Data from: Structural and functional brain connectome in motor neuron diseases: a multicenter MRI study

Objective. To investigate structural and functional neural organization in
amyotrophic lateral sclerosis (ALS), primary lateral sclerosis (PLS) and
progressive muscular atrophy (PMA) patients. Methods. 173 ALS, 38 PLS, 28
PMA sporadic patients and 79 healthy controls were recruited from three
Italian centers. Subjects underwent clinical, neuropsychological and brain
MRI evaluations. Using graph analysis and connectomics, global and lobar
topological network properties and regional structural and functional
brain connectivity were assessed. The association between structural and
functional network organization and clinical/cognitive data was
investigated. Results. Compared to healthy controls, ALS and PLS patients
showed altered structural global network properties, as well as local
topological alterations and decreased structural connectivity in
sensorimotor, basal ganglia, frontal and parietal areas. PMA patients
showed preserved global structure. Patient groups did not show significant
alterations of functional network topological properties relative to
controls. Increased local functional connectivity was observed in ALS
patients in the precentral, middle and superior frontal areas, and in PLS
patients in the sensorimotor, basal ganglia and temporal networks. In both
ALS and PLS patients, structural connectivity alterations correlated with
motor impairment, while functional connectivity disruption was closely
related to executive dysfunctions and behavioral disturbances.
Conclusions. This multicenter study showed widespread motor/extra-motor
network degeneration in ALS and PLS, suggesting that graph analysis and
connectomics might represent a powerful approach to detect upper motor
neuron degeneration, extra-motor brain changes and network reorganization
associated with the disease. Network-based advanced MRI provides an
objective in vivo assessment of motor neuron diseases, delivering
potential prognostic markers.