Alessandro Introna (Italy)
University of Bari Department of Basic Medical Sciences, Neurosciences and Sense OrgansAuthor Of 2 Presentations
KING’S PROGRESSION RATE: PROGNOSTIC VALUE IN AMYOTROPHIC LATERAL SCLEROSIS ACCORDING TO CEREBROSPINAL NEUROFILAMENT LIGHT CHAIN LEVELS
- Alessandro Introna (Italy)
Abstract
Background and Aims:
Neurofilament light chain (NFL) has been proposed as a reliable and validated prognostic biomarker for amyotrophic lateral sclerosis (ALS). Usually, fast and slow progressors (FP and SP respectively) of disease were identified according to the slopes of the revised ALS functional rating scale. We aim to evaluate the prognostic value of King’s stage progression rate (KPR) through cerebrospinal fluid (CSF) neurofilament light chain (NFL) assessment.
Methods:
The KPR was calculated as the following formula: (0 – King’s clinical stage at first visit)/disease duration from onset to first visit. CSF NFL levels were measured at the time of diagnosis in 58 ALS patients. The whole cohort was divided in FP and SP according to the median value of KPR. Linear regression analysis was performed to evaluate how changes in NFL levels were reflected in KPR modifications.
Results:
Clinical and demographic characteristics were summarized in Figure 1. The NFL levels differed in the two groups (p=0.014). High concentrations of CSF NFL significantly correlated with more negative KPR (rs = -0.357; p < 0.0003). Multivariate linear regression analysis (adjusted for sex and age) showed that every increase of 1000 ng/l in NFL was associated with a reduction in KPR of 0.02 points/month (p= 0.012).
Conclusions:
This study suggests that KPR could be a valid prognostic marker of disease mirroring levels of NFL, an ascertained reliable biomarker of ALS progression. Indeed, higher NFL levels were associated with more negative KPR reflecting a wider extension of the disease process.
MEDULLA OBLONGATA VOLUME PREDICTS SURVIVAL IN AMYOTROPHIC LATERAL SCLEROSIS PATIENTS
- Giammarco Milella (Italy)
Abstract
Background and Aims:
The impaired bulbar function represents the main source of disability in patients affected by amyotrophic lateral sclerosis (ALS). We investigated whether differences in medulla oblongata volumes may predict survival in an ALS cohort.
Methods:
Demographic and clinical data were recorded in 80 ALS patients from diagnosis to tracheostomy/death or censoring-date. At the time of diagnosis, patients underwent a 3D-T1 MRI. Medulla Oblongata (MO) volume was calculated by Freesurfer and FSL. The whole cohort was divided into lower and higher MO volume groups (L-MOV and H-MOV, respectively) according to the best value of MO volume by ROC curve able to discriminate short from long survivors, using median survival time as a cut-off. Univariate and multivariate survival analyses were performed using as covariates the L-MOV and H-MOV groups, gender, age, and site of onset.
Results:
The L-MOV group was characterized prevalently by patients with older age of onset, female gender, and a higher progression rate (Figure). The survival was significantly reduced in the L-MOV group (log-rank, p<0.0001). In multivariate analysis, L-MOV was the only variable significantly associated with survival [HR: 3.71; 95%CI: 2-6.9;p<0.001].
Conclusions:
Our findings show that the medulla oblongata atrophy itself represents the critical “epicenter” of the poor clinical outcome of ALS patients regardless of the site of onset. Therefore, this measure may be considered an attractive candidate biomarker predictive of survival in ALS.
Presenter of 1 Presentation
KING’S PROGRESSION RATE: PROGNOSTIC VALUE IN AMYOTROPHIC LATERAL SCLEROSIS ACCORDING TO CEREBROSPINAL NEUROFILAMENT LIGHT CHAIN LEVELS
- Alessandro Introna (Italy)
Abstract
Background and Aims:
Neurofilament light chain (NFL) has been proposed as a reliable and validated prognostic biomarker for amyotrophic lateral sclerosis (ALS). Usually, fast and slow progressors (FP and SP respectively) of disease were identified according to the slopes of the revised ALS functional rating scale. We aim to evaluate the prognostic value of King’s stage progression rate (KPR) through cerebrospinal fluid (CSF) neurofilament light chain (NFL) assessment.
Methods:
The KPR was calculated as the following formula: (0 – King’s clinical stage at first visit)/disease duration from onset to first visit. CSF NFL levels were measured at the time of diagnosis in 58 ALS patients. The whole cohort was divided in FP and SP according to the median value of KPR. Linear regression analysis was performed to evaluate how changes in NFL levels were reflected in KPR modifications.
Results:
Clinical and demographic characteristics were summarized in Figure 1. The NFL levels differed in the two groups (p=0.014). High concentrations of CSF NFL significantly correlated with more negative KPR (rs = -0.357; p < 0.0003). Multivariate linear regression analysis (adjusted for sex and age) showed that every increase of 1000 ng/l in NFL was associated with a reduction in KPR of 0.02 points/month (p= 0.012).
Conclusions:
This study suggests that KPR could be a valid prognostic marker of disease mirroring levels of NFL, an ascertained reliable biomarker of ALS progression. Indeed, higher NFL levels were associated with more negative KPR reflecting a wider extension of the disease process.