Abstract
Between May 1997 and November 2004 this national prospective surveillance study identified 1007 children with "progressive intellectual and neurological deterioration" (PIND). In most cases specific diagnoses were made, but of 92 undiagnosed children with PIND 46 had died and only four underwent full necropsy. There was no clinical evidence of variant Creutzfeldt-Jakob disease (vCJD) in these undiagnosed cases, but without necropsy it is not possible to exclude vCJD completely.