One of my sisters has been diagosed with this condition that is incurable. It is simply a shrinking of the cerebellum and it destroys motor functions, slurs speech, later on it will not allow her to swallow. They say it is similar to Lou Gehrigs disease. She has been battling it for a number of years until she was diagnosed at the Mayo Clinic this past June. They have no idea how progressive it is; I guess each person is different. She is in assisted living as she wants to be as independent as possible for as long as she can. She is already walking with a walker and her speech is definitely slurred. She has to think really hard to form her words. My heart just aches for her. She keeps her spirits up and has been told she has added a lot already to their community at the assisted living facility. I just wonder if anyone has heard of this condition and if you would have anymore information about it. Mostly its progression. Thank, Cathy

Hi Cathy,

I am so sorry to hear about your sister.

There is one thing I hope the doctors have considered, and that is gluten sensitivity as a possible cause for cerebellar ataxia (intolerance to gluten found in wheat, rye, barley). Some studies say it may be responsible for up to 40% of idiopathic cerebellar ataxia, and possibly even some forms of hereditary ataxia.

Do you know if your sister has been tested specifically for antigliadin antibodies? The reason this is so important is that some people with ataxia and antigliadin antibodies have improved on a gluten free diet. Unfortunately, sometimes existing damage may be permanent, but the progression may be slowed or stopped with gluten free diet. The studies can be found below.

Much of the research on this has been done by Dr. Marios Hadjivassiliou from the UK , but there are others who have found similar results. I know that Dr. Hadjivassiliou works with other doctors who have contacted him, so if your sister's doctors needed to know more in regard to appropriate testing they could try to contact him.

In particular, you may want to look at these articles of his:

Autoantibody targeting of brain and intestinal transglutaminase in gluten ataxia, Feb 2006

Cerebellar abnormalities on proton MR spectroscopy in gluten ataxia, July 2005 - free full text

Dietary treatment of gluten ataxia. Sept 2003 - free full text

Gluten ataxia in perspective: epidemiology, genetic susceptibility and clinical characteristics. Mar 2003 - free full text

The humoral response in the pathogenesis of gluten ataxia. Apr 2002

Clinical, radiological, neurophysiological, and neuropathological characteristics of gluten ataxia. Nov 1998

Gluten sensitivity as a neurological illness. May 2002 - free full text

The Neurology of Gluten Sensitivity: Science vs. Conviction -
Choreic syndrome and coeliac disease: a hitherto unrecognised association, Apr 2004



Also check out these pages of The Gluten File:

The Neurological Manifestations of Gluten Sensitivity

Gluten Ataxia

This may be a long shot~ but I just want to be sure that the doctors have considered this....because it may provide for a glimmer of hope. Here is a page about Diagnostic Testingfor celiac disease/ gluten sensitivity... but I would recommend contacting Dr. H. directly because I know there are other tests he'd likely recommend in regard to gluten ataxia.

Cara

I'm sorry too.

Please do pay close attention to the information in Cara's post. Gluten sensitivity and B12 malabsorption are probably two of the most underdiagnosed conditions, and it isn't dangerous or very costly to cover both.

rose

Thanks, Rose, for the specific mention of B12 deficiency. How could I have neglected to mention that ? Vitamin E and CoQ10 deficiencies are also associated with cerebellar ataxia, and googling cerebellar ataxia with any of these should bring up lots of supporting data. Now, one would hope that the good doctors at Mayo would have considered these things, but you never know, and lab tests are not 100% accurate in determining underlying deficiencies. We know from personal experience how often B12 deficiency goes unrecognized for so many reasons. I'd want to be sure I had strong levels of all of these.

Cara

You gals are so great, unbelievable really. I heard back from my sister this morning and she as well was so thankful for your kindness. However, she and her doctor, who was part of Dr. Andrew Weil's Integrative Medicine group at the University of AZ, (pretty impressive I thought), decided that more testing was not the right way to go as she struggled with this 3-4 years before diagnosis. She has pages of testing from Mayo so she might have been tested, she's not sure. I'd like to share her words as she really touched me with them, and, I suppose each of our journeys will take us one day to the point where she is: "My body can't take any more uphill climbs above and beyond the ones I already face each day. If I allow my body what it needs each day, my body reciprocates with the best it can each day. We are content with each other -- my body and I -- and my quality of life in Maria Joseph manor is good -- the best possible outcome I believe." So I think we now just hope and pray for a lot of good days and the strength to live the best we all can.
Thank you once again.
Gratefully,
Cathy

Hello. I am new to this site. Last year my father starting having problems with vision in his left eye. The eye doctor said it looked like a stroke behind the eye. Just as he was beginning to cope with that his speech became slurred and he would stumble around like he was drunk. He said his leg at times feels like it was not even a part of his body, but rater something heavy to carry around. He said that he is not actually dizzy, but feels unsteady. Also, his speech at times is very hard to understand. This past weekend he had a couple of episodes where he could not talk. Finally, drinking liquids (mainly water) chokes him. We too him to UAMS a diagnostic hospital/clinic. They ran all kinds of tests. They could not find anything. At first they were sure it was Lou Gherics, but then they decided it was a shrinking cerebellum. That too they could not say conclusively without a brain biopsy which my father has opted not to do. I began reading some of the posts here and he is wanting to try the enzyme to see if it helps with his unsteadiness. Plus we are looking for information about the gluten free diet. My questions are, what mg do you take of the CoQ10? I know the results take a while, but how long did it take before you started noticing a difference? Any help, advice, etc would be appreciated.

The patients with ataxia, take very high doses of CoQ-10. This nutrient has absorption problems, so some companies have special forms that they claim are more potent.

In this study, suggested doses were really high 600mg 4 times a day.
http://clinicaltrials.gov/ct2/show/NCT00957216

This study showed both low dose and high dose worked:
from http://www.ncbi.nlm.nih.gov/pubmed/19049556

The Tishcon company is often used by doctors and this place sells it. Its higher quality means you don't need gram range dosing but it is more pricey than others.
I'd start at the 60mg twice a day and increase if needed over time.
http://www.epic4health.com/noname.html

There are other makers, one is Doctor's Best... but it has Bioperine added to increase absorption. Bioperine may interfere with drug therapies if your father takes prescription drugs. So I would not use this type at this time.

Non Tischon CoQ-10 will need high doses, at least 300mg once or twice a day to start, and see if you need to move higher, say every 2wks-4wks. Increase slowly and give it time to work. This may save you money. CoQ-10 is not a toxic substance, so only your pocket book is a factor for most people.

This monograph may be helpful:
http://lpi.oregonstate.edu/infocenter/othernuts/coq10/
There are newer versions of CoQ-10 called Ubinquinol. These allow lower dosing, but are much more costly. They claim better absorption.

Cara, I am grateful that I don't suffer from this disease. Just wanted to commend you for providing so much useful information. In fact, it would be good if your posting were made a Sticky to prevent it from getting lost in the archives.


Gary

Hello Cathy,
I have no idea where to begin. My name is Linda and I care for my hubby Mike. See 6+ yrs ago my Mike became very ill he was only 47yrsold. At first he lost his sight(back in about 3 min) got a Real bad headache and started to sweat. To the hospital we went after church.They said he had a bad headache LOL. Then our crazy life began. Neuro dr1 said it was in his head and depression, 3 months of that and I was ready to kill the dr.. Neuro dr2 said depression,we never went back.. Neuro dr3 and many more test said Binswanger disease and strokes and gave him 5yrs to live..Then 1yr later said nope thats not it either,migraines and depression.. Neuro dr4 saw him for 1yr and knew if she did not figger it out fast he was going to die.. Our dr contacted the dr the other people talked about in here Dr H. He told her what test to do.. And 4yrs to the day when Mike got sick we Finely had the answer Gluten Ataxia... The wheel chair is in the attic(not the car). No more walker,balance is better. Short term memory is ok.. No more stroke like episodes.. Mikes TTG was 17.1 Dec 2005,, 11.8 Sept 2005,, 11.5 April 2006,,
6.40 March 2007.. TTG is a gluten blood test..It checks for antibodies in your blood.. He is on a very strike Gluten free diet.... Our story was in the magazine called Living Without winter issue(Dec2006)...Feel free to email me.. at mhowell3@rochester.rr.com
Take care ..Linda and Mike

Happily, B12 testing is not necessary. Whether tested or not, and whatever test results might have been, at least 1000 mcg per day B12 (not extended release) should be taken by anyone with symptoms, just in case. It is safe and inexpensive.

rose