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Gluten Ataxia in Children and Adolescents
Ala Fadilah1, Daniel Connolly2, Marios Hadjivassiliou3
1Department of Paediatric Neurology, Sheffield Children's Hospital NHS Foundation Trust Ryegate Children's Centre, Sheffield, S10 5DD, UK.
Abstract:
Neurological manifestations of gluten sensitivity (GS) with gluten ataxia (GA) are well-recognised in adults. We present our experience in children and adolescents (C/A) seen with suspected GS, mainly GA, over a 5-year period. Retrospective case note analysis was performed on clinical, serological andneuroimaging data of 20 C/A seen in our specialist ataxia referral centre withsuspected NGS. 14 female and 6 male C/A (range: 6-15 years, median: 12.5 years) were referred to our specialist ataxia clinic with suspected GS. All 20 patients had motor coordination/balance difficulties with ataxia. Headaches were present in 12/20 C/A (60%), sensory neuropathic symptoms in 6/20 C/a (30%), postural dizziness in 6/20 (30%). 8/20 C/A (40%) had pre-existing autism spectrum disorder (ASD). Family history of GS/GA/Coeliac disease was present in 10/20 C/A (50%). Serological abnormalities were present in all 20 CYP, with raised antibodies: antigliadin in 15/20 (75%), transglutaminase-6 in 12/20 (60%), transglutaminase-2 in 7/20 (35%), and endomysial in 4/20 (20%). MR Spectroscopy of the cerebellum showed a reduced N-acetylaspartate: Creatine (NAA/Cr) ratio in 14/20 (70%). 18/20 (90%) received a gluten-free diet (GFD). 13/18 (72%) of C/A reported improvement of symptoms on GFD. We suggest considering GS as a differential diagnosis in the presence of motor coordination difficulties with abnormal MRS of the cerebellum and raised antigliadin and/or transglutaminase-6 antibodies. Early diagnosis of GS is important, as it is a treatable cause of childhood ataxia.Further follow-up will help in defining the natural history and GS in C/A.
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