The following is a summary of “Supra- and infra-tentorial degeneration patterns in primary lateral sclerosis: a multimodal longitudinal neuroradiology study,” published in the March 2024 issue of Neurology by Kleinerova et al.
Researchers started a retrospective study to investigate the under-reported presence of cerebellar pathology in primary lateral sclerosis (PLS), a disease primarily known for upper motor neuron dysfunction.
They conducted a longitudinal neuroimaging study (multi-timepoint) to understand the evolution of intra-cerebellar disease burden and cerebro-cerebellar connectivity. Deep cerebellar nuclei volumes, cerebellar cortical volumes, and cerebro-cerebellar structural and functional connectivity were evaluated over time in 43 individuals with PLS.
The results showed that at baseline, structural disconnection occurred in cerebello-frontal, -temporal, -parietal, -occipital, and cerebello-thalamic pathways, as indicated by radial diffusivity (RD). Additionally, cerebello-frontal decoupling was evident based on fractional anisotropy (FA) alterations. Changes in functional connectivity were observed in cerebello-frontal, parietal, and occipital projections. Volume reductions were detected in the vermis, anterior, posterior, and crura. Among the deep cerebellar nuclei, atrophy was noted in the dorsal dentate. Longitudinal follow-up did not reveal statistically significant progressive changes. Significant atrophy in the primary motor cortex and inter-hemispheric transcallosal degeneration were also observed.
Investigators concluded that PLS extends beyond upper motor neuron dysfunction, encompassing cerebellar and connectivity pathology, potentially contributing to broader motor and bulbar dysfunction.
Source: link.springer.com/article/10.1007/s00415-024-12261-z