Abstract

Intrathecal administration of nusinersen in adult spinal muscular atrophy (SMA) patients with scoliosis and spondylodesis requires image guidance, which is preferably achieved with multi-detector computed tomography (MDCT). As long-term treatment is necessary and patients are young, radiation doses should be reduced to a minimum whilst a sufficient image quality for precise interventional performance should be kept. We compared 44 MDCT standard-dose scans (133.0–200.0 mA) with a hybrid iterative reconstruction (iDose4) to 20 low-dose scans (20.0–67.0 mA) with iterative model reconstruction (IMR), which were performed for procedure planning of intrathecal nusinersen administration in 13 adult patients with SMA and complex spinal conditions. Qualitative image evaluation, including confidence for intervention planning, was performed by two neuroradiologists for standard- and low-dose scans. All 64 MDCT-guided intrathecal administrations of nusinersen were successful. The dose length product (DLP) was significantly lower when using low-dose scanning with IMR (median DLP of standard-dose scans: 92.0 mGy•cm vs. low-dose scans: 34.5 mGy•cm; p < 0.0001). Image quality was significantly reduced for low-dose compared to standard-dose scanning. However, bone/soft tissue contrast and confidence for intervention planning were not significantly impaired in low-dose MDCT according to both readers, showing good inter-reader agreement. Thus, we hereby demonstrate a low-dose MDCT protocol combined with advanced image reconstruction for scanning during procedure planning as a viable option for image guidance in intrathecal nusinersen treatment of adult SMA patients with complex spinal conditions.

Highlights

  • Spinal muscular atrophy (SMA) is an autosomal-recessive neurodegenerative disease caused by mutations in the survival motor neuron 1 (SMN1) gene[1]

  • Median radiation dose indicated as the dose length product (DLP) in spinal muscular atrophy (SMA) patients with computed tomography (CT)-assisted procedures was previously reported to be considerably high (120.1 mGycm), with this value being even increased in patients with spinal fusion (246.5 mGycm)[6]

  • Out of the 64 CT-guided injections considered in this study, 44 interventions were performed before the update of the CT imaging protocols, using standard-dose imaging protocols with iDose[4] for reconstruction (Fig. 1)

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Summary

Introduction

Spinal muscular atrophy (SMA) is an autosomal-recessive neurodegenerative disease caused by mutations in the survival motor neuron 1 (SMN1) gene[1]. Intrathecal drug administration presents challenges in adult SMA patients due to severe neuromuscular scoliosis and frequent preceding spinal fusion surgeries. The tube current should only be reduced to a level at which image quality still guarantees an accurate and reliable treatment, in patients with demanding anatomies or implants at the level of the spine. Against this background, the present study evaluates low-dose multi-detector computed tomography (MDCT) for procedure planning of intrathecal nusinersen administration in a consecutive series of patients with SMA and complex spinal conditions

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