New Hope for Those with Huntington’s

xray brain scan imagingHuntington’s disease has long been a source of terror for people at risk of developing it. Huntington’s is genetic, and people with the medical condition don’t typically know they have it without genetic testing. Symptoms can manifest at any time, but typically begin in one’s 30s or 40s. Once symptoms appear, they can quickly become debilitating.

Huntington’s can destroy motor skills, alter emotional reactions, and lead to a steady decline in cognition. The disease is ultimately fatal. Legendary folk singer Woody Guthrie died of Huntington’s, and little progress has been made toward a cure or treatment for it. A new study, though, sheds light on a potential treatment.

An Animal Model for Treating Huntington’s

Huntington’s disease causes rapid cell death in the brain, and researchers have long known that a neurotransmitter called glutamate can speed up cell death. The University of British Columbia study examined the effects on animals of blocking a group of glutamate receptors called extra-synaptic NMDAR receptors. These receptors have previously been implicated in other neurodegenerative conditions such as Alzheimer’s. When researchers administered a drug designed to block NMDAR receptors, cell death slowed and motor skills and coordination improved.

Because Huntington’s disease typically affects motor skills first, the results are promising. The treatment is not a cure for Huntington’s, but it could be an effective treatment. It’s now routine for people whose parents or grandparents had the health issue to undergo genetic testing. Because people Huntington’s typically learn of their condition years or even decades before they experience symptoms, there’s often plenty of time to try out treatments. If blocking glutamate receptors proves as fruitful in humans as it does in animals, people who learn they have Huntington’s could begin treatment immediately, potentially delaying symptoms or even preventing them from developing entirely.

The research is preliminary, and animal models don’t always neatly apply to humans. Since NMDARs play roles in several brain disorders, though, it’s likely that blocking them could work as well in humans as it does in animals.

References:

  1. Huntington disease. (n.d.). Retrieved from http://ghr.nlm.nih.gov/condition/huntington-disease
  2. Promising approach to slow brain degeneration in Huntington’s disease uncovered. (2014, May 25). Retrieved from http://www.sciencedaily.com/releases/2014/05/140525204424.htm

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  • Rally

    Rally

    May 29th, 2014 at 2:17 PM

    I have never even heard of this but I guess that’s because I have never known anyone with the disease or a family history of it.
    It sounds kind of like a cross between Parkinson’s and Alzheimer’s, would that be close?

  • Michael

    Michael

    May 30th, 2014 at 7:38 AM

    That is exactly the comparison I use when I explain it to friends. It’s a neurodegenerative disease that severely impairs motor function as well as cognitive ability. It has a way of magnifying certain personality traits and hiding others.

    After time, everyday activities, walking, and even talking become extremely difficult or impossible and it’s just as hard on families as it is on the patient.

  • Sam

    Sam

    May 29th, 2014 at 6:39 PM

    The antagonist NMDR sounds very promising indeed!

  • wilson t

    wilson t

    May 30th, 2014 at 3:56 AM

    Isn’t it weird how there are some diseases that have such a vocal and outspoken lobby and then there are other ones like this that the only ones who are familiar with it are those who experience it firsthand?
    I mean, we have all heard of cancer, heart disease, etc but some of these that are genetic fly totally beneath the radar screen.
    That must make it very hard for reserach and funding when there are not a whole lot of people familiar with it.

  • powell

    powell

    May 30th, 2014 at 11:49 AM

    This has to be great news for the families who are losing loved ones to this- it sounds just terrible. I know that there is always hope out there that something is going to come along and at least help to slow the progression of the disease and for these families this could be what they have been hoping and praying for. Just now you have to wonder how long it takes to get this research and turn it into something that is actually useful for the patients who have Huntington’s or even those who are genetically predisposed to developing the illness.

  • Becky

    Becky

    May 31st, 2014 at 6:05 AM

    At least this is a disease that has very early warning signs and moat people are aware of their likelihood to develop it. You at least, when you ahev this type of forewarning, know what to be on the lookout for and some things that you may could do in the future to halt or at least slow the progression.

    I have often wondered if something like this ran in my family if I would want to know about it ahead of time and I think that with certain things I just wouldn’t wnat to know, especially if it felt like there was nothing that could be done to treat the disease once I discovered that I had it.

    With a disease like this though where there seems to be so much hope gaining, I think that I would wnat to know because it sounds like there are a multitude of treatments as well as things that I may could begin doing on my own to ensure that I still have a life after its onset.

  • Travis t

    Travis t

    June 11th, 2014 at 4:20 AM

    anything, even if it is just a halt in the progression of the disease if not an outright cure, must give this community som e hope to hold onto.

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