Gluten ataxia is a relatively common central nervous system manifestation of celiac disease and is usually encountered in individuals who do not have overt gastrointestinal symptoms.
Gluten ataxia is encountered in both pediatric and adult celiac populations. It is a fairly common cause of all sporadic ataxias 2.
Gluten ataxia presents with cerebellar ataxia, sometimes with concurrent sensory ataxia, primarily affecting the lower limbs and gait 2,3. A less common manifestation is ataxia with myoclonus 3.
Gastrointestinal symptoms are usually absent, found in only 10% of individuals, and on duodenal biopsy, a diagnosis of celiac disease can be made in on half of patients 2. As such, the diagnosis of gluten ataxia requires screening for antibodies associated with celiac disease: anti-gliadin, anti-EMA, anti-TG2, and anti-TG6 antibodies 2.
There is some evidence that gluten-dependent transglutaminase 6 (TG6) autoantibodies react against cells within the cerebellum 1.
The main imaging finding is that of cerebellar atrophy that is usually gradual but in some cases can be rapid 3.
Treatment and prognosis
Treatment is primarily with strict adherence to a gluten-free diet. In many individuals, objective and subjective improvement in ataxia can be demonstrated 2.
- 1. Laurikka P, Nurminen S, Kivelä L, Kurppa K. Extraintestinal Manifestations of Celiac Disease: Early Detection for Better Long-Term Outcomes. (2018) Nutrients. doi:10.3390/nu10081015 - Pubmed
- 2. Panagiotis Zis, Marios Hadjivassiliou. Treatment of Neurological Manifestations of Gluten Sensitivity and Coeliac Disease. (2019) Current Treatment Options in Neurology. 21 (3): 10. doi:10.1007/s11940-019-0552-7 - Pubmed
- 3. Hadjivassiliou M, Grünewald RA, Davies-Jones GA. Gluten sensitivity as a neurological illness. (2002) Journal of neurology, neurosurgery, and psychiatry. 72 (5): 560-3. doi:10.1136/jnnp.72.5.560 - Pubmed