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Variant Creutzfeldt-Jakob disease in UK children: a national surveillance study
C M Verity1, A Nicoll, R G Will
1PIND Surveillance Group, Addenbrooke's Hospital, Cambridge, UK.
Insights
This UK surveillance found few childhood variant Creutzfeldt-Jakob Disease (vCJD) cases, including the youngest ever. Continued monitoring is needed due to the unknown incubation period and recent vCJD diagnoses in children.
Area of Science:
- Neurology
- Epidemiology
- Pediatrics
Background:
- Variant Creutzfeldt-Jakob Disease (vCJD) emerged in 1996, with initial cases in adolescents.
- Understanding vCJD's impact on children is crucial for public health.
Purpose of the Study:
- To conduct prospective active surveillance for progressive intellectual and neurological deterioration (PIND) in UK children.
- To identify and investigate potential cases of vCJD among children with PIND.
Main Methods:
- A 3-year prospective surveillance system was implemented in the UK.
- Monthly cards were sent to UK consultant pediatricians to report PIND cases.
- An expert panel of pediatric neurologists reviewed and classified reported cases.
Main Results:
- 885 patients with suspected PIND were reported over 3 years.
- Two definite and one probable case of vCJD were identified, all reported in 1999.
- The youngest vCJD case on record, a 12-year-old girl, was identified; no other vCJD cases were found.
Conclusions:
- The low number of childhood vCJD cases identified is reassuring but requires further investigation.
- The unknown incubation period of vCJD necessitates continued surveillance.
- Recent vCJD diagnoses in children warrant ongoing vigilance for potential future cases.
Background:
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.
Methods:
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.
Findings:
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.
Interpretation:
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.
