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 2005, the first case of variant Creutzfeldt-Jakob disease (vCJD) was reported in Spain, in a woman born in 1978 with clinical onset of symptoms in 2004. She subsequently died in 2005. Recently, two more laboratory-confirmed vCJD cases were reported to the Spanish CJD state registry. In February 2006, a woman born in 1957 developed progressive cognitive deterioration, and died in December 2007 with suspected sporadic CJD. From October 1996 to March 2011, 5 cases of vCJD have been reported in Spain.
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 Spain on Creutzfeldt–Jakob disease include: PrP mRNA and protein expression in brain and PrP(c) in CSF in Creutzfeldt-Jakob disease MM1 and VV2, Lymphomatosis cerebri mimicking iatrogenic Creutzfeldt-Jakob disease, Neuronal antibodies in Creutzfeldt-Jakob disease.