The SEAC concluded that these were infected with a new variant of CJD and could well be linked to exposure to BSE before the introduction of the 1988 ban on the use of offal for cattle feed. In addition, the SEAC was careful to point out that there remains no scientific proof that BSE is transmissible to humans. A further regulation was introduced in the UK ensuring that carcasses from cattle over the age of 30 months had to be deboned in specifically licensed plants only. The pathologic findings in CJD are limited to the CNS , although the transmissible agent can be detected in many other organs . The immunochemical detection of the protease-resistant protein PrP , in situ or after extraction, allows rapid confirmation of the diagnosis.
Myoclonus is usually present and often prominent at some time during the course.
CJD patients are afebrile and have normal blood and cerebrospinal fluid profiles . Most patients develop signs of cerebellar and pyramidal tract dysfunction as the disease advances. The terminal stage is marked by decorticate and decerebrate postures, stupor and coma. Massive myoclonic responses to auditory or other sensory stimuli may create the false impression that the patient is alert and responsive. The optic type characterized by visual dis-turbances and usually cortical blindness.