Hypoglycaemia unawareness in a young boy with insulin-dependent diabetes mellitus and anti-adrenal medullary antibodies.

The main purpose of intensive insulin therapy in insulin-dependent diabetes mellitus is to prevent complications by maintaining short- and long-term euglycaemia. This therapeutic approach implies a high frequency of hypoglycaemic events which can be disturbing when hypoglycaemia unawareness is involved. Although this phenomenon can be reversed, it may lead to discontinuance of intensive insulin therapy. This paper concerns the case of a young insulin-dependent patient with early onset diabetes who had frequent severe occurrences of hypoglycaemia with convulsions, hypothermia, bradycardia and coma. These events were associated with hypoglycaemia unawareness not due to autonomic neuropathy. A defect in counterregulation was demonstrated during hypoglycaemic-hyperinsulinaemic clamp, expressed as a reduction of adrenaline response. A positive pattern of anti-adrenal medullary antibodies suggested that a functional defect of the adrenal medulla plays a role in the pathogenesis of hypoglycaemia unawareness.