Celiac.com Sponsors:

Celiac.com Sponsor:
No popular authors found.
Ads by Google:
Celiac.com Sponsors:
 
Celiac.com Sponsor:

Categories

No categories found.


Get Celiac.com's E-Newsletter





Ads by Google:


Follow / Share


  FOLLOW US:
Twitter Facebook Google Plus Pinterest RSS Podcast Email  Get Email Alerts
SHARE:

Popular Articles

No popular articles found.
Celiac.com Sponsors:

Myoclonus Ataxia and Refractory Celiac Disease

Celiac.com 10/27/2014 - There have been a few reports tying cortical myoclonus with ataxia to celiac disease. Such reports also suggest that the former is unresponsive to a gluten-free diet.

Photo: Wikimedia Commons--Stan ZurekA team of researchers recently set out to determine if there is any significant connection between the two conditions. The research team included Ptolemaios G. Sarrigiannis, Nigel Hoggard, Daniel Aeschlimann, David S. Sanders, Richard A. Grünewald, Zoe C. Unwin, and Marios Hadjivassiliou.

They are variously associated with the Departments of Gastroenterology, Neurology, Neurophysiology and Neuroradiology at Royal Hallamshire Hospital, in Sheffield, UK, and with the College of Biomedical and Life Sciences at Cardiff University in Cardiff, UK.

The team presented detailed electro-clinical characteristics of a new syndrome of progressive cortical hyperexcitability with ataxia and refractory celiac disease. Regular follow ups of over 600 patients with neurological manifestations due to gluten sensitivity revealed 9 patients with this syndrome.

They found that all nine patients, six men and three women, experienced asymmetrical irregular myoclonus involving one or more limbs and sometimes face. This was often stimulus sensitive and became more widespread over time. Three patients had a history of Jacksonian march, and five had at least one secondarily generalized seizure. Electrophysiology showed evidence of cortical myoclonus. Three showed a phenotype of epilepsia partialis continua at onset.

Ads by Google:

All patients showed clinical, imaging and/or pathological evidence of cerebellar involvement. All patients followed a strict gluten-free diet, and most successfully eliminated gluten-related antibodies. However, all patients still showed evidence of enteropathy, suggests that refractory celiac disease is to blame.

During the study, two patients died from enteropathy-associated lymphoma and one from status epilepticus. Five patients were treated with mycophenolate and one in addition with rituximab and IV immunoglobulins. These patients showed improvement of ataxia and enteropathy, but continued to suffer the effects of myoclonus.

These results indicate that myoclonus ataxia might be the most common neurological manifestation of refractory celiac disease.

The clinical involvement, apart from ataxia, covers the whole clinical spectrum of cortical myoclonus.

Source:

Celiac.com welcomes your comments below (registration is NOT required).










Related Articles



1 Response:

 
Irv
Rating: ratingfullratingfullratingfullratingfullratingfull Unrated
said this on
08 Nov 2014 5:18:35 AM PDT
Very interesting.




Rate this article and leave a comment:
Rating: * Poor Excellent
Your Name *: Email (private) *:




In Celiac.com's Forum Now:


The Bakery On Main story began in the back of Owner, Michael Smulders' Natural Food Store in Glastonbury, CT. Many of his customers with Celiac Disease would visit the bakery and mention the lack of good-tasting, gluten-free options available to them. Upon learning this, Michael Smulders believed...

I used to drink Guinness, sometimes a half-n-half over Harp or Bass. I call Sam Adams a good light beer, and probably gave up on Budwiper and the like 30 years ago. So while it is nice to find companies making lots of "Yeah, that almost tastes like cheapo beer"....There's not a one of them that c...

Megan- I am not Catholic but I owe my gluten awareness to whoever was the Pope in 2004. There was a BIG piece in the national nooze media about some Roman Catholic girl in NJ who had celiac. Her parents were told by her priest and bishop that she should take the communion wafer anyway, and ...

....yeah that is the sad story of many of us...I think I went through what was it 6 doctors? or was it 7. I ran into one JUST like that 4 doctors ago. She was very condescending, and this was before my celiac dia. She practically worshiped the whole eat all whole grains, avoid dairy lifestyle. Sh...

I've had it twice and it was served to me different each time. The first time it was served warm in a sealed bag. The second time it was served warm, not in a sealed bag, but on their traditional paper. I should have refused the possible contaminated order, but I was hungry and in a hurry so I ac...