Huntington's disease more prevalent than officially estimated

02 July 2010

An article in the Guardian in this week highlights the plight of Huntington's disease (HD) sufferers, who live in the shadows from fear and stigma of insurance companies.

Professor Sir Michael Rawlins, Chairman of the National Institute for Health and Clinical Excellence (NICE), argues in the Lancet journal that although 6.7 out of 100,000 people are estimated to suffer from HD, the number could well be higher.

The Huntington's Disease Association cares for 6,702 people in England and Wales. "Specialist neurologists have referred all of them, so their diagnoses can hardly be in doubt", he writes. "From these numbers alone, the minimum prevalence in England and Wales must therefore be at least 12·4 per 100,000."

HD is a neurodegenerative disease, caused by a faulty gene on chromosome 4 which is passed down through families.

If an individual has one parent with the HD gene, he or she has a 50/50 chance of inheriting the gene and developing HD themselves. The symptoms of the disease usually develop when people are between 30 and 50 years old and progress relentlessly over the next ten to twenty years.

Sarah Tabrizi, Professor of Clinical Neurology and head of the Institute of Neurology's active HD research team, says that currently only 20% of individuals at risk of inheriting the condition actually get tested for the faulty gene.