Variant Creutzfeldt-Jakob disease in UK children: a national surveillance study

Background Variant Creutzfeldt-Jakob Disease (vCJD) was first reported in 1 996; the youngest patient developed first reported in 1996; the youngest pa tient developed symptoms at 16 years of age. We have done 3 years of prospe ctive active surveillance for progressive intellectual and neurological det erioration (PIND) in UK children, and have searched for vCJD among the chil dren who were reported. Methods Since May, 1997, there has been active surveillance for patients yo unger than 16 years old with FIND by means of a monthly card sent to all UK consultant paediatricians by the British Paediatric Surveillance Unit. Cli nical details of cases of FIND are obtained from reporting paediatricians b y telephone interview or site visit, and an expert group of paediatric neur ologists then classifies the cases. Findings After 3 years, 885 patients with suspected FIND have been reported . Among them were two fatal cases of definite vCJD and one case, of probabl e vCJD; all were reported in 1999. One girl was age 12 years at onset-the y oungest ever case of vCJD. No other children with the clinical features of vCJD were identified. The expert group has discussed 655 cases, of which 36 0 have a confirmed underlying cause, being categorised into 88 known neurod egenerative diseases. Interpretation That this prospective active surveillance in the UK has foun d few children with suspected vCJD is relatively reassuring, However, 3 yea rs is a short time to survey a disease with an unknown incubation period. S ince one probable and two definite cases of vCJD were reported to the study in 1999, there is concern that more childhood cases may appear.