Creutzfeldt-Jakob
Disease (CJD) is a rare, fatal brain disorder, which causes a rapid,
progressive dementia and associated neuromuscular disturbances. The
disease in the United Kingdom is often referred to as a subacute spongiform
encephalopathy, BSE or as it is more commonly known, Mad Cow Disease.
The disease is named after Drs. Hans Gerhard Creutzfeldt and Alfons
Jakob, who documented the first cases of this illness in the 1920’s.
As there are many variants of CJD here we will be addressing the issues
of Bovine Spongiform Encephalopathy or “Mad Cow Disease”
and New Variant Creutzfeldt-Jakob Disease. As of writing "Mad
Cow Disease" has not been found in the United States.
Associated
Features:
Insomnia.
Depression.
Confusion.
Personality
and/or Behavioral change.
Strange Physical sensations.
Memory Difficulties.
Coordination difficulties.
Rapid Progressive Dementia.
Differential
Diagnosis:
Some disorders have similar symptoms. The clinician, therefore, in
his diagnostic attempt, has to differentiate against the following
disorders which need to be ruled out to establish a precise.
diagnosis.
Kuru
Gerstmann-Straussler-Scheinker Disease
Fatal Familial Insomnia
Dementia
Cause:
Initially, thought
to be caused by a slow virus having a long incubation period, further
research, however, has indicated that this agent differs significantly
from viruses and other conventional agents and therefore the virus
explanation is now in doubt. Recently, a new pathogen, called a "prion",
short for " proteinaceous infectious particle" have been
identified as the possible causation agent. Prions are thought to
transform normal, benign protein molecules into infectious, deadly
ones by altering the shape of the healthy molecules to the dangerous
conformation. This transformation then induces a chain reaction to
alter the shape of the other benign protein molecules into the deadly
form. Regardless of this debate Scientists in the UK have recognize
that a transmissible agent responsible for causing Creutzfeldt-Jakob
Disease, involves the consumption of meat products obtained from infected
animals. The identification of this transmissible agent has been the
subject of much scientific inquiry and debate.
There appear to
be three general categories for classifying the means through which
CJD may be acquired. First, the disease can occur sporadically, without
apparent cause. Second, the disease can be inherited. Third, the disease
can be transmitted through infection (meat products).
Treatment:
There is no treatment that can
cure or control CJD. Current treatment is aimed at alleviating symptoms
and making the patient as comfortable as possible.
Pharmacotherapy
[ See Psychopharmacology
Section ] :
Opiate
drugs can help relieve pain, and the drugs clonazepam and sodium valproate
may help relieve involuntary muscle jerks.
