introduction: as an inherited neurodegenerative disease, huntington's disease is accompanied with wide neuronal degeneration in neostriatum and neocortex. progress of the disease causes disabling clinical effects on movements, recognition and physiology of the body, and finally results in death. at this stage of knowledge we are, there is no effective therapeutic strategy for diminishing t...

BACKGROUND The Unified Huntington's Disease Rating Scale (UHDRS) is the principal means of assessing motor impairment in Huntington disease but is subjective and generally limited to in-clinic assessments. OBJECTIVE To evaluate the feasibility and ability of wearable sensors to measure motor impairment in individuals with Huntington disease in the clinic and at home. METHODS Participants wi...

Huntington disease is an incurable, dominant neurodegenerative disorder caused by polyglutamine repeat expansion in the huntingtin protein. Reducing mutant huntingtin expression may offer a treatment for Huntington disease. RNA interference has emerged as a powerful method to silence dominant disease genes. As such, it is being developed as a prospective Huntington disease therapy. Here I discu...

introduction: huntington disease (hd) is a progressive neurodegenerative disease which affects movement control system of the brain. hd symptoms lead to patient’s gait change and influence stride time intervals. in this study, we present a grey box mathematical model to simulate hddisorders. this model contains main physiological findings about bg. methods: we used artificial neural networks (a...

Huntington disease is a neurodegenerative disorder caused by an expanded CAG repeat in the Huntington disease gene. The symptomatic phase of the disease is defined by the onset of motor symptoms. However, psychiatric disturbances, including depression, are common features of Huntington disease and recent studies indicate that depression can occur long before the manifestation of motor symptoms....

PURPOSE To describe the clinical and radiologic manifestations of juvenile Huntington disease and to determine whether adult imaging criteria for Huntington disease are helpful for pediatric patients. METHODS Six patients (3 to 18 years of age; mean age, 9.8 +/- 5.6 years; 3 female, 3 male) with juvenile Huntington disease were studied with CT (n = 6) and/or MR (n = 3). CT and MR studies were...

OBJECTIVES Individuals at risk for Huntington disease face difficult decisions regarding their reproductive options. Most do not wish to pass on the gene for Huntington disease to their children, but may not be prepared themselves to undergo presymptomatic testing and learn their genetic status. For these reasons, many at-risk individuals with a family history of HD would choose a method of gen...

INTRODUCTION Various neuropsychological studies have been conducted to determine the cognitive functioning of patients with Huntington s disease and their results have shown that the pattern of cognitive decline is thought to be typical of a frontal subcortical dementia. OBJECTIVES To determine if significant differences exist between the cognitive performances of a group diagnosed with Hunti...