Huntington's Disease Protects Against Cancer

People with motor neuron diseases such as Huntington's disease seem to be protected against cancer, researchers reported.

The underlying cause of the "PolyQ" diseases is an increased number of glutamine units in different genes, according to Jianguang Ji, MD, and colleagues at Lund University in Malmö, Sweden.

But patients with the polyglutamine tracts – and therefore one of nine different PolyQ diseases - have significantly lower cancer risks than the general population, Ji and colleagues reported online in The Lancet Oncology.

The finding suggests that a common mechanism - perhaps related to the increased number of glutamine units - underlies the protective effect, they argued.

The PolyQ diseases include Huntington's, spinobulbar muscular atrophy (SBMA), dentatorubral and pallidoluysian atrophy, and six types of spinocerebellar ataxia.

Huntington's disease an inherited, genetic condition that affects the brain and nervous system. It is a slowly progressive condition that interferes with the movements of your body, can affect your cognition (your perception, reasoning, awareness, thinking and judgement) and can lead to a change in your behaviour. The symptoms occur because of damage and death of some of the neurones (brain cells) in particular parts of your brain. Genetic testing helps to diagnose Huntington's disease. At present there is no cure for Huntington's disease. Treatment is aimed at trying to control symptoms as much as possible when they develop.

They are all characterized by progressive degeneration of motor neurons, although the details vary. All have an increased number of repeated genetic sequences - cystosine-adenine-guanine - but in different genes.

What is Huntington's disease? Huntington's disease (HD) is named after George Huntington who first described it in 1872. It is an inherited, genetic condition that affects the brain and nervous system. It can interfere with movements of your body, can affect your cognition (your perception, reasoning, awareness, thinking and judgement) and can lead to a change in your behaviour.

Observations have suggested that Huntington's patients have a lower risk of cancer, the researchers noted, but those could be accounted for by a range of factors, including a tendency for doctors to miss the cancer diagnosis because of concern for the main disease.

Huntington's disease is a neurodegenerative disease where nerve cells die off, including in the basal ganglia. This is the part of the brain that co-ordinates movements, but that is also important for other functions, such as integrating cognitive tasks. The disease, that generally manifests itself around the age of forty, is characterised by hyperkinesia and cognitive degeneration in patients.

To help clarify the matter, Ji and colleagues turned to Sweden's nationwide medical records system, which allowed them to find patients with Huntington's, spinobulbar muscular atrophy, and hereditary ataxia (which was used as a proxy for spinocerebellar ataxia).