Gluten sensitivity in Japanese patients with adult-onset cerebellar ataxia.
OBJECTIVE
Gluten sensitivity is associated with multiple neurological abnormalities including gluten ataxia, motor neuron disease-like 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.


METHODS
Fourteen patients with idiopathic cerebellar ataxia with extracerebellar presentation (autonomic instability, parkinsonism, or pyramidal dysfunction in varying combinations) were screened for anti-gliadin 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 Parkinson's disease and 18 patients with amyotrophic lateral sclerosis were screened for AGA. Forty-seven 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 AGA-positive group (60%) than in the AGA-negative group (0%). In one of the ataxic patients with AGA, a gluten-free diet had positive effects on neurological symptoms and nutritional status.


CONCLUSIONS
Gluten sensitivity is involved in at least some of the unexplained neurological symptoms of Japanese patients with adult-onset, sporadic cerebellar ataxia.
Ihara
Masafumi
M
Department of Neurology, Kyoto University Graduate School of Medicine, Kyoto.
Makino
Fumi
F
Sawada
Hideyuki
H
Mezaki
Takahiro
T
Mizutani
Kotaro
K
Nakase
Hiroshi
H
Matsui
Makoto
M
Tomimoto
Hidekazu
H
Shimohama
Shun
S
D017668:Age of Onset; D000368:Aged; D000369:Aged, 80 and over; D000906:Antibodies / Q000032:analysis; D002446:Celiac Disease / Q000150:complications* / Q000175:diagnosis; D002524:Cerebellar Ataxia / Q000150:complications / Q000276:immunology*; D005260:Female; D005903:Gliadin / Q000276:immunology; D005983:Glutens / Q000276:immunology*; D006801:Humans; D007070:Immunoglobulin A / Q000032:analysis; D008297:Male; D008875:Middle Aged; D020734:Parkinsonian Disorders / Q000276:immunology
D000906:Antibodies; D007070:Immunoglobulin A; D005983:Glutens; D005903:Gliadin