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I have been getting a few inquiries about "What is gluten ataxia?" in
response to my post "Dietary Treatment of Gluten Ataxia". The following
might help might help. Links are provided for 2 free full-text articles.
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http://internaf.org/ataxia.html
"What is ataxia?
Ataxia is a symptom, not a specific disease or diagnosis. Ataxia means
clumsiness, or loss of coordination. Ataxia may affect the fingers and
hands, the arms or legs, the body, speech or eye movements. This loss of
coordination may be caused by a number of different medical or neurologic
conditions; for this reason, it is important that a person with ataxia seek
medical attention to determine the underlying cause of the symptom and to
get the appropriate treatment."
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Brain. 2003 Mar;126(Pt 3):685-91.
Gluten ataxia in perspective: epidemiology, genetic susceptibility and
clinical characteristics.
Hadjivassiliou M, Grunewald R, Sharrack B, Sanders D, Lobo A, Williamson C,
Woodroofe N, Wood N, Davies-Jones A.
Department of Neurology, The Royal Hallamshire Hospital, Sheffield, UK.
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We previously have described a group of patients with gluten sensitivity
presenting with ataxia (gluten ataxia) and suggested that this disease
entity may account for a large number of patients with sporadic idiopathic
ataxia. We have therefore investigated the prevalence of gluten sensitivity
amongst a large cohort of patients with sporadic and familial ataxia and
looked at possible genetic predisposition to gluten sensitivity amongst
these groups. Two hundred and twenty-four patients with various causes of
ataxia from North Trent (59 familial and/or positive testing for
spinocerebellar ataxias 1, 2, 3, 6 and 7, and Friedreich's ataxia, 132
sporadic idiopathic and 33 clinically probable cerebellar variant of
multiple system atrophy MSA-C) and 44 patients with sporadic idiopathic
ataxia from The Institute of Neurology, London, were screened for the
presence of antigliadin antibodies. A total of 1200 volunteers were
screened as normal controls. The prevalence of antigliadin antibodies in
the familial group was eight out of 59 (14%), 54 out of 132 (41%) in the
sporadic idiopathic group, five out of 33 (15%) in the MSA-C group and 149
out of 1200 (12%) in the normal controls. The prevalence in the sporadic
idiopathic group from London was 14 out of 44 (32%). The difference in
prevalence between the idiopathic sporadic groups and the other groups was
highly significant (P < 0.0001 and P < 0.003, respectively). The clinical
characteristics of 68 patients with gluten ataxia were as follows: the mean
age at onset of the ataxia was 48 years (range 14-81 years) with a mean
duration of the ataxia of 9.7 years (range 1-40 years). Ocular signs were
observed in 84% and dysarthria in 66%. Upper limb ataxia was evident in
75%, lower limb ataxia in 90% and gait ataxia in 100% of patients.
Gastrointestinal symptoms were present in only 13%. MRI revealed atrophy of
the cerebellum in 79% and white matter hyperintensities in 19%. Forty-five
percent of patients had neurophysiological evidence of a sensorimotor
axonal neuropathy. Gluten-sensitive enteropathy was found in 24%. HLA DQ2
was present in 72% of patients. Gluten ataxia is therefore the single most
common cause of sporadic idiopathic ataxia. Antigliadin antibody testing is
essential at first presentation of patients with sporadic ataxia.
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J Neurol Neurosurg Psychiatry. 2002 May;72(5):560-3.
Gluten sensitivity as a neurological illness.
Hadjivassiliou M, Grunewald RA, Davies-Jones GA.
Free full-text file:
http://jnnp.bmjjournals.com/cgi/content/full/72/5/560
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Brain. 2001 May;124(Pt 5):1013-9.
Free full-text file:
http://brain.oupjournals.org/cgi/content/full/124/5/1013
Sporadic cerebellar ataxia associated with gluten sensitivity.
Burk K, Bosch S, Muller CA, Melms A, Zuhlke C, Stern M, Besenthal I, Skalej
M, Ruck P, Ferber S, Klockgether T, Dichgans J.
Department of Neurology, University of Tubingen, Germany. buerk@uni-
tuebingen.de
A total of 104 patients with sporadic cerebellar ataxia were tested for
antigliadin and antiendomysium antibodies. Twelve individuals (11.5%) with
gluten sensitivity underwent duodenal biopsy and extensive clinical,
electrophysiological, neuropsychological, radiological and laboratory
investigations including human leucocyte antigen (HLA) typing. Two patients
showed typical changes of gluten-sensitive enteropathy with crypt
hyperplasia and mucosal flattening. In five patients, the intraepithelial
lymphocyte count was elevated. Sporadic ataxia with gluten sensitivity was
found to be tightly linked to the HLA DQB1*0201 haplotype (70%).
Neurological symptoms were not related to hypovitaminosis or inflammatory
CSF changes. The clinical syndrome was dominated by progressive cerebellar
ataxia with ataxia of stance and gait (100%), dysarthria (100%) and limb
ataxia (97%). Oculomotor abnormalities were gaze-evoked nystagmus (66.7%),
spontaneous nystagmus (33.3%), saccade slowing (25%) and upward gaze palsy
(16.7%). Extracerebellar features also included deep sensory loss (58.3%),
bladder dysfunction (33.3%) and reduced ankle reflexes (33.3%). In
accordance with clinical findings, electrophysiological investigations
revealed prominent axonal neuropathy with reduced amplitudes (50%) and
abnormal evoked potentials (58.3%). On neuropsychological testing, patients
presented with moderate verbal memory and executive dysfunction. All
patients had evidence of cerebellar atrophy on MRI. We conclude that
sporadic ataxia may be associated with positive antibodies against gliadin.
Nevertheless, mucosal pathology does not represent an obligatory condition
of ataxia with gluten sensitivity. The fact that the disease is strongly
associated with the same HLA haplotypes found in coeliac disease not only
demonstrates coeliac disease and ataxia with gluten sensitivity to be part
of the same disease entity but supports the hypothesis of an immunological
pathogenesis of cerebellar degeneration.
* * *
* Visit the Celiac Web Page at www.enabling.org/ia/celiac/index.html *
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