Abstract
BackgroundAmyotrophic lateral sclerosis (ALS) a highly heterogeneous neurodegenerative condition. Accurate diagnostic, monitoring and prognostic biomarkers are urgently needed both for individualised patient care and clinical trials. A multimodal magnetic resonance imaging study is presented, where MRI measures of ALS-associated brain regions are utilised to predict 18-month survival.MethodsA total of 60 ALS patients and 69 healthy controls were included in this study. 20% of the patient sample was utilised as an independent validation sample. Surface-based morphometry and diffusion tensor white matter parameters were used to identify anatomical patterns of neurodegeneration in 80% of the patient sample compared to healthy controls. Binary logistic ridge regressions were carried out to predict 18-month survival based on clinical measures alone, MRI features, and a combination of clinical and MRI data. Clinical indices included age at symptoms onset, site of disease onset, diagnostic delay from first symptom to diagnosis, and physical disability (ALSFRS-r). MRI features included the average cortical thickness of the precentral and paracentral gyri, the average fractional anisotropy, radial-, medial-, and axial diffusivity of the superior and inferior corona radiata, internal capsule, cerebral peduncles and the genu, body and splenium of the corpus callosum.ResultsClinical data alone had a survival prediction accuracy of 66.67%, with 62.50% sensitivity and 70.84% specificity. MRI data alone resulted in a prediction accuracy of 77.08%, with 79.16% sensitivity and 75% specificity. The combination of clinical and MRI measures led to a survival prediction accuracy of 79.17%, with 75% sensitivity and 83.34% specificity.ConclusionQuantitative MRI measures of ALS-specific brain regions enhance survival prediction in ALS and should be incorporated in future clinical trial designs.
Highlights
Amyotrophic lateral sclerosis (ALS) a highly heterogeneous neurodegenerative condition
The direct comparison of patients surviving more than 18 months and those surviving less than 18 months, did not reach statistical significance with family-wise error corrections
The combination of objective Magnetic resonance imaging (MRI) measures and key clinical indices enable the accurate prediction of 18month survival in ALS
Summary
Amyotrophic lateral sclerosis (ALS) a highly heterogeneous neurodegenerative condition. It is widely accepted that a Clinical heterogeneity has multiple dimensions in ALS such as site of onset, coexisting cognitive and behavioural deficits, dominance of upper or lower motor neurodegeneration, variability in progression rates and the relatively distinct clinical profile of various ALS genotypes [3, 4]. All of these variables make accurate individual prognostication challenging. The planning and timing of supportive interventions such as feeding tube insertion, initiation of non-invasive ventilation, addressing end-of-life decisions and palliative measures could be guided by accurate prognostic markers
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