The longest recorded survivor of variant Creutzfeldt–Jakob disease (vCJD) was Jonathan Simms, a Northern Irish man who lived 10 years after his diagnosis. Epidemiology
CDC monitors the occurrence of CJD in the United States through periodic reviews of national mortality data. According to the CDC:
CJD occurs worldwide at a rate of about 1 case per million population per year. On the basis of mortality surveillance from 1979 to 1994, the annual incidence of CJD remained stable at approximately 1 case per million people in the United States. In the United States, CJD deaths among people younger than 30 years of age are extremely rare (fewer than five deaths per billion per year). The disease is found most frequently in people 55–65 years of age, but cases can occur in people older than 90 years and younger than 55 years of age. In more than 85% of cases, the duration of CJD is less than one year (median: four months) after the onset of symptoms.Further information from the CDC:
Risk of developing CJD increases with age. CJD incidence was 3.5 cases per million among those over 50 years of age between 1979 and 2017. Approximately 85% of CJD cases are sporadic and 10-15% of CJD cases are due to inherited mutations of the prion protein gene. CJD deaths and age-adjusted death rate in the United States indicate an increasing trend in the number of deaths between 1979 and 2017.Although not fully understood, additional information suggests that CJD rates in African American and nonwhite groups are lower than in whites.
Diffuse cortical vacuolization occurs in Alzheimers disease, and superficial cortical vacuolization occurs in ischemia and frontotemporal dementia. These vacuoles appear clear and punched-out. Larger vacuoles encircling neurons, vessels, and glia are a possible processing artifact. Classification
Types of CJD include:
Sporadic (sCJD), caused by the spontaneous misfolding of prion-protein in an individual. This accounts for 85% of cases of CJD. Familial (fCJD), caused by an inherited mutation in the prion-protein gene. This accounts for the majority of the other 15% of cases of CJD. Acquired CJD, caused by contamination with tissue from an infected person, usually as the result of a medical procedure (iatrogenic CJD). Medical procedures that are associated with the spread of this form of CJD include blood transfusion from the infected person, use of human-derived pituitary growth hormones, gonadotropin hormone therapy, and corneal and meningeal transplants. Variant Creutzfeldt–Jakob disease (vCJD) is a type of acquired CJD potentially acquired from bovine spongiform encephalopathy or caused by consuming food contaminated with prions. Treatment
As of 2022, there is no cure or effective treatment for CJD. Some of the symptoms like twitching can be managed, but otherwise treatment is palliative care. Psychiatric symptoms like anxiety and depression can be treated with sedatives and antidepressants. Myoclonic jerks can be handled with clonazepam or sodium valproate. Opiates can help in pain. Seizures are very uncommon but can nevertheless be treated with antiepileptic drugs. Prognosis
The condition is universally fatal. As of 1981, no one is known to have lived longer than 2.5 years after the onset of CJD symptoms.