Brain structural and perfusion changes in amyotrophic lateral sclerosis-frontotemporal dementia patients with cognitive and motor onset: a preliminary study.

Amyotrophic lateral sclerosis-frontotemporal dementia (ALS-FTD) is rare but exhibits worse prognosis than either ALS or FTD alone. However, cognitive onset ALS-FTD (ALS-FTD-C) confers significantly better patient survival than does motor onset ALS-FTD (ALS-FTD-M), underscoring a meager understanding of pathological group differences. This study aimed to assess disparities in cortical atrophy and perfusion shown by patients with the above disease variants. A total of 38 participants (ALS-FTD-C, 8; ALS-FTD-M, 6; simultaneous-onset ALS-FTD [ALS-FTD-S], 4; healthy controls [HC], 20) qualified for the study and underwent magnetic resonance imaging scan. Three-dimensional T1-weighted structural brain imaging and 3D-pseudocontinuous arterial spin-labeled imaging were routinely collected. Gray matter volume (GMV) and cerebral blood flow (CBF) in ALS-FTD-C and ALS-FTD-M were compared through voxel-based analysis. Correlations between imaging parameters and clinical data were also assessed. Compared with HC, ALS-FTD had significant GMV reduction mainly in bilateral limbic system. GMV reduction in ALS-FTD-C was similar in pattern but less widespread, whereas ALS-FTD-M lacked any significant GMV reduction. In CBF analyses, ALS-FTD displayed hypoperfusion in bilateral motor cortex, frontotemporal lobe, and left basal ganglia. Hypoperfusion involved bilateral temporal lobe, prefrontal cortex, and putamen in ALS-FTD-C but was limited to left parahippocampal gyrus in ALS-FTD-M. Correlations between clinical data and GMV/CBF changes in specific regions were also identified in ALS-FTD. Group-specific patterns of cortical atrophy and perfusion were evident in ALS-FTD-C and ALS-FTD-M. ALS-FTD-C showed pronounced cortical atrophy and hypoperfusion, which were otherwise minimal in ALS-FTD-M. Above findings preliminarily revealed the pathological group differences that may help in classifying patients with ALS-FTD.