Abstract
Primary lateral sclerosis and amyotrophic lateral sclerosis are primarily associated with motor cortex and corticospinal tract pathology. A standardised, prospective, single-centre neuroimaging protocol was used to characterise thalamic, hippocampal and basal ganglia involvement in 33 patients with primary lateral sclerosis (PLS), 100 patients with amyotrophic lateral sclerosis (ALS), and 117 healthy controls. "Widespread subcortical grey matter degeneration in primary lateral sclerosis: a multimodal imaging study with genetic profiling" [1] Imaging data were acquired on a 3 T MRI system using a 3D Inversion Recovery prepared Spoiled Gradient Recalled echo sequence. Model based segmentation was used to estimate the volumes of the thalamus, hippocampus, amygdala, caudate, pallidum, putamen and accumbens nucleus in each hemisphere. The hippocampus was further parcellated into cytologically-defined subfields. Total intracranial volume (TIV) was estimated for each participant to aid the interpretation of subcortical volume alterations. Group comparisons were corrected for age, gender, TIV, education and symptom duration. Considerable thalamic, hippocampal and accumbens nucleus atrophy was detected in PLS compared to healthy controls and selective dentate, molecular layer, CA1, CA3, and CA4 hippocampal pathology was also identified. In ALS, additional volume reductions were noted in the amygdala, left caudate and the hippocampal-amygdala transition area of the hippocampus. Our imaging data provide evidence of extensive and phenotype-specific patterns of subcortical degeneration in PLS.