
THE NERVOUS SYSTEM
pronounced in Pick’s disease, as is impairment of visuospatial
function. As the disease progresses, all cognitive functions
decline. A rapid decline in expressive language, resulting in
aphasia, is diagnostic of PcD.
Prion diseases are neurodegenerative diseases caused by
abnormally folded proteins called
prions, also known as “slow
viruses.” Prions are resistant to enzymatic deactivation
because they do not possess nucleic acids as do viruses and
other infectious agents. When examined with a microscope,
the brain of a person with prion disease is seen to have a
spongiform encephalopathy, in which vacuoles (tiny, fluid-
filled cavities) fill the neuronal cytoplasm and make the
damaged tissue look porous like a sponge when viewed under
the microscope. Symptoms may not appear for 1 to 20 years,
but once the disease is active, it progresses rapidly to dementia
and death. There may also be ataxia due to cerebellar degener-
ation. Prion disease can be inherited, infectious, or sporadic
(of unknown cause). Inherited, or familial, prion disease is
genetic. There are 20 known genetic mutations that cause
prion disease. One of these is Creutzfeldt-Jakob disease.
Infectious, or acquired, prion disease is transmitted by eating
infected tissues or from medical procedures that use infected
tissues. Sporadic prion disease may be due to one of the other
two causes but without evidence of causation.
Thiamine deficiency (which can result from excessive
alcohol intake, malnutrition, dialysis, severe morning sickness
with vomiting during pregnancy, or prolonged administration
of intravenous fluids without vitamins) can cause Wernicke-
Korsakoff syndrome. About 10% of chronic alcoholics develop
We rnicke-Korsakoff syndrome. Wernicke’s syndrome is the
early,or acute, stage of the disorder, and Korsakoff’s amnesic
syndrome is the chronic phase of the disorder. Wernicke’s
syndrome is characterized by ataxia, paralysis of certain eye
muscles, and confusion. Hemorrhagic lesions of the walls
of the cerebral aqueduct and the third and fourth ventricles
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