Abstract
Background: Patients with gluten ataxia (GA) without enteropathy have lower levels of antigliadin antibodies (AGA) compared to patients with coeliac disease (CD). Magnetic Resonance Spectroscopy (NAA/Cr area ratio) of the cerebellum improves in patients with GA following a strict gluten-free diet (GFD). This is associated with clinical improvement. We present our experience of the effect of a GFD in patients with ataxia and low levels of AGA antibodies measured by a commercial assay. Methods: Consecutive patients with ataxia and serum AGA levels below the positive cut-off for CD but above a re-defined cut-off in the context of GA underwent MR spectroscopy at baseline and after a GFD. Results: Twenty-one consecutive patients with GA were included. Ten were on a strict GFD with elimination of AGA, 5 were on a GFD but continued to have AGA, and 6 patients did not go on a GFD. The NAA/Cr area ratio from the cerebellar vermis increased in all patients on a strict GFD, increased in only 1 out of 5 (20%) patients on a GFD with persisting circulating AGA, and decreased in all patients not on a GFD. Conclusion: Patients with ataxia and low titres of AGA benefit from a strict GFD. The results suggest an urgent need to redefine the serological cut-off for circulating AGA in diagnosing GA.
Highlights
Gluten ataxia (GA) is defined as otherwise idiopathic sporadic ataxia with serological evidence of gluten sensitivity in the absence of an alternative cause [1]
The N-acetyl aspartate (NAA)/Cr area ratio taken from the cerebellar vermis increased in all 10 patients on a strict diet, but in only 1 out of 5 (20%) patients on a partial gluten-free diet (GFD) with persistent circulating antigliadin antibodies (AGA)
The current study demonstrates that in patients with gluten ataxia (GA) with low titres of AGA, NAA/Cr area ratio within the cerebellar vermis improves with strict adherence to a gluten-free diet, worsens with on-going exposure to gluten, and largely worsens with partial adherence to a gluten-free diet, as indicated by persistently positive circulating
Summary
Gluten ataxia (GA) is defined as otherwise idiopathic sporadic ataxia with serological evidence of gluten sensitivity in the absence of an alternative cause [1]. Up to 50% of patients with GA do not have an enteropathy, yet they still benefit from a gluten-free diet (GFD) [2]. For this reason, IgG and IgA native antigliadin antibodies (AGA) are currently the most sensitive marker for GA when compared to endomysium (EMA) and transglutaminase 2 antibodies (TG2), both of which are specific for the presence of enteropathy (Coeliac Disease-CD) [2]. Spectroscopy (NAA/Cr area ratio) of the cerebellum improves in patients with GA following a strict gluten-free diet (GFD).
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