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Perspective on the paper by Verity et al (see page608)
Dr Verity and his colleagues publish the results of their continuing study of progressive intellectual and neurological deterioration (PIND) in childhood1. The most surprising aspect of this study is the low rate of necropsies in this carefully followed group of children; only four of the 46 deaths were examined after death. This is an obstacle to detection of variant CJD (vCJD). Although most of this group of children had undergone investigations in life that allow a “probable” diagnosis to be made in the adult population, detailed neuropathological study remains the only way to confirm this diagnosis. Although the annual incidence of new cases of variant CJD has declined overall since 1999, more onsets have been observed in 2004 than in 2003, suggesting that it is premature to assume that this disease will soon disappear.2 Furthermore, the age specific incidence of variant CJD has remained essentially unchanged since the disorder was first described in 1996, reinforcing the need for continued paediatric surveillance.
While this low necropsy rate has serious implications for public health and for understanding of the epidemiology of vCJD, there are far wider implications. PIND is heterogeneous and 92 children (almost 10% of the study group) had no diagnosis; half of these died. They never will have a diagnosis. How will we begin to understand the nature of their disease? How can we prevent it?
WHY WERE NECROPSY RATES SO LOW?
Surely any parent who has watched their child succumb to such a slow and distressing syndrome must want a diagnosis in its own right, as well as to know any risks to siblings and their offspring. The authors did not have contact with the families so we do not know how often necropsy was requested or how the approach …
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