Creutzfeldt-Jakob disease in the UK

The most important figures in this table are in the bottom right- hand corner: the nine deaths so far from the "new variant" of Creutzfeldt- Jakob Disease (CJD) in the UK. While every death from CJD (and its other forms) is a personal tragedy, those nine deaths have further importance. Besides those nine deaths, two people with the disease are still alive.

Diagnosis is usually only made after a death has been referred to the CJD Unit from hospitals or GPs. As the left-hand column shows, referrals have been holding steady. "Sporadic" CJD is the common form, usually found in people aged over 60. "Iatrogenic" is caused by the disease being introduced to the victim - mostly through treatment with human growth hormone. "Familial" cases occur in families which have a mutation of the PrP gene (see main text). "GSS" - Gerstmann-Straussler-Scheinker syndrome - is an extremely rare inherited disease also associated with PrP mutation.

The CJD Unit is convinced vCJD is a new phenomenon. One non-UK case has been discovered - retrospectively - in Lyons, bringing the total number of definite vCJD cases recorded to 12. The possibility that more might be out there is occupying many medics' time.