Creutzfeldt–Jakob or CJD is a degenerative neurological disease that is incurable and invariably fatal. CJD is at times called a human form of mad cow disease. CJD is caused by an agent called a prion. Prions are misfolded proteins that replicate by converting their properly folded counterparts, in their host, to the same misfolded structure they possess. CJD causes the brain tissue to degenerate rapidly, and as the disease destroys the brain, the brain develops holes and the texture changes to resemble that of a kitchen sponge. The first symptom of CJD is rapidly progressive dementia, leading to memory loss, personality changes, and hallucinations. Other frequently occurring features include anxiety, depression, paranoia, obsessive-compulsive symptoms, and psychosis.
In the year 1999, 32 CJD cases were reported per 30,492,106 of Canadian population. The incidence rate was found to be 1.05 per million population. The high incidence of CJD in Canada was in the year 2009 which accounts to 1.57 per million population. From the year 2009 to 2015, the incidence rate was decreased to 0.94 per million population. 22 cases were reported in the 2015 per 35,702,707 of Canadian population.
No generally accepted treatment for CJD exists; the disease is invariably fatal and research continues. Amphotericin B and Doxorubicin have been investigated as potentially effective against CJD, but as yet there is no strong evidence that either drug is effective in stopping the disease. Further study has been taken with other medical drugs, but none are effective. However, drugs to reduce suffering do exist, and include valproate, an anticonvulsant agent, clonazepam and benzodiazepine, to reduce muscle jerks.
The ongoing researches in Canada on Creutzfeldt–Jakob disease include: Update on the provisional estimation of developing iatrogenic variant Creutzfeldt-Jakob disease from human islet cell transplantation procedures, Diaschisis as the presenting feature in sporadic Creutzfeldt-Jakob disease, The genetics of susceptibility to variant Creutzfeldt-Jakob disease.