The Problem of Creutzfeldt-Jakob Disease

Creutzfeldt–Jakob disease (CJD) which was often called ‘mad cow disease’ in the popular media is a degenerative brain disease that is fatal. It has a number of similarities to Alzheimer’s disease as it starts with a rapid progress of memory problems; behavioural changes such as paranoia, obsessive-compulsive symptoms and psychosis; poor coordination and falls and visual disturbances. Later on the symptoms include dementia and involuntary jerky movements. It is much more rapid in its progress than Alzheimer’s disease and they soon end up in a coma with around 70% of the people with CJD dying within a year of the diagnosis. The disease was named after the German neurologists Hans Gerhard Creutzfeldt and Alfons Maria Jakob back in 1920. The cause of CJD is an infectious type of abnormal proteins that are known as prions. Most cases of CJD occur for unknown reasons with almost 10% are inherited from a person's parents in an autosomal dominant manner. There are 3 types of CJD, sporadic, familial and acquired. Being exposed to brain or spinal tissue from an infected person may also result in its spread. There is no evidence that CJD can spread among people via normal contact with an infected person. CJD affects about one person per million people per year and the onset is typically around 60 years of age.

A variant form of Creutzfeldt-Jakob disease is the human version of mad cow disease that affects cows and is the popular term for the whole group of conditions that get reported in the popular media. It is believed to be caused by the eating of beef products contaminated with central nervous system tissue, such as brain and spinal cord, from cattle that are infected with mad cow disease. There was an outbreak of this in the UK in the 80’s and 90’s with a reported 178 deaths from it during that time after eating infected beef. British beef was banned from export around the world with some bans remaining in place until as recently as 2019. The outbreak led to a significant public health response to deal with the crisis and the panic in the mass media. In the USA it is required that all brain and spinal cord materials be removed from high-risk cattle that shows any signs of a neurological problem. This is so that cow products do not enter the food supply chain to safeguard the public health. The chances of this being a problem now are quite low if any due to the measures that have been put in place.

There is no specific treatment for Cjd. Drugs can be used to help with the pain and the involuntary movements. Psychiatric symptoms like anxiety and depression can be treated with sedatives and antidepressants. There are a number of treatments that are under investigation that might be able to slow the progress of the disease. The most promising of these are the monoclonal antibodies with have been shown to almost halt the progress of the disease in a few people.

Craig Payne is a University lecturer, runner, cynic, researcher, skeptic, forum admin, woo basher, clinician, rabble-rouser, blogger and a dad.