Physiotherapy and Huntington’s disease

 

Physiotherapy and Huntington’s disease

 

Kenny Vuong, Tim Sheehy, Paige Hooper, Chris Jones and Dharsha Petrie of the APA Neurology national group present five discussion points about physiotherapy management of Huntington’s disease. 

1.    Huntington’s disease is not just a hyperkinetic movement disorder

Huntington’s disease (HD) is a genetic neurodegenerative disorder with a typical age of onset in the 30s and 40s.

It has devastating consequences for individuals and their families, with symptoms that include motor disturbances, cognitive impairment and psychiatric disorders.

 Although HD is a progressive disorder, the progression is usually relatively slow—patients often live 10–30 years post-diagnosis—and effective, multidisciplinary care that includes physiotherapy can optimise quality of life for patients.

 One of the most recognisable features of HD is chorea, which can be described as irregular, involuntary movements that are sudden, brief, unpredictable and non-stereotypical.

It commonly affects the upper and lower limbs and can affect trunk movements.

People with HD present not only with hyperkinetic features, but also with other motor disorders such as bradykinesia, akinesia and dystonia (Vuong et al 2018).

In later stages of the disease, hypokinesia and rigidity are very common (Pagan et al 2017).

Cognitive disturbances and behavioural and psychiatric symptoms are often present early on in the disease and can precede the motor symptoms.

Due to frontal lobe dysfunction, clients with HD often lack insight into their symptoms and impairments and thus family members and support networks are crucial for important decision-making about ongoing care.

2.    Exercise can stabilise motor function in people with HD

People with HD are weaker, walk less and fall more (Dawes et al 2015).

While studies are limited, it is reported that people with HD have reduced work capacity and lower limb muscle strength (Busse et al 2008), leading to a reduced ability to perform aerobic exercise with increased lactate synthesis during intense physical exercise (Ciammola et al 2011).

Although clinical muscle weakness isn’t always detected when a person has received an early diagnosis of HD, there are subclinical features indicating its presence, including myopathic changes on biopsy and biochemical evidence of mitochondrial dysfunction (Busse et al 2008).

Prescribing exercise is safe, feasible and effective in individuals with HD (Quinn et al 2020).

 

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