Objective: To assessed functional connectivity by using functional Magnetic Resonance Imaging (fMRI) at 7 Tesla as baseline and one year thereafter.

Background: Our previous study showed reduced cortico-striatal functional connectivity at rest in Huntington’s disease (HD) at 3 Tesla (Unschuld et al. Neurosci Letter 2012) and at 7 Tesla (Kronenbuerger et al. work in progress). Little is known about changes of cortico-striatal functional connectivity in HD over time.

Methods: 9 HD subjects [age 46+/-12 years, CAG repeat 43+/-3, Unified Huntington’s disease Rating Scale (UHDRS) motor score -14+/-18, estimated years to symptom onset (YTO) 5+/14 according to Langbehn et al. Clin Genet 2004], and 8 healthy controls [age 47+/-12 years] were studied. The functional connectivity between the striatum and several cortical areas were analysis. Two tailed t-test was applied.

Results: Functional connectivity between striatum and premotor area was impaired comparing all HD subjects with controls at baseline (p<0.01) and the one year follow-up (p<0.01) (Table 1). HD patients showed greater decline of functional connectivity between striatum and premotor area over time, with a trend to statistical significance (p=0.09). All other comparisons did not reveal statistical significance differences.

Conclusions: These data confirm impairment of functional connectivity between striatum and premotor area in HD compared to healthy controls. In this small sample, worsening with time in HD is suggested. We plan to examine a larger number of subjects in the follow-up to assess if the impairment of functional connectivity between striatum and premotor area is worse in premanifest compared to manifest HD, and to determine if there is longitudinal progression progresses over time.

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MDS Abstracts - https://www.mdsabstracts.org/abstract/longitudinal-study-of-cortico-striatal-functional-connectivity-in-huntingtons-disease-measured-with-high-field-functional-mri/