Clinical features and pathological findings of sural nerve biopsy are
reported of a 3-year-old child affected with coeliac disease who devel
oped a progressive polyneuropathy, unresponsive to a gluten-free diet,
nor to vitamin E and folic acid supply. There was no evidence of mala
bsorption, nor of blood factor deficiency, nor of metabolic abnormalit
ies which might account for the neuropathy. A sural nerve biopsy showe
d marked loss of myelinated fibres without evidence of regenerative ph
enomena. Distal involvement and features of nerve pathology are consis
tent 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.