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

2020 ◽  
Author(s):  
D. Veiga‐Canuto ◽  
M. Cifrián‐Pérez ◽  
I. Pitarch‐Castellano ◽  
J.F. Vázquez‐Costa ◽  
F. Aparici
Keyword(s):  
Spinal Muscular Atrophy ◽  
Lumbar Puncture ◽  
Muscular Atrophy ◽  
Ultrasound Guided

scholarly journals Ultrasound-guided Nusinersen Administration for Spinal Muscular Atrophy Patients With Severe Scoliosis: a Retrospective Single-arm Cohort Study

2021 ◽  
Author(s):  
Jiao Zhang ◽  
Xulei Cui ◽  
Si Chen ◽  
Yi Dai ◽  
Yuguang Huang ◽  
...  
Keyword(s):  
Spinal Muscular Atrophy ◽  
Real Time ◽  
Lumbar Puncture ◽  
Ultrasound Guidance ◽  
Muscular Atrophy ◽  
Csf Flow ◽  
Ultrasound Guided ◽  
Severe Scoliosis ◽  
Type 3

Abstract BackgroundThis observational study describes our experience delivering nusinersen through lumbar puncture with real-time ultrasound guidance in spinal muscular atrophy (SMA) patients with severe scoliosis.ResultsIntrathecal nusinersen via real-time ultrasound-guided lumbar puncture was given to 3 patients who had severe thoracic and lumbar scoliosis: a 34-year-old female with type 3 SMA, a 28-year-old male with type 2 SMA, and a 14-year-old girl with type 3 SMA. Lumbar puncture was performed without sedation under ultrasound guidance using a 22G echogenic needle in the interlaminar aspect of the L4-L5 or L5-S1 interspace and a full dose of nusinersen (12 mg/5 mL) was injected after visualizing free cerebrospinal fluid (CSF) flow. Patients completed their 4 loading doses and 1 maintenance dose of nusinersen. All 15 procedures were successful and well tolerated.ConclusionsReal-time ultrasound-guided lumbar puncture is an effective and radiation-free technique to administer intrathecal nusinersen in SMA patients with severe scoliosis when done by practitioners with expertise in this procedure.

scholarly journals Ultrasound-guided nusinersen administration for spinal muscular atrophy patients with severe scoliosis: an observational study

2021 ◽  
Vol 16 (1) ◽  
Author(s):  
Jiao Zhang ◽  
Xulei Cui ◽  
Si Chen ◽  
Yi Dai ◽  
Yuguang Huang ◽  
...  
Keyword(s):  
Cerebrospinal Fluid ◽  
Observational Study ◽  
Spinal Muscular Atrophy ◽  
Real Time ◽  
Lumbar Puncture ◽  
Ultrasound Guidance ◽  
Muscular Atrophy ◽  
Ultrasound Guided ◽  
Cerebrospinal Fluid Flow ◽  
Severe Scoliosis

Abstract Background This observational study describes our experience delivering nusinersen through lumbar puncture with real-time ultrasound guidance in spinal muscular atrophy (SMA) patients with severe scoliosis. Results Intrathecal nusinersen via real-time ultrasound-guided lumbar puncture was given to three patients who had severe thoracic and lumbar scoliosis: a 34-year-old female with type 3a SMA, a 28-year-old male with type 2a SMA, and a 14-year-old girl with type 3a SMA. Lumbar puncture was performed without sedation under ultrasound guidance using a 22G echogenic needle in the interlaminar aspect of the L4–L5 or L5–S1 interspace and a full dose of nusinersen (12 mg/5 mL) was injected after visualizing free cerebrospinal fluid flow. Patients completed their four loading doses and one maintenance dose of nusinersen. All 15 procedures were successful and well tolerated. Conclusions Real-time ultrasound-guided lumbar puncture is an effective and radiation-free technique to administer intrathecal nusinersen in SMA patients with severe scoliosis when done by practitioners with expertise in this procedure.

Lumbar Puncture Opening Pressure in Patients with Spinal Muscular Atrophy

2021 ◽  
Author(s):  
Lena-Luise Becker ◽  
Claudia Weiß ◽  
Anna Tietze ◽  
Viktoria Martiny ◽  
Angela M. Kaindl
Keyword(s):  
Intracranial Pressure ◽  
Spinal Muscular Atrophy ◽  
Lumbar Puncture ◽  
Clinical Data ◽  
Side Effect ◽  
Treatment Initiation ◽  
Muscular Atrophy ◽  
Underlying Disease ◽  
Increased Intracranial Pressure ◽  
Opening Pressure

AbstractThe announcement of a hydrocephalus as a possible side effect in patients with spinal muscular atrophy (SMA) receiving the drug nusinersen, promoted major concern and warrants further evaluation. In this retrospective monocentric study, we analyzed clinical data, lumbar puncture opening pressure (LOP) measurement, and ophthalmologic and neuroimaging results in 34 patients with SMA types 1 to 3 undergoing treatment with nusinersen. None of the patients reported symptoms indicative of increased intracranial pressure. In our cohort, the LOP was >20 cm H2O in 25 patients (70.5%), and within this group ≥28 cm H2O in 12 patients (35.3%), in two patients, it was increased prior to treatment initiation. Signs of increased intracranial pressure in ophthalmological assessments or brain imaging were only seen in one patient. We did not identify a correlation between increased LOP and SMA type, scoliosis, or age of the patients; however, it was slightly higher in patients receiving sedation. Our results raise the question whether the LOP is generally increased in SMA as part of the underlying disease, if so, what the etiology is, and whether the increased LOP needs to be treated.

Computed tomography-guided transforaminal lumbar puncture using local anesthesia and a straight 22-gauge spinal needle for intrathecal nusinersen in adults: Findings in 77 procedures

2021 ◽  
pp. 159101992110328
Author(s):  
Mougnyan Cox ◽  
Kofi-Buaku Atsina ◽  
Preethi Ramchand ◽  
Jonathan Ji ◽  
Neda Sedora-Roman ◽  
...  
Keyword(s):  
Computed Tomography ◽  
Spinal Muscular Atrophy ◽  
Lumbar Puncture ◽  
Local Anesthesia ◽  
Muscular Atrophy ◽  
Intrathecal Administration ◽  
Spinal Needle ◽  
Technical Success ◽  
Computed Tomography Guidance ◽  
Blood Patch

Background Intrathecal nusinersen is the first Food and Drug Administration-approved treatment for spinal muscular atrophy. Reliable intrathecal access is critical for initial and maintenance therapy; however, this can be challenging in older patients with spinal muscular atrophy many of whom have had prior lumbar instrumentation and osseous fusion. Transforaminal lumbar punctures have emerged as a technique for intrathecal access that avoids the hazards of cervical punctures. We describe our technique for transforaminal lumbar punctures under computed tomography guidance using local anesthesia and a straight 22-gauge needle. Methods Following local institutional review board approval, medical records of all patients undergoing computed tomography-guided transforaminal lumbar puncture for intrathecal nusinersen injection were obtained and analyzed. The rate of technical success and immediate complications were recorded. Any delayed complications noted in a 3-day follow-up phone call and future office visit were also recorded. Data collation and analysis were performed using Excel. Results A total of 77 transforaminal lumbar punctures were performed with intrathecal administration of nusinersen, for a 100% technical success rate. Local anesthesia was used in 76 cases, with conscious sedation used in one case. General anesthesia was not used in any case. There were no major complications. One patient had a postdural puncture headache that resolved completely after a transforaminal epidural blood patch performed 4 days later. Conclusions Intrathecal administration of nusinersen is critical for treatment of patients with spinal muscular atrophy. Our described technique allows for reliable access to the intrathecal space using local anesthesia and a straight 22-gauge spinal needle under computed tomography guidance, and is easily reproducible.

scholarly journals Ultra-low radiation dose protocol for CT-guided intrathecal nusinersen injections for patients with spinal muscular atrophy and severe scoliosis

2021 ◽  
Author(s):  
Grzegorz Rosiak ◽  
Anna Lusakowska ◽  
Krzysztof Milczarek ◽  
Dariusz Konecki ◽  
Anna Fraczek ◽  
...  
Keyword(s):  
Radiation Dose ◽  
Spinal Muscular Atrophy ◽  
Lumbar Puncture ◽  
Low Dose ◽  
Muscular Atrophy ◽  
Radiation Doses ◽  
Standard Protocol ◽  
Ct Guided ◽  
Low Radiation Dose ◽  
Severe Scoliosis

Abstract Purpose Intrathecal injection of nusinersen is an approved treatment of spinal muscular atrophy (SMA). CT-guided injection is a method of nusinersen administration in patients with severe scoliosis, in whom standard lumbar puncture is not feasible. The injections are repeated every 4 months for life, and accumulated radiation doses absorbed by the patient can increase the risk of cancer. In this study, we present the results of CT-guided intrathecal nusinersen injections with an ultra-low radiation dose protocol. Methods Eighteen patients (15 adults and three children) in whom standard lumbar puncture was not feasible due to severe scoliosis or spinal stabilization were included in this retrospective study. The first 23 injections were performed with a standard radiation dose protocol and the next 42 injections with an ultra-low-dose protocol. The radiation doses, measured as total dose length product (DLP), were acquired and compared between the protocols. Results Injections were successful in 100% of patients with both ultra-low-dose and standard protocols. The radiation dose, measured as DLP, was 111.2–1100.7 (Me = 248.1) mGy*cm for the standard protocol. For the ultra-low-dose protocol, the dose range was 5.0–54.4 (Me = 26.7) mGy*cm, which was significantly lower than with the standard protocol (p < 0.001, η2 = 0.67). Conclusion Radiation doses can be significantly decreased in the CT-guided injection of nusinersen. The proposed protocol allows for effective CT-guided intrathecal nusinersen administration in patients with SMA and severe scoliosis.

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