Magnetic Resonance Imaging to Detect Structural Brain Changes in Huntington's Disease: A Review of Data from Mouse Models

Abstract

Structural magnetic resonance imaging (MRI) is a powerful tool to visualize 3D neuroanatomy and assess pathology and disease progression in neurodegenerative disorders such as Huntington's disease (HD). The development of mouse models of HD that reproduce many of the psychiatric, motor and cognitive impairments observed in human HD has improved our understanding of the disease and provided opportunities for testing novel therapies. Similar to the clinical scenario, MRI of mouse models of HD demonstrates onset and progression of brain pathology. Here, we provided an overview of the articles that used structural MRI in mouse models of HD to date, highlighting the differences between studies and models and describing gaps in the current state of knowledge and recommendations for future studies.

Keywords: Brain structure; Huntington’s disease; magnetic resonance imaging; mouse models; neuroanatomy.

Fig. 1

Percent changes in brain regions compared to controls across studies in all mouse models, and in only the R6/2 mouse model. Only data from articles that provided percent differences or the absolute structure volumes was included.

Fig. 2

Striatum volume loss as measured by structural MRI with CAG repeat length in 6 different mouse models. The CAG repeat lengths were determined at the time of genotyping by PCR. Only data from articles that provided percent differences or the absolute structure volumes was included. For mouse models with multiple imaging time points, only data post-symptom onset was included. The average time between motor and cognitive symptom onset and MR imaging was 14 weeks.