"An emotional research team became tearful as they described how data shows the disease was slowed by 75% in patients.
It means the decline you would normally expect in one year would take four years after treatment, giving patients decades of "good quality life", Prof Sarah Tabrizi told BBC News.
The new treatment is a type of gene therapy given during 12 to 18 hours of delicate brain surgery.
The first symptoms of Huntington's disease tend to appear in your 30s or 40s and is normally fatal within two decades – opening the possibility that earlier treatment could prevent symptoms from ever emerging."
Results from the trial - which involved 29 patients - have been released in a statement by the company uniQure, but have not yet been published in full for review by other specialists.
The data showed that three years after surgery there was an average 75% slowing of the disease based on a measure which combines cognition, motor function and the ability to manage in daily life.
The data also shows the treatment is saving brain cells. Levels of neurofilaments in spinal fluid – a clear sign of brain cells dying – should have increased by a third if the disease continued to progress, but was actually lower than at the start of the trial.
"This is the result we've been waiting for," said Prof Ed Wild, consultant neurologist at the National Hospital for Neurology and Neurosurgery at UCLH.
"There was every chance that we would never see a result like this, so to be living in a world where we know this is not only possible, but the actual magnitude of the effect is breathtaking, it's very difficult to fully encapsulate the emotion."
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Prof Wild anticipates the therapy "should last for life" because brain cells are not replaced by the body in the same manner as blood, bone and skin are constantly renewed.
Approximately 75,000 people have Huntington's disease in the UK, US and Europe with hundreds of thousands carrying the mutation meaning they will develop the disease.
UniQure says it will apply for a licence in the US in the first quarter of 2026 with the aim of launching the drug later that year. Conversations with authorities in the UK and Europe will start next year, but the initial focus is on the US."
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Dr Walid Abi-Saab, the chief medical officer at uniQure, said he was "incredibly excited" about what the results mean for families, and added that the treatment had "the potential to fundamentally transform" Huntington's disease.
However, the drug will not be available for everyone due to the highly complex surgery and the anticipated cost.
"It will be expensive for sure," says Prof Wild.
There isn't an official price for the drug. Gene therapies are often pricey, but their long-term impact means that can still be affordable. In the UK, the NHS does pay for a £2.6m-per-patient gene therapy for haemophilia B.
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Prof Tabrizi says this gene therapy "is the beginning" and will open the gates for therapies that can reach more people.
She paid tribute to the "truly brave" volunteers who took part in the trial, saying she was "overjoyed for the patients and families".
She is already working with a group of young people who know they have the gene, but don't yet have symptoms – known as stage zero Huntington's – and is aiming to do the first prevention trial to see if the disease can be significantly delayed or even stopped completely.
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