Creutzfeldt Jakob Disease

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Creutzfeldt Jakob Disease

CJD (Creutzfeldt-Jakob Disease)

Creutzfeldt-Jakob disease is a degenerative brain disorder that leads to dementia and, ultimately, death. Symptoms of Creutzfeldt-Jakob disease (CJD) sometimes resemble those of other dementia-like brain disorders, such as Alzheimer's, but Creutzfeldt-Jakob disease usually progresses much more rapidly.

Anatomy

A progressive neurologic disorder, one of the subacute spongiform encephalopathies caused by prions. Clinical features of CJD include a progressive cerebellar syndrome, including ataxia, abnormalities of gait and speech, and dementia. In most patients, these symptoms are followed by involuntary movements (myoclonus) and the appearance of a typical electroencephalogram pattern. Changes in the CSF are absent or nonspecific.

The majority of cases of CJD (about 85%) occur as sporadic disease, a smaller proportion of patients (5-15%) develop CJD because of inherited mutations of the prion protein gene. These inherited forms include Gerstmann-Straussler-Scheinker syndrome and fatal familial insomnia.

Classic CJD has been recognized since the early 1920s. The most common form of classic CJD is believed to occur sporadically, caused by the spontaneous transformation of normal prion proteins into abnormal prions. This sporadic disease occurs worldwide, including the United States, at a rate of roughly one case per 1 million population per year, although rates of up to two cases per million are not unusual. The risk of CJD is higher in older persons; in those 60 years of age and older, the average annual rate has been approximately 4.6 cases per million.

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