Gluten ataxia in perspective: epidemiology, genetic susceptibility and clinical characteristics

Abstract

We previously have described a group of patients with gluten sensitivity presenting with ataxia (gluten ataxia) and suggested that this disease entity may account for a large number of patients with sporadic idiopathic ataxia. We have therefore investigated the prevalence of gluten sensitivity amongst a large cohort of patients with sporadic and familial ataxia and looked at possible genetic predisposition to gluten sensitivity amongst these groups. Two hundred and twenty-four patients with various causes of ataxia from North Trent (59 familial and/or positive testing for spinocerebellar ataxias 1, 2, 3, 6 and 7, and Friedreich's ataxia, 132 sporadic idiopathic and 33 clinically probable cerebellar variant of multiple system atrophy MSA-C) and 44 patients with sporadic idiopathic ataxia from The Institute of Neurology, London, were screened for the presence of antigliadin antibodies. A total of 1200 volunteers were screened as normal controls. The prevalence of antigliadin antibodies in the familial group was eight out of 59 (14%), 54 out of 132 (41%) in the sporadic idiopathic group, five out of 33 (15%) in the MSA-C group and 149 out of 1200 (12%) in the normal controls. The prevalence in the sporadic idiopathic group from London was 14 out of 44 (32%). The difference in prevalence between the idiopathic sporadic groups and the other groups was highly significant (P < 0.0001 and P < 0.003, respectively). The clinical characteristics of 68 patients with gluten ataxia were as follows: the mean age at onset of the ataxia was 48 years (range 14-81 years) with a mean duration of the ataxia of 9.7 years (range 1-40 years). Ocular signs were observed in 84% and dysarthria in 66%. Upper limb ataxia was evident in 75%, lower limb ataxia in 90% and gait ataxia in 100% of patients. Gastrointestinal symptoms were present in only 13%. MRI revealed atrophy of the cerebellum in 79% and white matter hyperintensities in 19%. Forty-five percent of patients had neurophysiological evidence of a sensorimotor axonal neuropathy. Gluten-sensitive enteropathy was found in 24%. HLA DQ2 was present in 72% of patients. Gluten ataxia is therefore the single most common cause of sporadic idiopathic ataxia. Antigliadin antibody testing is essential at first presentation of patients with sporadic ataxia.

Effect of Therapeutic Dietary Regimen on Wound Healing For Patients with Moderate Degree Burn

“…Also it considered a good source of traces minerals like selenium and magnesium, nutrients essential to wound healing. Hadjivassiliou 2003 concluded that wholewheat grain supplies elements needed by the human body as Starch and gluten which provide heat and energy; phosphates and other mineral salts, vitamins B and E and protein that help in building and repairing of muscular tissue (52)(53)(54)(55)(56)(57) .As regard the relation between macro, micronutrient and hemoglobin level, serum albumin and total protein the present study illustrated that there was a positive significant relation between dietary protein intake and hemoglobin level, serum albumin and total protein. Due stated that heme iron absorption is less affected by substances in the diet than non-heme iron, indicating that heme iron absorption is determined mainly by the body iron levels and, to a very minor extent, by dietary factors (58)(59)(60)(61)(62)(63) .…”

Celiac Disease and Autoimmunity in the Gut and Elsewhere

“…nystagmus were reported most frequently. [56] Anti-gliadin antibodies cross-reacting with epitopes on cerebellar Purkinje fibers is suggested by the authors as the likely mechanism. Whether gluten ataxia exists in the absence of celiac disease is controversial.…”

“…Gluten ataxia is considered a distinct disease process which can occur with or without enteropathy and is associated with high anti-gliadin antibody titers. [56] Hadjivassiliou et al demonstrated a 32% prevalence of anti-gliadin antibodies among patients with sporadic idiopathic ataxia. 24% of this gluten ataxia group had evidence of gluten-sensitive enteropathy and only 13% reported gastrointestinal symptoms.…”

Neuropsychiatric Features of Celiac Disease

“…Clinical features include ocular signs of cerebellar dysfunction such as gaze evoked nystagmus in over 80% of cases, upper or lower limb ataxia, gait ataxia, and dysarthria [37,40,41]. MRI findings indicate atrophy of the cerebellum and white matter hyperintensities [40].…”

“…Clinical features include ocular signs of cerebellar dysfunction such as gaze evoked nystagmus in over 80% of cases, upper or lower limb ataxia, gait ataxia, and dysarthria [37,40,41]. MRI findings indicate atrophy of the cerebellum and white matter hyperintensities [40]. Hadjivassiliou et al [42] reported finding prevalent deposits of anti-tissue transglutaminase IgA antibodies in the brain, most notably in the medulla, pons and cerebellum.…”