Regensburger Adrian P, Wagner Alexandra L, Danko Vera, Jüngert Jörg, Federle Anna, Klett Daniel, Schuessler Stephanie, Buehler Adrian, Neurath Markus F, Roos Andreas, Lochmüller Hanns, Woelfle Joachim, Trollmann Regina, Waldner Maximilian J, Knieling Ferdinand
Department of Pediatrics and Adolescent Medicine, Friedrich-Alexander-University (FAU) Erlangen-Nuremberg, Erlangen, Germany.
Medical Department 1, Friedrich-Alexander-University (FAU) Erlangen-Nuremberg, Erlangen, Germany.
Photoacoustics. 2021 Nov 10;25:100315. doi: 10.1016/j.pacs.2021.100315. eCollection 2022 Mar.
Proximal spinal muscular atrophy (SMA) is a rare progressive, life limiting genetic motor neuron disease. While promising causal therapies are available, meaningful prognostic biomarkers for therapeutic monitoring are missing. We demonstrate handheld Multispectral Optoacoustic Tomography (MSOT) as a novel non-invasive imaging approach to visualize and quantify muscle wasting in pediatric SMA. While MSOT signals were distributed homogeneously in muscles of healthy volunteers (HVs), SMA patients showed moth-eaten optoacoustic signal patterns. Further signal quantification revealed greatest differences between groups at the isosbestic point for hemoglobin (SWL 800 nm). The SWL 800 nm signal intensities further correlated with clinical phenotype tested by standard motor outcome measures. Therefore, handheld MSOT could enable non-invasive assessment of disease burden in SMA patients.
近端脊髓性肌萎缩症(SMA)是一种罕见的、进行性的、危及生命的遗传性运动神经元疾病。虽然有前景的因果治疗方法已经可用,但用于治疗监测的有意义的预后生物标志物却缺失。我们展示了手持式多光谱光声断层扫描(MSOT)作为一种新型非侵入性成像方法,用于可视化和量化小儿SMA中的肌肉萎缩。虽然MSOT信号在健康志愿者(HV)的肌肉中均匀分布,但SMA患者显示出蚕食状光声信号模式。进一步的信号量化显示,在血红蛋白的等吸收点(SWL 800 nm),两组之间的差异最大。SWL 800 nm的信号强度进一步与通过标准运动结果测量测试的临床表型相关。因此,手持式MSOT能够对SMA患者的疾病负担进行非侵入性评估。