Hood River County in Oregon has reported a cluster of three cases of Creutzfeldt-Jakob Disease (CJD). This even rar rarer and fatal brain disorder is genetically related to “mad cow” disease. The Hood River County Health Department is using these cases to engage in active case investigation. They are out there to detect any emerging public health hazards and provide information to the public.
Creutzfeldt-Jakob Disease or CJD is a degenerative lifelong neurodegenerative condition with a 100% fatality rate. Globally, CJD strikes about one to two people per million each year. In the US, health officials say they see only about 350 new cases of the disease each year. Sadly, about 70% of those diagnosed with CJD will succumb to the disease within a year. This staggering number exemplifies the ferocity with which this disease attacks.
It’s prions that are the agents of this disease. These infectious proteins induce unprecedented misfolding of normal cellular proteins throughout the brain, leading to a devastating and continually progressive decline in neurological function. There are three primary types of CJD: sporadic, genetic, and acquired. The sporadic form accounts for approximately 85% of all cases. The genetic form is linked to inherited mutations in a gene known as PRNP. You can get this form by consuming beef from cattle that have been contaminated with Bovine Spongiform Encephalopathy (BSE). At its most extreme, this disease is more colloquially known as mad cow disease. Remarkably, only seven cases of BSE have ever been detected in cattle inside the United States.
CJD can take root not only through dietary exposure but through medical procedures. This includes procedures as intricate and life-giving as organ transplants and blood transfusions. Crucially, the disease has a long incubation period, sometimes taking years or even decades before symptoms appear. As with other forms of CJD, definitive diagnosis can only happen after death, through examination of brain tissue and cerebrospinal fluid.
“The risk of getting CJD is extremely low,” – Hood River County Health Department.
They reaffirmed that the disease is not transmitted through air particles, physical contact, social relations or water supply.
“It does not spread through the air, touch, social contact, or water.” – Hood River County Health Department.
While the investigation into these specific cases continues, local health officials remain dedicated to keeping watch over an outbreak that further illustrates the importance of vaccination. In return, they have been committed to being open and honest with residents about any dangers to public health that may arise.
“The health department will continue to monitor the situation and keep you informed of any risk to public health.” – Hood River County Health Department.
Public health experts, including the CDC and WHO, recommend educating yourself on the disease and its transmission. Please reach out to your healthcare professional if you have questions or concerns. Community members are encouraged to stay alert but not alarmed, as CJD is still a very rare condition.
