No abstract

A BS TRACT: Background: Sporadic adult-onset ataxias without known genetic or acquired cause are subdivided into multiple system atrophy of cerebellar type (MSA-C) and sporadic adult-onset ataxia of unknown etiology (SAOA). Objectives: To study the differential evolution of both conditions including plasma neurofilament light chain (NfL) levels and magnetic resonance imaging (MRI) markers. Methods: SPORTAX is a prospective registry of sporadic ataxia patients with an onset >40 years. Scale for the Assessment and Rating of Ataxia was the primary outcome measure. In subgroups, blood samples were taken and MRIs performed. Plasma NfL was measured via a single molecule assay. Regional brain volumes were automatically measured. To assess signal changes, we defined the pons and middle cerebellar peduncle abnormality score (PMAS). Using mixed-effects models, we analyzed changes on a time scale starting with ataxia onset. Results: Of 404 patients without genetic diagnosis, 130 met criteria of probable MSA-C at baseline and 26 during follow-up suggesting clinical conversion to MSA-C. The remaining 248 were classified as SAOA. At

Vertebral Modic type 1 (MT1) degeneration may mimic infectious disease on conventional spine magnetic resonance imaging (MRI), potentially leading to additional costly and invasive investigations. This study evaluated the diagnostic performance of the proton density fat fraction (PDFF) for distinguishing MT1 degenerative endplate changes from infectious spondylitis. A total of 31 and 22 patients with equivocal diagnosis of MT1 degeneration and infectious spondylitis, respectively, were retrospectively enrolled in this IRB-approved retrospective study and examined with a chemical-shift encoding (CSE)-based water-fat 3D six-echo modified Dixon sequence in addition to routine clinical spine MRI. Diagnostic reference standard was established according to histopathology or clinical and imaging follow-up. Intravertebral PDFF [%] and PDFFratio (i.e., vertebral endplate PDFF/normal vertebrae PDFF) were calculated voxel-wise within the single most prominent edematous bone marrow lesion per patient and examined for differences between MT1 degeneration and infectious spondylitis. Mean PDFF and PDFFratio of infectious spondylitis were significantly lower compared to MT1 degenerative changes (mean PDFF, 4.28 ± 3.12% vs. 35.29 ± 17.15% [p < 0.001]; PDFFratio, 0.09 ± 0.06 vs. 0.67 ± 0.37 [p < 0.001]). The areas under the curve (AUC) and diagnostic accuracies were 0.977 (p < 0.001) and 98.1% (cut-off at 12.9%) for PDFF and 0.971 (p < 0.001) and 98.1% (cut-off at 0.27) for PDFFratio. Our data suggest that quantitative evaluation of vertebral PDFF can provide a high diagnostic accuracy for differentiating erosive MT1 endplate changes from infectious spondylitis.

Patients with BM are in advanced stages of systemic cancer, which may translate into significant alterations of body composition biomarkers, such as BMD. The present study investigated the prognostic value of BMD on overall survival (OS) of 95 patients with surgically-treated BM related to NSCLC. All patients were treated in a large tertiary care neuro-oncological center between 2013 and 2018. Preoperative BMD was determined from the first lumbar vertebrae (L1) from routine preoperative staging computed tomography (CT) scans. Results were stratified into pathologic and physiologic values according to recently published normative reference ranges and correlated with survival parameters. Median preoperative L1-BMD was 99 Hounsfield units (HU) (IQR 74–195) compared to 140 HU (IQR 113–159) for patients with pathological and physiologic BMD (p = 0.03), with a median OS of 6 versus 15 months (p = 0.002). Multivariable analysis revealed pathologic BMD as an independent prognostic predictor for increased 1-year mortality (p = 0.03, OR 0.5, 95% CI 0.2–1.0). The present study suggests that decreased preoperative BMD values may represent a previously unrecognized negative prognostic factor in patients of BM requiring surgery for NSCLC. Based on guideline-adherent preoperative staging, BMD may prove to be a highly individualized, readily available biomarker for prognostic assessment and treatment guidance in affected patients.