Striatal morphology and neurocognitive dysfunction in Huntington disease: The IMAGE-HD study

Rate of torque development and striatal shape in individuals with prodromal Huntington's disease

“…Consistent with previous studies, significant differences in striatal shape metrics were observed between individuals with pro-HD and HC 26 , 27 . In particular, individuals with pro-HD displayed significant shape deflation in comparison with HC that was more evident for the right hemispheres of the putamen and caudate than for the left.…”

“…Together, these findings highlight the inherent variability in motor features across individuals with pro-HD, which has been well-documented in previous epidemiological studies and is presumed to relate to differences in striatal pathology, genetic burden, lifestyle and genetic modifiers, which could be the subject of future investigations. Consistent with previous studies, significant differences in striatal shape metrics were observed between individuals with pro-HD and HC 26,27 . In particular, individuals with pro-HD displayed significant shape deflation in comparison with HC that was more evident for the right hemispheres of the putamen and caudate than for the left.…”

Reduced Fractalkine Levels Lead to Striatal Synaptic Plasticity Deficits in Huntington’s Disease

“…Neuropathology of HD is prominent in the striatum, the main primary input of basal ganglia (Morigaki and Goto, 2017) and the cerebral cortex, which extensively projects glutamatergic afferents to medium spiny neurons (MSNs) in the striatum. HD disrupts the mechanisms by which cortical and MSNs communicate before the onset of symptoms (Unschuld et al, 2012;Burgold et al, 2019), making corticostriatal pathway dysfunction a keystone in the pathophysiology of the disease (Miller et al, 2011;Wilkes et al, 2019). Some data from HD mouse models suggest that deficits in synaptic plasticity and neuronal processing are the cellular basis for this specific neurodegeneration.…”

A biological classification of Huntington's disease: the Integrated Staging System

“…[13][14][15][16][17][18] For example, in the last two decades there have been foundational observational studies that contribute decisively to what is currently known about HD before clinical motor diagnosis -TRACK-HD, Track On-HD, PREDICT-HD, IMAGE-HD, Registry, Enroll-HD, and more recently HD-YAS. [13][14][15][16][17][18][19][20][21] However, these studies each established distinct methods, including differently-adjusted products of age and CAG, 4 to select and categorize participant disease progression. The lack of standardization makes it difficult to describe and compare populations across studies.…”