The discovery will show whether the disease is spreading and which patients with early signs of dementia would respond to treatment. The test involves removing a piece of tissue from the tonsils , possible under local anaesthetic. John Collinge and his colleagues at Imperial College and St Mary's Hospital, London, report in The Lancet that they have found a prion protein characteristic of the new strain of CJD in a tonsil sample taken from a woman who had died of the disease. The prion protein was discovered by the same team last October in the brains of people who had died with the disease. Although there is currently no treatment for CJD , confirming the diagnosis is important because in its early stages the symptoms are similar to other degenerative diseases which are treatable.
Diagnosis is at present only possible by brain biopsy, a dangerous and unreliable operation.
We identified 19 cases of spongiform encephalopathy of which only 11 were diagnosed before death. Their brains showed little or no external abnormality. In contrast, only two of the eight clinically unrecognized cases had characteristic symptoms. The brains showed moderate or severe cerebral atrophy. This suggests that human prion disease may be more common than previously supposed and that a further review of the epidemiology of the disease is required.