Background and purpose: The EFNS guidelines on neuroimaging of motor neuron diseases (MNDs) are designed to provide practical help for neurologists to make appropriate use of neuroimaging techniques in patients with MNDs, which ranges from diagnostic and monitoring aspects to the in vivo study of the pathobiology of such conditions.

Methods: Literature searches were performed before expert members of the Task Force wrote proposals. Then, consensus was reached by circulating drafts of the manuscript to the Task Force members and by discussion of the classification of evidence and recommendations.

Results and conclusions: The use of conventional magnetic resonance imaging (MRI) in patients suspected of having a MND is restricted to exclude other causes of signs and symptoms of MN pathology (class IV, good clinical practice). Although the detection of corticospinal tract hyperintensities on conventional MRI and a T2-hypointense rim in the precentral gyrus can support a pre-existing suspicion of MND, the specific search of these abnormalities for the purpose of making a firm diagnosis of MND is not recommended (class IV, good clinical practice). At present, advanced neuroimaging techniques, including diffusion tensor imaging and proton magnetic resonance spectroscopic imaging, do not have a role in the diagnosis or routine monitoring of MNDs (class IV, good clinical practice). However, it is strongly advisable to incorporate measures derived from these techniques into new clinical trials as exploratory outcomes to gain additional insights into disease pathophysiology and into the value of these techniques in the (longitudinal) assessment of MNDs (class IV, good clinical practice).