Case Records of the Massachusetts General Hospital

CASE RECORDS OF THE MASSACHUSETTS GENERAL HOSPITAL
with relative sparing of the Purkinje cells (Fig. 8).
This so-called synaptic pattern of staining 40 is distinct
from the more discrete agglomeration seen in kurutype
plaques.
The mechanism by which the prion protein involved
in Creutzfeldt–Jakob disease causes the histopathological
changes that are characteristic of the
disorder is unknown. The transmissible agent of the
disease has marked resistance to inactivation by standard
means of decontamination, although various
methods have been shown to inactivate the agent.
Specific techniques are used to handle tissue in cases
of Creutzfeldt–Jakob disease. 41,42
Over the past few years, the emergence of a prion
disease in cattle (bovine spongiform encephalopathy),
43 as well as an unusual type of Creutzfeldt–
Jakob disease, termed new-variant Creutzfeldt–Jakob
disease, has sparked much scientific and public interest.
The characteristics of new-variant Creutzfeldt–
Jakob disease include a young age at its onset, a slower
clinical course, the presence of psychiatric and sensory
symptoms early in the disease, and the finding of
widespread kuru-type plaques on neuropathological
examination. 44,45 Studies of PrP Sc have demonstrated
that there are several distinct patterns of fragment size
and glycosylation among the various prion diseases.
46 Such studies have been used to support the link
between bovine spongiform encephalopathy and newvariant
Creutzfeldt–Jakob disease. 47 Support for this
relation is also found in transmission studies. 48
ANATOMICAL DIAGNOSIS
Creutzfeldt–Jakob disease.
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