My husband recently found out his Mother had Huntington's Disease. She has since passed away, but it is beleived that his older sister has this desease but she will not get tested. My husband is 39 years old and is terrified he also may have this. We don't know too much about it, but I was wondering about movement while he is sleeping. I've noticed that during his sleep he twitches kind of. He usually does it with his legs at about 5 minute intervals. One night I timed him for about 45 minutes. He is not aware he is doing it, but I remember his Mom twitching all the time and constantly moving (waving) her hands. Could this be a sign of the disease?

Hi Stephanie,
Although I'm sure the twitching doesn't necessarily indicate Huntington's Disease, it most definitely is a symptom of HD for many Phds. My wife, who was diagnosed with HD about five years ago has been going through the night-time leg twitching episodes for the last three years. It usually stops after she is sound asleep but just before she falls asleep it can be rather annoying. Before I "accepted it" as part of HD I used to tell her to stop it but a couple of times she told me she wasn't doing anything. My wifes "twitching intervals" when she is "doing the dance" is about every three seconds.

I don't know if you've visited any of the chat rooms but please do so. There are many different experiences with HD and all of the participates would love to share their experiences with you and your husband. HD is a terrifying disease for both the person at risk and the family but, and I speak from my own personal experience here, it is a LOT less terrifying when you have others who've been there helping to coach you through this unknown territory. The MGH chat room is usually occupied. You can also sign up with one or more of the Yahoo Clubs. HD Support and The Monster are the more active clubs that I know about.

Hope to visit with you soon,
Steve

Hi Stephanie, my name is Loretta Church and I read your posting on the website. I do not know if the movements during the night are a symptom of hd. Have you contacted a genetic counselor in your home town??? Have you contacted anyone form the national HSDA office?? This can be done by email. I want to offer my love and support to you and your family. My son Marc, 24, has jhd so I truly understand your anger, fear and pain. Please email me if you are comfortable. Loretta

Hi Loretta, I just read your story on the home page and it brought me to tears. I can only imagine what you are going through. As I said, my husband's mother had this disease but she lived with his sister and they were not very close so we did not observe alot of her behavior. No one bothered to figure out what was wrong with her until the last year of her life, she was placed in a nursing home, and that is when she was diagnosed.

My husband refuses to get any testing done. The thought of him having this terrible disease scares him to death! I haven't really thought much about it until recently when I noticed the leg twitching in the night. It is my understanding that if your parent has it, then the children have a 50% chance of having the disease. Is that right? His sister definately exhibits the same behavior as I saw in his mom, yet she has never been tested either. It's like everyone knows, but they are in denial. As I've stated, we are not very close with his side of the family, so I really don't get alot of information out of them. His Mom passed away in her mid seventies. His sister is in her late forties. I have also heard that if you have Huntington's disease you normally to not live as long as his mom did. Also that if you do not show the signs by the time you are 50, odds are you do not have this either. Is there a average age of death?

Thanks for your response to my message.

God bless you and your family.

Stephanie, welcome to HDAC! If an individual's parent has the HD gene, his chance of also having the gene is 50 %. As time goes by and the person is still symptom free, the likelihood that he did in fact get the gene goes down. Only 31.5 percent of people who were at 50 percent risk of having the gene and who have reached age 50 without having signs of HD actually do have the gene.

Click on this link and scroll down to Table 2 to see how the likelihood decreases with every symptom free year:

[www.geneclinics.org]

Does anyone know about a "light" case of HD? Or a "stiff" form? My grandma moved her torso back and forth and her arms were stiff, not jerky. She died at age 87. My father died at age 73 of the disease. He also was more stiff than jerky. But my brother is very jerky. He is 51. I am 46 and I think I have a "light" case (if there is such a thing) and my movements are stiffish. Any experience of this kind from others would be interesting to read.

Hi Debbie,

In regards to a"light" case of HD, my mother was diagnosed with HD 1 1/2 yrs ago and her CAG repeat was 42 and the Dr. at John Hopkins said she had a "mild" although she is now in the final stages at 75 yrs. old She also has very stiff arms and legs. Hope this helps. Jeanne Wilson

My grandmother died with HD at age 87, also. I guess you could consider her case a "light" case in that she progressed very slowly over a period of about 30 years rather than the more typical 15 or so. Also, her mental capacities seemed to remain intact for quite some time and her speech was understandable until the last two years. Her choreaic movements were not very pronounced at all. That's one reason, I think, it took so long for her to get a diagnosis of HD. I think her movements could be categorized as more stiff than the classic HD movements that my mom now has.

I have read that late onset HD will sometimes result in reduced symptoms and better cognitive retention. I also read that a woman with late onset will sometimes pass that late onset on to her pHD children. I also know that, unfortunately, it doesn't guarantee a late onset for children. It's just that women who pass on the HD gene tend to have a more stable CAG count and sometimes a lower CAG means later onset.

salt

Debbie

Your question is a very interesting one.

No two cases of HD are the same. Some pHDs (people with HD) start out with
movements (chorea), others with pesonality changes or mental problems. The
movements can be different, too.

Complicating all of this is the effect of age. The age of onset may affect
the sorts of movements. (See below for juvenile HD.) While not everyone
agrees about this, many of us believe that, in late-onset HD, which may be
what your grandma and father had, the symptoms are less severe and the
disease progresses more slowly. Also, as the disease progresses, the
symptoms change, and it's quite common for the chorea to be replaced by
stiffness.

Stiffness is also characteristic of what is called the "Westphal variant" of
HD.

HDSA's "Physician's Guide to HD" (at least, the old version) says,
"When HD begins in childhood or adolescence (juvenile-onset HD), it usually
presents with school failure, bradykinesia and dystonia, with or without
hypertonia. Involuntary movements may take the form of tremors instead of
chorea and seizures may occur. This rigid-akinetic form (called the
Westphal variant of HD) tends to worsen more rapidly, with duration rarely
exceeding 14 years."

The age of your grandma might rule that out, however. I also found this:

"HUNTINGTON'S DISEASE (WESTPHAL VARIANT)
[www.icondata.com]
A progressive neurodegenerative disorder characterized initially by
bradykinesia and rigidity then choreiform movements."

(I apologize for the fact that I don't have definitions for bradykinesia,
dystonia, and hypertonia handy.)

I don't think there really is such a thing as a "light" case of HD, except,
perhaps in some people with late-onset HD; in that case, the disease
progresses so slowly that they die of other things before the HD can get
really bad. A case may seem "light" at first simply because the symptoms
almost always progress slowly.

Why do you think you have HD? Those of us who are at risk are always
diagnosing ourselves and others, and we're frequently wrong. If you haven't
already done so, I'd urge you to consult a neurologist with some HD
expertise. If she/he believes you have it, then she/he will probably give
you the genetic test to confirm it. Then you'll know for sure and can make
plans for dealing constructively with HD. If not, you may decide to be
tested anyway, or you can rest a little easier (for a while), then go back
to symptom-hunting again.

I wish you the best.

Tom Caldwell
Tucson, Arizona
mailto:TECaldwell*at*email.msn.com

Thanks for your interesting info. I've already been diagnosed with HD by a neurologist, though didn't have a genetic test. I'm just curious about what others have experienced. For awhile I thought I might be imagining the symptoms, but one symptom couldn't be imaginary, and that is my strange inability to type correctly. I've always typed a lot, all my life, but suddenly my letters get mixed up and I have to go back to correct them. Debbie.

Dear Mr. Caldwell, My boyfriend recently told me hes been diagnosed with H.D., please explain what this is and its symptoms because hes been no help at all and I fear that my best friend and soul mate will die before we can share our lives. Hes only 35 and said he knows of noone in his family with this disease.

Tom Caldwell wrote:
-------------------------------
Debbie

Your question is a very interesting one.

No two cases of HD are the same. Some pHDs (people with HD) start out with
movements (chorea), others with pesonality changes or mental problems. The
movements can be different, too.

Complicating all of this is the effect of age. The age of onset may affect
the sorts of movements. (See below for juvenile HD.) While not everyone
agrees about this, many of us believe that, in late-onset HD, which may be
what your grandma and father had, the symptoms are less severe and the
disease progresses more slowly. Also, as the disease progresses, the
symptoms change, and it's quite common for the chorea to be replaced by
stiffness.

Stiffness is also characteristic of what is called the "Westphal variant" of
HD.

HDSA's "Physician's Guide to HD" (at least, the old version) says,
"When HD begins in childhood or adolescence (juvenile-onset HD), it usually
presents with school failure, bradykinesia and dystonia, with ....