Neurological complications of celiac disease and autoimmune mechanisms: preliminary data of a prospective study in adult patients.

Celiac disease (CD) is an autoimmune disorder, affecting 0.3% to 1% of the population of the Western world. It is mediated by a T cell immune response against ingested gluten in genetically predisposed persons, with more than 95% of patients sharing the major histocompatibility complex class II human leukocyte antigen (HLA) DQ2 or DQ8 haplotype. Tissue transglutaminase (TG) is the antigenic target recognized by anti-endomysial antibodies in CD, and serum anti-TG antibodies are a specific marker for the disease. Autoimmune disorders and organ-specific autoantibodies may be associated, especially type I diabetes mellitus and autoimmune thyroiditis. Intestinal biopsy allows a definitive diagnosis, showing villous atrophy of the small intestinal mucosa, which improves after a gluten-free diet. Although the small bowel is often involved, approximately 50% of adult patients present with extraintestinal manifestations, mainly iron-deficiency anemia, but also osteoporosis, infertility, and neurological alterations. Among the latter, peripheral neuropathy and cerebellar ataxia are the most common.