Altered Fractional Anisotropy in Early Huntington's Disease

Abstract

Huntington's disease (HD) is a dominantly inherited neurodegenerative disease best known for chorea. The disorder includes numerous other clinical features including mood disorder, eye movement abnormalities, cognitive disturbance, pendular knee reflexes, motor impersistence, and postural instability. We describe a mild case of HD early in the disease course with depression and subtle neurological manifestations. In addition, we review MRI and diffusion tensor imaging features in this patient. The bicaudate ratio, a measure of caudate atrophy, was increased. Fractional anisotropy values of the bilateral caudate and putamen were increased, signifying neurodegeneration of these structures in HD.

Early whole-body mutant huntingtin lowering averts changes in proteins and lipids important for synapse function and white matter maintenance in the LacQ140 mouse model

“…Changes in white matter detected through imaging are one of the first signs of disease in people with HD (PwHD) (Rosas et al, 2006;Paulsen et al, 2008;Paulsen, 2009;Hobbs et al, 2010;Di Paola et al, 2012;Phillips et al, 2013;Singh et al, 2013;Matsui et al, 2015;Odish et al, 2015;Poudel et al, 2015;Faria et al, 2016;Bourbon-Teles et al, 2019;Tereshchenko et al, 2019;Sweidan et al, 2020;Oh et al, 2021). Morphometric studies of postmortem HD brain showed reduced cross-sectional area of white matter in addition to gray matter atrophy (de la Monte et al, 1988;Vonsattel and DiFiglia, 1998).…”

Early whole-body mutant huntingtin lowering averts changes in proteins and lipids important for synapse function and white matter maintenance in the LacQ140 mouse model

“…Changes in white matter detected through imaging are one of the first signs of disease in people with HD (PwHD) ( Bourbon-Teles et al, 2019 ; Di Paola et al, 2012 ; Faria et al, 2016 ; Hobbs et al, 2010 ; Matsui et al, 2015 ; Odish OF et al, 2015 ; Oh et al, 2021 ; Paulsen, 2009 ; Paulsen et al, 2008 ; Phillips et al, 2013 ; Poudel et al, 2015 ; Rosas et al, 2006 ; Singh et al, 2013 ; Sweidan et al, 2020 ; Tereshchenko et al, 2019 ). Morphometric studies of postmortem HD brains showed reduced cross-sectional area of white matter as well as gray matter atrophy ( de la Monte et al, 1988 ; Vonsattel and DiFiglia, 1998 ).…”

Fixel-Based Analysis Effectively Identifies White Matter Tract Degeneration in Huntington’s Disease

“…Changes in tensor-derived indices located in the right caudate nucleus in our study was in line with the reverse changes as reported by Gatto and Weissmann (2019) . This might be potentially attributed to the surrounding white matter loss which caused the appearance of comparatively more organized striatal gray matter ( Douaud et al, 2009 ; Singh et al, 2013 ). Studies on preclinical mouse models have indicated that cortical neuronal death is not proportionally accompanied by white matter tract degeneration ( Rattray et al, 2013 ), and early white matter changes are a main feature of neuronal and axonal connectivity impairment in fluorescent-tagged animals ( Gatto et al, 2015 ).…”