Objective: To analyze the relationship between motor scales and brain magnetic resonance imaging (MRI) features in a cohort of Huntington disease (HD) patients.

Background: Cerebellar symptoms, as other motor features, largely affects HD patients; nonetheless, their clinical significance is scarcely studied. Here, we evaluated the relationship between cerebellar and other motor scales (UHDRS, UPDRS, SARA) and volumetric brain measurements in patients with HD.

Method: Cross-sectional analysis of sociodemographic and clinical data of 38 patients and MRI features of a subset of 22 patients from a Movements Disorders Clinic in Mexico. Also, brain volumes were compared with a control group (n=27).

Results: The mean age of patients was 47.0±14.7y; 55.3% were men.[table1] Comparing MRI volumetric findings [table2], significant differences between groups were observed, except for the thalamus, right, and total cerebellum volumes (p>0.05). We observed that patients have lower brain volumes than the control group, except for cerebellum that was higher in HD. For the total UHDRS score, there is a significant association with the total caudate and cerebellum volumes. This was reflected in an average increase of 17.52 points of the total UHDRS score for each cm³ that the caudate volumes decreases; and an average increase of 1.41 points of the total UHDRS score for each cm³ that the total cerebellum volume decrease, even when other variables such as alleles length, duration of the disease, genre, and the total globus pallidus and putamen volumes were considered [table3]. Analyzing other adjusted models shown significant associations between the motor score of the UPDRS scale and the total volume of the putamen; total SARA score and total cerebellar volume; the whole brain volume and chorea subscale of the UHDRS; and the whole brain volume and UHDRS eye movement score.

Conclusion: Our data showed, as expected, that the brain volumes were lower in HD than controls; nevertheless, cerebellum volumen were higher in HD patients, as described before in early-onset HD. Overall, a negative relationship of brain volumes with clinical scales scores was found. Volumetric MRI features contribute in the understanding of the clinical manifestations of this complex disease.

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MDS Abstracts - https://www.mdsabstracts.org/abstract/cerebellar-and-other-motor-symptoms-relationship-with-mri-features-in-huntington-disease/