| Summary: | Neuropathological studies revealed the propagation of amyotrophic lateral sclerosis (ALS) in a sequence of four separate disease-related regional patterns. Diffusion tensor imaging (DTI)-based analysis was established for the individual mapping of sequential disease spreading in ALS as the in vivo transfer to neuroimaging. The aim of this review is to summarize cross-sectional and longitudinal results of these technical approaches in ALS as an in vivo tool to image ALS propagation stages. This concept was also applied to restricted phenotypes of ALS, e.g., lower motor neuron disease (LMND) or primary lateral sclerosis (PLS). In summary, the regional disease patterns in the course of ALS have been successfully mapped by DTI in vivo both cross-sectionally and longitudinally so that this technique might have the potential as a read-out in clinical trials.
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