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
