CJD includes variants of Prion disease and typically the disease infects younger adults, though cases are known in the age range of 12 to 74 years

As the disease progresses the brain becomes sponge-like and psychiatric & neurological symptoms develop. There are four variants of this disease and diagnosis can be very difficult and often the exact type is not known till after the disease has run it's course

• Sporadic CJD affects mainly the over 50s and is of unknown cause. It is marked by rapid onset of dementia followed by neurological symptoms – which distinguish it from depression. The course of the disease is typically measured in months

• Familial CJD is inherited, with younger onset and usually a longer time course than sporadic CJD

• Iatrogenic CJD occurs through contamination with infected tissue via medical procedures such as treatment with human growth hormone

• Variant CJD is caused by exposure to BSE and typically affects younger people. It has a relatively longer time course than most other forms – with an average of 14 months between onset of symptoms to death. Early symptoms are often psychiatric, such as anxiety and depression, and there may be persistent pain, with odd sensations in the face and limbs. These are followed by more obvious neurological symptoms and progressive dementia. Be alert to psychiatric illness with atypical features and course, especially if more neurological features appear such as sensory symptoms, pain or ataxia

There is no treatment for the disease, although drugs such as valproate and clonazepam are used to relieve certain symptoms.

Research is continuing and recent treatment with the drug pentosan polysulphate, which is directly injected into the brains of the patients, may show positive results. This was highlighted recently on national news (September 2003). Pentosan Polysulphate a large Polysulphonated polyglycoside - made from beechwood - has been shown from animal studies to slow the accumulation of prions. If Pentosan Polysulphate is the only drug currently available to have a benefical effect on a patient, then it should (with the patient/relatives consent) be administered. It is licensed in the USA, but not in UK and the medical profession continues to stall by trialing other drugs instead

However, at present the best treatment seems to be prevention and measures are now in place to prevent infection from contaminated meat. Blood and transplant operations are also monitored for CJD

These are two good sources of further information:
http://bmj.bmjjournals.com/cgi/content/full/327/7420/886-a

http://www.cjdsupport.net/