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Creutzfeldt-Jacob Disease ( "Mad Cow Disease" UK )

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.
  

related books
  Statistical Aspects of BSE and vCJD. Models for Epidemics
UK Support Groups
  Alzheimer's Society UK

CJD national coordinator:
Gillian Turner Email:cjdnet@alzheimers.org.uk)
Website: http://www.alzheimers.org.uk/cjd/
DSM IV
  None Specific
ICD 10
  None Specific
Chat Room
  None Located
PsychNet-UK Links
  Cognitive Disorders
Page Updated
12th August 2003