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TOPLINE:

A 27-year active surveillance study of 5222 UK children identified six children with variant Creutzfeldt-Jakob disease (vCJD); the last two children developed symptoms in 2000 and died in 2003.

METHODOLOGY:

• This active prospective epidemiologic study was conducted through the British Paediatric Surveillance Unit to monitor children with progressive intellectual and neurologic deterioration (PIND) in the United Kingdom.
• Clinical data were collected using questionnaires or telephonic interviews with the child's paediatrician or through site visits.
• The PIND study expert group provided summaries of clinical details and investigations for each notified child with PIND and identified children who fulfilled the criteria for definite or probable vCJD.
• A total of 5222 children were notified between May 1997 and April 2024. 

TAKEAWAY:

• Overall, the 5222 children were categorised into four groups: "Not cases" (n = 2540) — those who did not meet the case definition or notification errors were present; PIND with diagnosis (n = 2367) — those who had a known underlying diagnosis other than vCJD; undiagnosed children (n = 309) — those who met the criteria for PIND but had no known diagnosis; and children with vCJD (n = 6).
• Of the six children with vCJD, two were male children and four were female children who developed symptoms between 1998 and 2000 and were aged between 12 and 15 years.
• The earliest symptoms were behavioural or psychiatric. Abnormal neurologic signs included ataxia, dysarthria, abnormal movements (chorea and dystonia), myoclonic jerks, variable tone in the limbs, and others.
• Four children were classified as those with definite vCJD and two children classified as those with probable vCJD. All six children died: Three in 2000, one in 2001, and two in 2003.

IN PRACTICE:

"In the absence of a reliable screening test for vCJD, the PIND study has been the only means of searching for vCJD cases in children. It has provided reasonable certainty that vCJD cases have not been missed among the many rare neurodegenerative diseases of childhood," the authors wrote.

SOURCE:

This study was led by Christopher Verity, Addenbrooke's Hospital, Cambridge, England. It was published online on April 02, 2025, in Archives of Disease in Childhood.

LIMITATIONS:

Notified patients were not followed up by the study after a diagnosis was made. vCJD might have been hidden among the undiagnosed children. PIND cases might have been underreported.

DISCLOSURES:

This study was funded by the National Institute for Health and Care Research Policy Research Programme. The authors did not report any conflicts of interest.

This article was created using several editorial tools, including AI, as part of the process. Human editors reviewed this content before publication.