Surveillance for progressive intellectual and neurological deterioration in the Canadian paediatric population

Area of Science:

• Neurology
• Epidemiology
• Public Health

Background:

• Creutzfeldt-Jakob disease (CJD) and its variant form (vCJD) are rare, fatal neurodegenerative disorders.
• Active surveillance is crucial for detecting and monitoring rare diseases, especially in vulnerable paediatric populations.
• Understanding the incidence of CJD and vCJD in children is important for public health preparedness.

Purpose of the Study:

• To implement an enhanced active surveillance system for progressive intellectual and neurological deterioration in Canadian children.
• To detect and report cases of Creutzfeldt-Jakob disease (CJD) and variant Creutzfeldt-Jakob disease (vCJD) in the paediatric population.
• To determine the occurrence of CJD and vCJD in children under 18 years of age.

Main Methods:

• An enhanced active surveillance system was established across Canada from July 1999 to July 2001.
• Paediatricians and paediatric neurologists received monthly reporting forms for eligible children (≤18 years) with >3 months of progressive intellectual/neurological decline.
• Reported cases were reviewed, classified, and assessed for potential CJD or vCJD by a principal investigator and a review panel.

Main Results:

• Over 2200 physicians participated, with an 80% monthly return rate.
• Ninety-nine possible cases of progressive neurological and intellectual deterioration were reported; 60 met the criteria.
• One case of classic Creutzfeldt-Jakob disease was identified; no cases of variant Creutzfeldt-Jakob disease were detected.

Conclusions:

• Active surveillance effectively identified cases of progressive neurological deterioration in Canadian children.
• Classic Creutzfeldt-Jakob disease is exceedingly rare in the Canadian paediatric population.
• The absence of variant Creutzfeldt-Jakob disease cases suggests a very low or non-existent risk in this demographic during the study period.