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Background: Huntington disease (HD) is a progressive neurodegenerative disease that causes psychiatric and neurological symptoms, including involuntary and irregular muscle movements (chorea). Chorea can disrupt activities of daily living, pose safety issues, and may lead to social withdrawal. The vesicular monoamine transporter 2 inhibitors tetrabenazine, deutetrabenazine, and valbenazine are approved treatments that can reduce chorea.

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Total functioning capacity scale in Huntington's disease: natural course over time.

J Neurol

January 2025

LUMC Department of Neurology, Albinusdreef 2, 2333 ZA, Leiden, The Netherlands.

Background And Objectives: The total functioning capacity (TFC) assessment has been integral to Huntington's disease (HD) research and clinical trials, measuring disease stage and progression. This study investigates the natural progression of function in HD, focusing on changes in TFC scores related to age and CAG-repeat length, and evaluates TFC's strengths and weaknesses in longitudinal studies.

Methods: Using Enroll-HD platform's clinical dataset version 5, including Registry-3, we analysed data from 21,079 participants, with 16,083 having an expanded CAG repeat.

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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.

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Effects and mechanisms of computerized cognitive training in Huntington's disease: protocol for a pilot study.

Neurodegener Dis Manag

January 2025

Turner Institute for Brain & Mental Health, School of Psychological Sciences, Faculty of Medicine, Nursing & Health Sciences, 18 Innovation Walk, Monash University, Clayton VIC 3800, Australia.

Huntington's disease (HD) causes progressive cognitive decline, with no available treatments. Computerized cognitive training (CCT) has shown efficacy in other populations, but its effects in HD are largely unknown. This pilot study will explore the effects and neural mechanisms of CCT in HD.

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Background: Diabetic striatopathy (DS) is a rare disorder characterized by clinical manifestations of hemichorea, non-ketotic hyperglycemia, and high signal on T1-weighted MRI or high density on CT scan in basal ganglia, typically associated with poor glycemic control.

Objective: This study aimed to analyze clinical characteristics of patients with diabetic striatopathy to raise awareness amongst physicians, especially endocrinologists, about this rare neurological manifestation in patients with diabetes.

Methods: We retrospectively analyzed the data on clinical presentations, laboratory workups, and cranial CT and MRI of six patients with DS who were admitted to our hospital from October 2013 to June 2022.

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