Comprehensive longitudinal imaging analysis in primary lateral sclerosis

A single-center, prospective, longitudinal neuroimaging study to determine the association between primary lateral sclerosis (PLS) and progressive primary motor cortex degeneration.
Analysis was performed using standardized neuroimaging, genetic profiling, and clinical assessments including upper motor neuron scores, tapping rates, mirror movements, spasticity assessment, cognitive screening and evaluation for pseudobulbar affect.
Results confirmed the association between PLS and progressive primary motor cortex degeneration, substantial supplementary- and pre-motor area pathology, brainstem atrophy that is progressive, cortico-medullary and inter-hemispheric disconnection, and links between clinical upper motor neuron scores and somatotopic connectivity indices. Authors confirmed that the clinical assessments examined here could be useful tools in assessing disease burden non-invasively.