Objective: To identify structural involvement of the corticospinal pathway in Huntington disease (HD) with clinical upper motor neuron signs using diffusion tensor imaging (DTI).

Background: Although, HD is characterized by a selective degeneration of striatal neurons, several studies suggest a corticospinal tract involvement. A marked loss of pyramidal neurons in the primary motor cortex has been related to motor dysfunction. However, only scarce information is available about the presence of pyramidal motor signs in patients with HD.

Methods: This observational study was approved by institutional review board. Six HD patients from our data base with upper motor neuron signs (excluding potential spinal or other unrelated HD causes) composed the sample. We used Diffusion Tensor Imaging (DTI) to investigate corticospinal tract.

Results: 6 HD patients (1 man, 5 women) composed the sample, mean age at onset 35 years old; mean diseases duration 6.83 years; mean nCAG expanded allele 46.5. Pyramidal signs included Hoffman and Babinski signs, hyperreflexia and clonus. DTI failed to identify corticospinal tract impairment in this population.

Conclusions: In our patients we failed to identify abnormalities in corticospinal tract with DTI. This results should be taken cautiously due to the small sample size. A study with a larger sample of patients is ongoing to confirm this preliminar findings.

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MDS Abstracts - https://www.mdsabstracts.org/abstract/magnetic-resonance-imaging-and-pyramidal-impairment-in-huntington-disease/