b. Clinical presentation: The major features are dementia and
myoclonus. Other symptoms, less consistently seen, include motor disorders
and cerebellar incoordination.
c. Duration: death usually in 3-12 months
d. Transmission: A small percentage of cases are familial, but
most are sporadic. In a few cases, transmission has occurred by corneal
transplant or administration of contaminated growth hormone.
e. CSF findings: usually normal; possible slight increase in
2. Pathological changes
a. Gross pathology: Cortical atrophy and ventricular dilatation
may be present; these changes are usually mild and are not diagnostic.
b. Microscopic pathology: The major diagnostic change is spongiform
encephalopathy in gray matter throughout brain and spinal cord. The
tissue appears "spongy", i.e. containing holes. Severe neuronal
loss and gliosis also are present and mild demyelination may occur. Ultrastructural
changes include intracytoplasmic vacuoles as the basis for the spongy change.
This microscopic image shows
spongiform encephalopathy in a case of Creutzfeldt-Jakob disease.
Prominent gliosis in Creutzfeldt-Jakob
disease is illustrated in this image.