen administration in patients with difficult spine. The absence of radiation exposure and the lack of need for intravenous sedation or general anesthesia are additional potential advantages to US assisted administration.

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Section: Discussionsupporting

confidence: 76%

Section: Discussionmentioning

confidence: 99%

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

et al. 2021

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“…Most AEs are related to the administration procedure and could be more frequent and severe in patients with complex spines, 1040 in whom transforaminal approaches are frequently tried. The use of non-traumatic needles and ultrasound-guided parasagittal approaches 16 could help to reduce the frequency and severity of AEs. Moreover, the use of reservoirs and port devices for the intrathecal administration of nusinersen could improve the risk-benefit ratio but should be first evaluated in the setting of clinical trials.…”

Section: Discussionmentioning

confidence: 99%

“…Treated patients were injected with the 12 mg loading doses of nusinersen (at days 0, 14, 28 and 65) and maintenance doses every 4 months, as per label. Conventional and imaging-guided (including ultrasound, 16 fluoroscopy and CT) lumbar punctures were performed by experienced neurologists and neuroradiologists, respectively. All treated patients received at least four doses of nusinersen, except one patient, 16 who was discontinued after the second dose of nusinersen due to the lack of lumbar access and was excluded from efficacy analysis.…”

Section: Methodsmentioning

confidence: 99%

Nusinersen in adult patients with 5q spinal muscular atrophy: a multicenter observational cohorts’ study

Povedano

et al. 2021

Preprint

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Objective To assess safety and efficacy of nusinersen in adult 5q spinal muscular atrophy (SMA) patients. Methods Patients older than 15 years and followed at least for 6 months with one motor scale (Hammersmith Functional Motor Scale Expanded, HFMSE; Revised Upper Limb module, RULM) in five referral centers were included. Clinical and patients global impression of change (CGI-C and PGI-C) were recorded in treated patients at the last visit. Functional scales (Egen Klassification, EK2; Revised Amyotrophic Lateral Sclerosis Functional Rating Scale, ALSFRS-R) and the percent-predicted forced vital capacity were collected when available. Results Seventy-nine SMA patients (39 treated with nusinersen) were included. Compared with untreated patients, treated patients showed a significant improvement of 2 points (±0.46) in RULM (p<0.001) after six months. After a mean follow-up of 16 months, nusinersen treatment was associated with a significant improvement in HFMSE (OR=1.15, p=0.006), 6MWT (OR=1.07, p<0.001), and EK2 (OR=0.81, p=0.001). Compared with untreated patients, more treated patients experienced clinically meaningful improvements in all scales, but these differences were statistically significant only for RULM (p=0.033), ALSFRS-R (p=0.005), and EK2 (p<0.001). According to the CGI-C and PGI-C, 64.1% and 61.5% of treated patients improved with treatment. Being non-sitter was associated with less response to treatment, while longer time of treatment was associated with better response. Most treated patients (77%) presented at least one adverse event, mostly mild. Conclusions Nusinersen treatment associates to some improvements in adult SMA patients. Most severely affected patients with complex spines are probably those with the most unfavorable risk-benefit ratio.

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Ultrasound‐assisted and landmark‐based nusinersen delivery in spinal muscular atrophy adults: A retrospective analysis

Zanfini,

Patanella,

Vassalli

et al. 2024

Australas J Ultrason Med

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Introduction/PurposeNusinersen, the first treatment approved for all spinal muscular atrophy (SMA) types, is administered intrathecally through lumbar puncture. We used ultrasound assistance or a landmark‐based technique to access the lumbar intrathecal space in adult SMA patients. This study aimed to evaluate the technical success and adverse events (AEs) in such patients using either technique over a long observation period.MethodsFifty‐one adult patients received 507 consecutive interlaminar nusinersen administrations. Patients presented with both ‘uncomplicated spines’ or ‘complicated spines’; two patients had previous back surgery. Technical success and AEs were recorded using either technique. A generalised linear mixed model was applied to evaluate predictors of technical success and complications.ResultsAn overall success rate of 99.6%, with only two procedures failing to reach the intrathecal space, and an overall optimal procedure rate of 90.3% have been reported. A total of 455 procedures (89.7%) were uneventfully performed. One (0.2%) case of severe AE (puncture of a bulky abdominal annexal cyst) was recorded. Twenty‐seven episodes (5.3%) of post‐dural puncture headache (PDPH) and 24 episodes (4.7%) of radicular or back pain, both successfully treated with medical therapy, have also been reported. Technical success was significantly associated with ‘complicated spines’ (P = 0.022) and the use of ultrasound assistance (P = 0.01), and the use of ultrasound was the only independent predictor of uncomplicated procedures (P = 0.007).DiscussionIn adult patients with SMA both landmark‐based and ultrasound‐assisted techniques are safe and effective even in the long term. The use of assistance is associated with technical success and can predict uncomplicated procedures.ConclusionOur results support the use of ultrasonography in order to improve the success and reduce the burden of nusinersen intrathecal administration.

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

Cited by 23 publications

References 14 publications

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

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

Nusinersen in adult patients with 5q spinal muscular atrophy: a multicenter observational cohorts’ study

Ultrasound‐assisted and landmark‐based nusinersen delivery in spinal muscular atrophy adults: A retrospective analysis

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