PT  - JOURNAL ARTICLE
AU  - McKeon, Andrew
AU  - Lennon, Vanda A.
AU  - Pittock, Sean J.
AU  - Kryzer, Thomas J.
AU  - Murray, Joseph
TI  - The neurologic significance of celiac disease biomarkers
AID  - 10.1212/WNL.0000000000000970
DP  - 2014 Nov 11
TA  - Neurology
PG  - 1789--1796
VI  - 83
IP  - 20
4099  - http://n.neurology.org/content/83/20/1789.short
4100  - http://n.neurology.org/content/83/20/1789.full
SO  - Neurology2014 Nov 11; 83
AB  - Objective: To report neurologic phenotypes and their etiologies determined among 68 patients with either (1) celiac disease (CD) or (2) no CD, but gliadin antibody positivity (2002–2012).Methods: Neurologic patients included both those with the CD-prerequisite major histocompatibility complex class II human leukocyte antigen (HLA)-DQ2/DQ8 haplotype, and those without. The 3 groups were as follows: group 1 (n = 44), CD or transglutaminase (Tg)-2/deamidated gliadin immunoglobulin (Ig)A/IgG detected; group 2 (n = 15), HLA-DQ2/DQ8 noncarriers, and gliadin IgA/IgG detected; and group 3 (n = 9), HLA-DQ2/DQ8 carriers, and gliadin IgA/IgG detected. Neurologic patients and 21 nonneurologic CD patients were evaluated for neural and Tg6 antibodies.Results: In group 1, 42 of 44 patients had CD. Neurologic phenotypes (cerebellar ataxia, 13; neuropathy, 11; dementia, 8; myeloneuropathy, 5; other, 7) and causes (autoimmune, 9; deficiencies of vitamin E, folate, or copper, 6; genetic, 6; toxic or metabolic, 4; unknown, 19) were diverse. In groups 2 and 3, 21 of 24 patients had cerebellar ataxia; none had CD. Causes of neurologic disorders in groups 2 and 3 were diverse (autoimmune, 4; degenerative, 4; toxic, 3; nutritional deficiency, 1; other, 2; unknown, 10). One or more neural-reactive autoantibodies were detected in 10 of 68 patients, all with autoimmune neurologic diagnoses (glutamic acid decarboxylase 65 IgG, 4; voltage-gated potassium channel complex IgG, 3; others, 5). Tg6-IgA/IgG was detected in 7 of 68 patients (cerebellar ataxia, 3; myelopathy, 2; ataxia and parkinsonism, 1; neuropathy, 1); the 2 patients with myelopathy had neurologic disorders explained by malabsorption of copper, vitamin E, and folate rather than by neurologic autoimmunity.Conclusions: Our data support causes alternative to gluten exposure for neurologic dysfunction among most gliadin antibody–positive patients without CD. Nutritional deficiency and coexisting autoimmunity may cause neurologic dysfunction in CD.AChR=acetylcholine receptor; AQP4=aquaporin-4; CD=celiac disease; GAD65=glutamic acid decarboxylase 65; HLA=human leukocyte antigen; ICD-9=International Classification of Diseases, ninth revision; Ig=immunoglobulin; NMO=neuromyelitis optica; OD=optical density; Tg=transglutaminase; VGKC=voltage-gated potassium channel