The rare inherited disorder hyperprolinaemia type II presents with fits in
childhood, usually precipitated by infection. A diagnosis of hyperprolinaem
ia type II and vitamin B-6 deficiency was made in a well nourished child wi
th fits. It is thought that pyridoxine deficiency was implicated in her fit
s and was the result of inactivation of the vitamin by the proline metaboli
te, pyrroline-5-carboxylate.