Huntington’s Disease

Disease

Huntington’s disease (HD) is an inherited neurodegenerative disease caused by an enlarged repetition of the cytosine-adenine-guanine gene (the CAG sequence). In the carriers of the gene, a protein called huntingtin, which we all produce, repeats itself in an amplified way, thus undergoing a mutation that alters its functions. It attacks first the striatum and then the whole encephalon. The exact mechanism is still unknown, but it causes the death of cerebral neurons. The average age of diagnosis is 40-45 years, but the symptoms are often present for several years prior to diagnosis. The duration of the disease usually spreads over 15-20 years, but the onset of symptoms and severity vary from one individual to another.

Symptoms

HD is characterized by motor disorders, psychiatric/psychological symptoms, and cognitive decline. Motor disorders are the most visible signs of the disease. Individuals with HD exhibit an excess of movements known as chorea. In most cases, they have an impaired gait, loss of balance and a lack of coordination, often leading to falls. On the psychiatric/psychological level, depression and irritability are common. As the illness evolves, apathy, hallucinations and delusions can appear. At the cognitive level, complex thinking skills can be affected early in the course of illness, leading to planning and organizational problems and increasingly severe memory loss. Although there are three stages of the disease, the onset of symptoms and their development varies greatly from one person to another. The physical, psychological and cognitive state can be completely different from one person to another, even in an equivalent stage.

Exercise and Illness

Although the literature on the effects of physical activity on HD is still scarce, research conducted since the beginning of the 21st century is generally positive. On the one hand, research suggests that regular exercise may slow the progression of the disease [1]. On the other hand, several symptoms can be improved by interdisciplinary care that includes physical activity. There are positive effects on quality of life, anxiety, depression and, more importantly, on walking and balance, two factors at the base of falls prevention [2, 3]. Recent studies report that it is realistic, safe and beneficial for people with HD to do home exercise programs [4-6]. The support of a professional remains essential so that the program is adapted to the health status and the symptomatology of each person. NeuroMotrix trainers are able to respond adequately to these needs.

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Sources

  1. Trembath, M.K., Z.A. Horton, L. Tippett, V. Hogg, V.R. Collins, A. Churchyard, D. Velakoulis, R. Roxburgh, and M.B. Delatycki, A retrospective study of the impact of lifestyle on age at onset of Huntington disease. Mov Disord, 2010. 25(10): p. 1444-50.
  2. Piira, A., M.R. van Walsem, G. Mikalsen, K.H. Nilsen, S. Knutsen, and J.C. Frich, Effects of a One Year Intensive Multidisciplinary Rehabilitation Program for Patients with Huntington’s Disease: a Prospective Intervention Study. PLoS Curr, 2013. 5.
  3. Zinzi, P., D. Salmaso, R. De Grandis, G. Graziani, S. Maceroni, A. Bentivoglio, P. Zappata, M. Frontali, and G. Jacopini, Effects of an intensive rehabilitation programme on patients with Huntington’s disease: a pilot study. Clin Rehabil, 2007. 21(7): p. 603-13.
  4. Busse, M., L. Quinn, K. Debono, K. Jones, J. Collett, R. Playle, M. Kelly, S. Simpson, K. Backx, D. Wasley, H. Dawes, A. Rosser, and C.-H.D.M.G. Members of the, A randomized feasibility study of a 12-week community-based exercise program for people with Huntington’s disease. J Neurol Phys Ther, 2013. 37(4): p. 149-58.
  5. Khalil, H., L. Quinn, R. van Deursen, H. Dawes, R. Playle, A. Rosser, and M. Busse, What effect does a structured home-based exercise programme have on people with Huntington’s disease? A randomized, controlled pilot study. Clin Rehabil, 2013. 27(7): p. 646-58.
  6. Khalil, H., L. Quinn, R. van Deursen, R. Martin, A. Rosser, and M. Busse, Adherence to use of a home-based exercise DVD in people with Huntington disease: participants’ perspectives. Phys Ther, 2012. 92(1): p. 69-82.

 

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