Effect of Triheptanoin on Caudate Atrophy and Motor Scores in Patients With Early-Stage Huntington Disease: A Phase II Study.

Background And Objectives: Brain energy deficiency occurs at the early stage of Huntington disease (HD). Triheptanoin, a drug that targets the Krebs cycle, can restore a normal brain energetic profile in patients with HD. In this study, we aimed at assessing its efficacy on clinical and neuroimaging structural measures in HD.

Dysphagia in Huntington's disease: a review.

Huntington's disease (HD) is a progressive neurodegenerative autosomal dominant disease characterized by disturbed movements and behavior and cognitive decline. The motor disturbances are both choreiform and hypokinetic. As a result of the combination of these signs, it is known that many patients with HD suffer from dysphagia.

Hypokinesia in Huntington's disease co-occurs with cognitive and global dysfunctioning.

Besides chorea, hypokinesia is an important motor disturbance in Huntington's disease (HD) but its clinical, neuropsychiatric, and cognitive functioning correlates are largely unknown. This cross-sectional study investigates correlates of hypokinesia in HD and its effect on global functioning. Among 150 HD gene carriers, 96 patients were clinically motor manifest.