Survival prediction models in motor neuron disease

• Published in: European journal of neurology Vol. 26; no. 9; pp. 1143 - 1152
• Main Authors: Agosta, F., Spinelli, E. G., Riva, N., Fontana, A., Basaia, S., Canu, E., Castelnovo, V., Falzone, Y., Carrera, P., Comi, G., Filippi, M.
• Format: Journal Article
• Language: English
• Published: England John Wiley & Sons, Inc 01.09.2019
• Subjects: Amyotrophic lateral sclerosis; atrophy; Brain; Brain damage; Cognitive ability; Cortex; Delay; Diagnostic systems; diffusion imaging; Magnetic resonance imaging; motor neuron disease; Motor neuron diseases; Motor neurone disease; Motor task performance; MRI; Neuroimaging; NMR; Nuclear magnetic resonance; Prediction models; Pyramidal tracts; Substantia alba; Substantia grisea; Survival; Tensors; atrophy; motor neuron disease; diffusion imaging; MRI; amyotrophic lateral sclerosis; survival
• ISSN: 1351-5101; 1468-1331
• DOI: 10.1111/ene.13957

Background and purpose This study aimed to assess the predictive value of multimodal brain magnetic resonance imaging (MRI) on survival in a large cohort of patients with motor neuron disease (MND), in combination with clinical and cognitive features. Methods Two hundred MND patients were followed up prospectively for a median of 4.13 years. At baseline, subjects underwent neurological examination, cognitive assessment and brain MRI. Grey matter volumes of cortical and subcortical structures and diffusion tensor MRI metrics of white matter tracts were obtained. A multivariable Royston–Parmar survival model was created using clinical and cognitive variables. The increase of survival prediction accuracy provided by MRI variables was assessed. Results The multivariable clinical model included predominant upper or lower motor neuron presentations and diagnostic delay as significant prognostic predictors, reaching an area under the receiver operating characteristic curve (AUC) of a 4‐year survival prediction of 0.79. The combined clinical and MRI model including selected grey matter fronto‐temporal volumes and diffusion tensor MRI metrics of the corticospinal and extra‐motor tracts reached an AUC of 0.89. Considering amyotrophic lateral sclerosis patients only, the clinical model including diagnostic delay and semantic fluency scores provided an AUC of 0.62, whereas the combined clinical and MRI model reached an AUC of 0.77. Conclusion Our study demonstrated that brain MRI measures of motor and extra‐motor structural damage, when combined with clinical and cognitive features, are useful predictors of survival in patients with MND, particularly when a diagnosis of amyotrophic lateral sclerosis is made.