Creutzfeldt-Jakob Disease

• First case of mother-to-child transmission of vCJD - WHW Dec 2000
• New tests for CJD - WHW Jan 1997

First case of mother-to-child transmission of vCJD

December 2000 Women's Health Watch

United Kingdom newspapers have reported that doctors suspect an 11-month girl has variant CJD which she may have contracted from her mother. The child, whose mother died from the disease in May, has brain damage, is unable to swallow and has convulsions and stiff limbs. Doctors have not been able to give other explanations for her symptoms but cannot definitely confirm vCJD which requires a brain biopsy. Paediatric neurologists from Addenbrooke's Hospital in Cambridge stress the need for caution before drawing conclusions from the case. They say there are many reasons why children have developmental problems.
Ref: Lancet 2000; 356: 1085

New tests for CJD

January 1997 Women's Health Watch

An American group has announced it can diagnose Creutzfeldt-Jakob Disease (CJD) in possible sufferers. Previously, an accurate diagnosis could only be made at post-mortem. The group from the National Institutes of Health and the Californian University of Technology can identify markers for the protein found in humans with CJD and animals with bovine spongiform encephalopathy (BSE), or mad cow disease.

The researchers tested a wide range of patients with neurological diseases and found after post-mortem reports on 260 people that the new test is 98% sensitive and 99% specific.

The new test can be used to accurately diagnose suspected sufferers, although there is no cure, and also to screen potential blood donors.

Still on the subject of BSE: Research by the UK Ministry of Agriculture, Fisheries and Food has shown that the risk of infected cows passing BSE to their offspring is 10%. They say that outside of study conditions this means that 1% of calves born to cows who die of BSE will themselves die from BSE caught from their mothers.

The route of transmission is still not known: whether it occurs in utero, at birth, or soon after birth.

Maternal transmission also occurs in sheep, goats and ruminants such as the kudu, an African antelope.
Ref: BMJ 1996; 313: 836; BMJ 1996; 313: 317 Also the BMJ is on the Internet: www.bmj.com