Simonati A, Battistella P A, Guariso G, Clementi M, Rizzuto N
Department of Neurological and Visual Sciences, University of Verona, Italy.
Neuropediatrics. 1998 Jun;29(3):155-8. doi: 10.1055/s-2007-973553.
Clinical features and pathological findings of sural nerve biopsy are reported of a 3-year-old child affected with coeliac disease who developed a progressive polyneuropathy, unresponsive to a gluten-free diet, nor to vitamin E and folic acid supply. There was no evidence of malabsorption, nor of blood factor deficiency, nor of metabolic abnormalities which might account for the neuropathy. A sural nerve biopsy showed marked loss of myelinated fibres without evidence of regenerative phenomena. Distal involvement and features of nerve pathology are consistent with a "dying-back" mechanism to underlie the nerve damage. The lack of response to a gluten-free diet suggests that direct toxicity of gliadin is not implicated.