What should we expect? How fast does it usually progress? So far he is slurring words, facial expressions have changed, leg cramps and numbness in 1 foot. We thought he might have ALS which is what my husband died from 3 years ago.

Susan:

You've come to the right place for information.

Just from personal experience, ALS and Kennedy's are very similar at the beginning and KD in particular used to be quite often misdiagnosed.

My suggestion would be to make sure your brother in law has KD. There are very reliable tests available that can identify the disease. Once you know for sure, it'll be easier to get most of facts about it.

From my own personal opinion, it seems like not everyone experiences the same symptoms. For me, I've never experienced slurring (ask my wife), leg cramps or pains of any sort, even though these are some of the most common ailments suffered in KD.

Progression is also quite different for each individual. The only common thing is the weakness of muscles (specially arms and legs) along with an increase of mental toughness and hope that we will all beat this disease.

Are you talking years before his muscles will become weak and wasted or a shorter time? I know it is different for all, but just kind of an estimate of how fast this progresses. Do people with this disease end up with feeding tubes or need help with breathing?

Susan:
One of the major differences between ALS and KD is life expectancy. When I was first (mis)diagnosed, I was told I had ALS and roughly 3 years to live. That was 26 years ago.

Progression of this disease has been slowly but consistent and that's why my first suggestion was to make sure whether your brother in law suffers from ALS or KD. To put it bluntly, if it is ALS, progression will normally occur fairly rapidly with a life expentancy of less than 5 years; however, if it is KD, I've read that quite a few of us either die of old age or another unrelated disease.

My uncle (non-blood related) passed away from ALS complications many years ago and I know that he eventually went thru the feeding and breathing tubes. I do not know anyone with KD having to go thru this same ordeal but I suspect it may be the same.

The above information only reflects my own personal experiences and beliefs. As mentioned in my previous response, everyone is affected differently and therefore may not totally agree with this response.

Once again, my suggestion would be to have your brother in law tested to make sure what he/you are dealing with.

Good Luck!!!

He is being tested by a neurologist and they are leaning heavily towards Kennedy's Disease and do not think it is ALS. He should have the results in another couple of weeks. They took alot of blood, etc. We just thought it might be ALS at the beginning before he went to the specialist because of the symptoms since my husband did have ALS and I am quite familiar with that disease.

Susan:
It is my understanding that determining whether an individual has KD is a simple process when done properly. There are several lab locations listed on this website that can help you and your brother in law if you don't feel confident with his neurologist's results.

Hello Susan,

On the question of what symptoms to expect, I suggest you read this page .

On the question of whether your relative actually has SBMA or not, the only way to know for sure is the DNA test, which begins with a blood sample. When receiving the results of the test, he should insist upon both (1) getting a copy of the test results in writing, and (2) understanding the number of CAG-repeats in his DNA. He will need to know the number of repeats for future reference. He may have to get quite pushy with the neurologist to get this information, but he must insist on it.

If he does have either ALS or SBMA/Kennedy's Disease, he will want to be referred to a physical therapist right away. Even a small amount of targeted exercise helps a lot with the symptoms.

Just one last thought. If your brother-in-law receives his DNA test results and turns out to have SBMA/Kennedy's Disease, and assuming your brother-in-law and your husband are brothers, then it is possible that your husband did not have ALS, but actually had SBMA/Kennedy's Disease. The two diseases have similar symptoms, and mistaking the diagnosis is very common even for experienced neurologists. The only way to tell for sure is the DNA test.

Good luck.

This message has been edited. Last edited by: Dan B,

Susan, you have been given some good advice by others in this post. The DNA test is about 99+% accurate. The test usually takes 4-6 weeks to be processed at a DNA lab.

Progression is dependent upon two things ... CAG repeats (the higher the quicker the onset) and the natural progression cycle of Kennedy's Disease. Once symptoms become apparent, the progression is fairly predictable in regards to time. What is not predictable is what symptoms he will experience. There is little uniformity in regards to symptoms. Some experience one thing while others might never experience that particular symptoms.