The Centre for Health Protection (CHP) recorded two probable cases of sporadic Creutzfeldt-Jakob disease (CJD) in late August 2015. The first case was a 57-year-old woman who enjoyed good past health. She presented with vertigo and deteriorated cognitive function on July 1 and was admitted to a public hospital on July 24. She was found to have progressive dementia with cerebellar signs and subsequently developed myoclonus. Findings from magnetic resonance imaging (MRI) of the brain and electroencephalography were suggestive of CJD. The patient’s condition deteriorated and succumbed on September 12.
The second case was a 58-year-old woman with underlying illnesses. She presented with progressive dementia in June 2015 and was admitted to a public hospital on July 3. Findings of electroencephalography and brain MRI were suggestive of CJD. She was discharged on July 10. Subsequently, her condition deteriorated rapidly and was readmitted to the same hospital on August 10 with pyramidal and extrapyramidal dysfunction, cerebellar and visual disturbance, myoclonus and akinetic mutism. She is currently in serious condition.
Both cases had no known family history of CJD. No risk factors for either iatrogenic or variant CJD were identified and they were classified as probable cases of sporadic CJD.
According to the Centers for Disease Control and Prevention, Classic CJD is a human prion disease. It is a neurodegenerative disorder with characteristic clinical and diagnostic features. This disease is rapidly progressive and always fatal. Infection with this disease leads to death usually within 1 year of onset of illness.
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.
In the United States this translates to 250-300 new cases per year.
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.
Whereas 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.
To learn more about CJD and other prion diseases, or to help or find support– visit the Creutzfeldt-Jakob Disease Foundation