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Q: neurological disorders from celiacs disease ( Answered,   0 Comments )
Subject: neurological disorders from celiacs disease
Category: Health > Conditions and Diseases
Asked by: darcia-ga
List Price: $25.00
Posted: 08 Apr 2003 16:09 PDT
Expires: 08 May 2003 16:09 PDT
Question ID: 187942
Untreated celiacs disease can result in serious neurological
disorders, including loss of balance, progressing over time.  What is
this condition called, and what are it's symptoms and treatment?
Subject: Re: neurological disorders from celiacs disease
Answered By: kevinmd-ga on 08 Apr 2003 19:48 PDT
Hello - thanks for asking your question.

As you stated in your question, celiac disease may have as its primary
manifestation neuropsychiatric symptoms such as ataxia, depression,
anxiety, or epilepsy.  It has been suggested that the neurological
manifestations of celiac disease be termed as "gluten ataxia":

"BACKGROUND: Ataxia is the commonest neurological manifestation of
coeliac disease. Some individuals with genetic susceptibility to the
disease have serological evidence of gluten sensitivity without overt
gastrointestinal symptoms or evidence of small-bowel inflammation. The
sole manifestation of disease in such patients may be ataxia. We
describe the clinical, radiological, and neurophysiological features
of this disorder. METHODS: Patients with ataxia attending the
neurology outpatient clinics at the Royal Hallamshire Hospital,
Sheffield, UK, were screened for gluten sensitivity as shown by the
titre of antibody to gliadin. Those with other causes of ataxia were
excluded. We carried out clinical, neurophysiological,
neuroradiological, and, in two cases, neuropathological examinations.
FINDINGS: 28 patients with gluten ataxia were identified. All had gait
ataxia and most had limb ataxia. Those with more severe gait ataxia
had longer disease duration. No patient had tremor or other
extrapyramidal features. 19 patients showed some form of peripheral
neuropathy on neurophysiological examination. 16 patients had no
gastrointestinal symptoms. Distal duodenal biopsy showed lymphocytic
infiltration in two patients, and changes compatible with coeliac
disease in 11. Six patients had evidence of cerebellar atrophy on
magnetic-resonance imaging. Necropsy was done on two patients who
died; there was lymphocytic infiltration of the cerebellum, damage to
the posterior columns of the spinal cord, and sparse infiltration of
the peripheral nerves. INTERPRETATION: Gluten sensitivity is an
important cause of apparently idiopathic ataxia and may be
progressive. The ataxia is a result of immunological damage to the
cerebellum, to the posterior columns of the spinal cord, and to
peripheral nerves. We propose the term gluten ataxia to describe this
disorder." (3)

A study by Cronin (1998) suggests a possible connection between celiac
disease and epilepsy:
"Whether there is an association between coeliac disease and epilepsy
is uncertain. Recently, a syndrome of coeliac disease, occipital lobe
epilepsy and cerebral calcification has been described, mostly in
Italy. We measured the prevalence of coeliac disease in patients
attending a seizure clinic, and investigated whether cerebral
calcification occurred in patients with both coeliac disease and
epilepsy. Screening for coeliac disease was by IgA endomysial
antibody, measured by indirect immunofluorescence using sections of
human umbilical cord. Of 177 patients screened, four patients were
positive. All had small-bowel histology typical of coeliac disease.
The overall frequency of coeliac disease in this mixed patient sample
was 1 in 44. In a control group of 488 pregnant patients, two serum
samples were positive (1 in 244). Sixteen patients with both coeliac
disease and epilepsy, who had previously attended this hospital, were
identified. No patient had cerebral calcification on CT scanning.
Coeliac disease appears to occur with increased frequency in patients
with epilepsy, and a high index of suspicion should be maintained.
Cerebral calcification is not a feature of our patients with epilepsy
and coeliac disease, and may be an ethnically-or
geographically-restricted finding." (2)

The cornerstone of treatment of celiac disease is the elimination of
gluten in the diet. The principle sources of dietary gluten are wheat,
rye, and barley.

Studies show that a gluten free diet may improve the neurological
manifestations.  In a study by Hadjivassiliou (2001) where patients
had ataxia secondary to celiac disease, a majority had improvement of
their neurological symptoms after following a gluten free diet:
"The authors describe 10 patients with gluten sensitivity and abnormal
MRI. All experienced episodic headache, six had unsteadiness, and four
had gait ataxia. MRI abnormalities varied from confluent areas of high
signal throughout the white matter to foci of high signal scattered in
both hemispheres. Symptomatic response to gluten-free diet was seen in
nine patients." (4)

For those who do not respond to a gluten free diet, corticosteroids
and immunosupressants (azathioprine, intravenous cyclosporine and
6-mercaptopurine) have been used with varying results.  You may want
to discuss these options with your personal physician.

This answer is not intended as and does not substitute for medical   
advice - the information presented is for patient education only.   
Please see your personal physician for further evaluation of your   
individual case.   
Please use any answer clarification before rating this answer. I will
be happy to explain or expand on any issue you may have.         
Kevin, M.D.         

1) Schuppan, et. al. Pathogenesis, epidemiology, and clinical
manifestations of celiac disease.  UptoDate, 2002.
2) Cronin, CC, Jackson, LM, Feighery, C, et al. Coeliac disease and
epilepsy. QJM 1998; 91:303.
3) Hadjivassiliou, M, Grunewald, RA, Chattopadhyay, AK, et al.
Clinical, radiological, neurophysiological, and neuropathological
characteristics of gluten ataxia. Lancet 1998; 352:1582.
4) Hadjivassiliou, M, Grunewald, RA, Lawden, M, et al. Headache and
CNS white matter abnormalities associated with gluten sensitivity.
Neurology 2001; 56:385.

Search strategy:      
No internet search engine was used in this answer.  All sources are 
from physician-written and peer-reviewed sources.

Request for Answer Clarification by darcia-ga on 09 Apr 2003 09:31 PDT
Could progressive loss of balance, to the point of not being able to
stand upright without props, be a result of this cerebellum ataxia? 
And can celiacs be diagnosed through a blood test, and is the
screening difficult to obtain?

Clarification of Answer by kevinmd-ga on 09 Apr 2003 09:59 PDT
Hello - thanks for asking your question.  

The loss of balance can definitely be attributed to gluten ataxia. 
However, there are many, many other reasons for ataxia (i.e.
hereditary disease, enzyme deficiencies, infection, tumor etc.) and a
neurological evaluation should be undertaken to comprehensively
evaluate this.

Celiac disease can be diagnosed through serums blood tests.  The IgA
endomysial antibody test is most commonly performed and this could be
obtained through your primary care physician or gastroenterologist.

I will be happy to discuss the details of diagnosis and the various
causes of ataxia in seperate questions.

Kevin, M.D.
There are no comments at this time.

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