A rare, fatal brain disorder that causes a rapid, progressive dementia and associated neuromuscular disturbances. The disease is often referred to as subacute spongiform encephalopathy because it usually produces microscopic cavities in neurons that appear sponge-like. The illness is believed to be caused by prions, proteinaceous infectious particles, which are abnormal forms of a human gene.
A disease of the nervous system caused by a slow-acting prion which eventually affects the brain. It may be linked to BSE in cows.
Progressive fatal neurological disorder caused by an infectious protein particle contracted from cows infected with bovine spongiform encephalopathy.
A rare fatal condition characterized by a rapidly progressive mental deterioration and muscle wasting. An abnormal cellular protein causes degeneration of nerves and glial (connective) tissue in the brain causing vacuoles (holes) within the brain called spongiform encephalopathy. People with Creutzfeldt-Jakob disease develop seizures and involuntary movements, as well as weakness, imbalance, and confusion. These changes ultimately lead to death.
A rapidly progressive, fatal, degenerative disease in humans caused by an abnormal prion protein. There are three aetiological forms of CJD: sporadic, iatrogenic, and inherited. Sporadic CJD occurs randomly in all countries and has an annual incidence of one per million. Iatrogenic CJD is caused by accidental exposure to human prions through medical and surgical procedures, and cannibalism in the case of the human prion disease known as kuru that occurs in a tribe in New Guinea, where it is called the trembling disease. Inherited or familial CJD accounts for 15 per cent of human prion disease and is caused by a mutation in the prion protein gene. In recent years a new variant of CJD has been identified that is caused by bovine spongiform encephalopathy (BSE), called variant CJD. The incubation period for the acquired varieties ranges from four to 40 years, with an average of 10—15 years. The symptoms of CJD are dementia, seizures, focal signs in the central nervous system, myoclonus, and visual disturbances.
A central nervous system disease that causes rapidly progressive dementia usually accompanied by muscle jerking, difficulty walking, and aphasia. The causative agent is assumed to be a prion and may be related to the agent that causes bovine spongiform encephalopathy (“mad cow disease”). Creutzfeldt-Jakob disease has developed in the recipient of a cornea from a donor with the disease and in a few recipients of human growth hormone. There is no treatment, and the disease is fatal.
Within the realm of central nervous system disorders, there exists a degenerative ailment triggered by a prion, commonly referred to as a “slow virus.”
Creutzfeldt-Jakob disease (CJD), an uncommon neurodegenerative disorder, gradually affects the brain. It is believed to stem from prion infection, a specific type of transmissible protein-based pathogen. In sheep, a related agent leads to scrapie, while in cattle, it causes bovine spongiform encephalopathy (BSE).
One variant of CJD predominantly impacts individuals in their middle age or elderly years, and its etiology remains elusive. Another form emerges among younger individuals and is linked to contamination resulting from brain surgeries or transplants involving infected donors, as well as the administration of infected human growth hormone or gonadotrophins as part of medical treatment.
A third variant of CJD, known as new variant (nv) CJD, was initially discovered in 1995 and predominantly impacts individuals in their teenage and early adult years. It is believed to be contracted through the consumption of beef contaminated with BSE. This particular form induces pathological alterations in the brain that bear resemblance to the changes observed in cattle afflicted with BSE.
The symptoms of all disease variants share notable similarities. They encompass a gradual decline in brain function, leading to progressive dementia. Additionally, sudden muscular contractions, known as myoclonus, manifest, while coordination abilities diminish. The individual experiences deterioration in intellect and personality, and in some cases, blindness may ensue. As the disease advances, speech abilities are lost, and the body becomes rigid.
Regrettably, no effective treatment currently exists for the disease, and the prognosis is generally grim, with individuals typically succumbing within a span of two to three years.