Therapy in Huntington’s Disease: Where Are We?

Abstract

As of 2012, almost 20 years after the discovery of the causative gene, clinical research has yet to find a disease-modifying treatment for Huntington's disease. However, both pharmacologic and nonpharmacologic therapies are available for many of the common symptoms of the disease. Recent studies of gene-positive patients in the prodromal, not clinically diagnosable, stages of the disease, are changing our perception of when the process of neurodegeneration begins. Once disease-modifying therapies become available, the approach to the diagnosis of Huntington's disease will likely shift from an examination-based clinical diagnosis, to one that includes a more complex combination of imaging, examination, and biomarker analysis.

The Q175 Mouse Model of Huntington’s Disease Shows Gene Dosage- and Age-Related Decline in Circadian Rhythms of Activity and Sleep

“…HD patients present primarily with involuntary movements (chorea) and loss of motor control. It is invariably fatal, age of onset varies with the severity of the mutation, and there are no significant treatment options to date [2], [3]. In addition to the motor dysfunction, HD patients often present with non-motor symptoms that include cognitive dysfunction [4], [5], affective disorders [6]–[8] and sleep and circadian rhythm disruptions [9]–[12], which can all precede the onset of chorea.…”

The influence of motor ability rehabilitation on temporal-spatial parameters of gait in Huntington's disease patients on the basis of a three-dimensional motion analysis system: An experimental trial

“…Among the three publications, we can find analysis of changes in kinematic parameters, and only one publication analyses, according to the concept by Elftman, the relations of the ''locomotor'' and ''passenger'' during gait in people with HD [19]. The conducted review of existing literature relating to treatment available in HD still shows an insufficient number of scientific reports confirming the influence of physiotherapy on kinematic and spatial-temporal parameters of gait in people with HD [20]. The article by Busse et al [21], based on surveys conducted among physiotherapists who work with HD patients every day, provides a good basis for the creation of standards of conduct in the physiotherapy of people with HD, and for the avoidance of basic mistakes in the course of research planning.…”

Huntington's disease: advances, controversies, and challenges

“…3 To date, psychotherapy, speech therapy, and physical and occupational therapy are the most effective treatment methods available. 6 Advances are being made through stem cell research to replace lost neurons as a form of latestage intervention, making it possible to regain some loss of function. 5 Evidence also suggests that coenzyme Q10 and daily doses of creatine may improve the functional decline in HD patients; Phase III studies are under way to assess these effects.…”