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

• Published in: The Lancet (British edition) 2000-10, Vol.356 (9237), p.1224-1227
• Main Authors: Verity, CM, Nicoll, A, Will, RG, Devereux, G, Stellitano, L
• Format: Article
• Language: English
• Subjects: Adolescent; Age; Ataxia; Biological and medical sciences; Bovine spongiform encephalopathy; Child; Child, Preschool; Children; Childrens health; Cognition Disorders - epidemiology; Consent; Creutzfeldt-Jakob disease; Creutzfeldt-Jakob Syndrome - diagnosis; Creutzfeldt-Jakob Syndrome - epidemiology; Creutzfeldt-Jakob Syndrome - mortality; Degenerative and inherited degenerative diseases of the nervous system. Leukodystrophies. Prion diseases; Ethics; Female; Follow-Up Studies; Humans; Laboratories; Male; Mass Screening; Medical sciences; Nervous System Diseases - epidemiology; Neurodegenerative diseases; Neurological diseases; Neurology; Patients; Population Surveillance; Surveillance; Survival Rate; United Kingdom - epidemiology
• ISSN: 0140-6736; 1474-547X
• DOI: 10.1016/S0140-6736(00)02785-9

Variant Creutzfeldt-Jakob Disease (vCJD) was first reported in 1996; the youngest patient developed symptoms at 16 years of age. We have done 3 years of prospective active surveillance for progressive intellectual and neurological deterioration (PIND) in UK children, and have searched for vCJD among the children who were reported. Since May, 1997, there has been active surveillance for patients younger than 16 years old with PIND by means of a monthly card sent to all UK consultant paediatricians by the British Paediatric Surveillance Unit. Clinical details of cases of PIND are obtained from reporting paediatricians by telephone interview or site visit, and an expert group of paediatric neurologists then classifies the cases. After 3 years, 885 patients with suspected PIND have been reported. Among them were two fatal cases of definite vCJD and one case of probable vCJD; all were reported in 1999. One girl was age 12 years at onset–the youngest ever case of vCJD. No other children with the clinical features of vCJD were identified. The expert group has discussed 655 cases, of which 360 have a confirmed underlying cause, being categorised into 88 known neurodegenerative diseases. That this prospective active surveillance in the UK has found few children with suspected vCJD is relatively reassuring. However, 3 years is a short time to survey a disease with an unknown incubation period. Since one probable and two definite cases of vCJD were reported to the study in 1999, there is concern that more childhood cases may appear.