Objective: Gluten sensitivity is associated with multiple neurological abnormalities including gluten ataxia, motor neuron diseaselike neuropathy, small fiber type neuropathy, cognitive impairment, and even parkinsonism. We investigated whether or not gluten sensitivity is involved in Japanese patients with idiopathic cerebellar ataxia with extracerebellar presentation.
Patients or Materials: Fourteen patients with idiopathic cerebellar ataxia with extracerebellar presentation (autonomic instability, parkinsonism, or pyramidal dysfunction in varying combinations) were screened for antigliadin antibodies (AGA) to analyze for the presence or absence of gluten sensitivity. Patients with typical MR findings of multiple system atrophy of the cerebellar type were excluded. As disease controls without cerebellar ataxia, 9 patients with Parkinsons disease and 18 patients with amyotrophic lateral sclerosis were screened for AGA. Fortyseven normal controls were also screened for AGA.
Results: We found a high prevalence of AGA in 5 (36%) of 14 cerebellar ataxia patients, but in only 1 (4%) of 27 disease controls without cerebellar ataxia (odds ratio, 14.4; 95% CI, 1.41147; p<0.05) and in only 1 (2%) of 47 normal controls (odds ratio, 25.6; 95% CI, 2.66246; p<0.001). Among the cerebellar ataxia patients, atypical features such as sensorimotor neuropathy and/or mild cognitive impairment were more prevalent in the AGApositive group (60%) than in the AGAnegative group (0%). In one of the ataxic patients with AGA, a glutenfree diet had positive effects on neurological symptoms and nutritional status.
Conclusion: Gluten sensitivity is involved in at least some of the unexplained neurological symptoms of Japanese patients with adultonset, sporadic cerebellar ataxia.