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Creutzfeldt–Jakob disease (CJD), also known as subacute spongiform encephalopathy or neurocognitive disorder due to prion disease, is a fatal neurodegenerative disease. [ 4 ] [ 1 ] Early symptoms include memory problems, behavioral changes, poor coordination, and visual disturbances. [ 4 ]
In 2019, through routine case management between the New Brunswick Regional Health Authorities and the federal Ottawa-based Creutzfeldt-Jakob Disease Surveillance System (CJDSS), CJDSS had observed that a significant number of referrals from New Brunswick had "some common symptoms and similar potential diagnostic profiles". [1]
For example, Creutzfeldt–Jakob disease has been transmitted to patients taking injections of growth hormone harvested from human pituitary glands, from cadaver dura allografts and from instruments used for brain surgery (Brown, 2000) (prions can survive the "autoclave" sterilization process used for most surgical instruments).
Variant Creutzfeldt–Jakob disease (vCJD), formerly known as New variant Creutzfeldt–Jakob disease (nvCJD) and referred to colloquially as "mad cow disease" or "human mad cow disease" to distinguish it from its BSE counterpart, is a fatal type of brain disease within the transmissible spongiform encephalopathy family. [7]
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Fatal insomnia is an extremely rare neurodegenerative prion disease that results in trouble sleeping as its hallmark symptom. [2] The majority of cases are familial (fatal familial insomnia [FFI]), stemming from a mutation in the PRNP gene, with the remainder of cases occurring sporadically (sporadic fatal insomnia [sFI]).
The Creutzfeldt-Jakob Disease Surveillance System (CJDSS) is a unit of the Public Health Agency of Canada. It studies the various variants of Creutzfeldt-Jakob Disease , and at least as of 2017, assisted "with DNA sequencing , autopsy and case confirmation". [ 1 ]
Creutzfeldt–Jakob disease, a fatal degenerative brain disorder caused by prions involving the cerebral cortex, the basal ganglia and the spinal cord.; Adrenoleukodystrophy, a rare demyelination disorder also known as Siemerling-Creutzfeldt disease that causes damage to the myelin sheaths of neurons in the brain, resulting in seizures and hyperactivity.