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Abstract
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.