Walsh & Hoyt: Variant Creutzfeldt-Jakob Disease (vCJD)

In 1995, two cases of prion disease were reported in teenagers. These patients exhibited unique clinical characteristics in addition to their young age, and this disease was designated ""new variant"" or ""variant"" CJD (vCJD). An initial series of 10 patients with vCJD included 4 males and 6 females, 8 of whom had died at the time of publication. The ages of these 8 patients ranged from 19 to 41 years (median, 29 years), and the duration of illness ranged from 7.5 to 22.5 months. Nine of the patients presented with psychiatric symptoms, and 9 developed ataxia early in the course of the disease. Although all 10 patients developed progressive dementia, dementia was not a part of the initial presentation in eight. None of the patients developed typical EEG changes. Spongiform changes and amyloid plaques resembling those found in patients with kuru were identified neuropathologically in all 10 cases. None of the patients had a history of potential iatrogenic exposure or blood transfusions or had ever worked on a farm. These cases resulted in a world-wide media frenzy and a ban on British beef. As of December of 2003, a total of 153 cases of vCJD had been documented, including 143 cases (137 deaths) in the United Kingdom, 6 cases in France, and 1 case each in Italy, Canada, Ireland, and the United States. The patients in Ireland and the United States had lived in the UK for at least 5 years during the BSE epidemic.