Yesterday we learned of the death of the prestigious Catalan oncologist, Dr. Josep Baselga, at the age of 61, as a result of Creutzfeldt-Jakob disease.
Dr. Baselga and Dr. Tabernero founded in 1998 the Institute of Oncology (IOB), a member center of Top Doctors and a national and international reference unit in cancer research and treatment.
Top Doctors regrets the death of Dr. Baselga, a renowned oncologist who dedicated his life to saving thousands around the world. During his career he researched to better understand the molecular mechanisms of cancer, and that this translated into clinical advances to improve the treatment of tumors.
Creutzfeldt-Jakob disease: what it is and who it affects
Creutzfeldt-Jakob disease is a rapidly progressive degenerative neurological disorder. It usually results in dementia and, ultimately, death. Symptoms may be similar to those of similar brain disorders, such as Alzheimer’s disease, but CJD progresses more rapidly.
It is a disease that usually develops late, around 60 years of age, being a very uncommon pathology. It is estimated that one to two cases per million people are diagnosed each year worldwide.
Why does Creutzfeldt-Jakob disease occur?
Creutzfeldt-Jakob disease, as well as its variants, are included in the human (and animal) diseases known as transmissible spongiform encephalopathies. These pathologies are abnormal versions of a type of protein called a prion. As a rule, these proteins are produced in antibodies and are harmless. However, when deformed, they become infectious.
What are the symptoms of the disease?
The main characteristic of Creutzfeldt-Jakob disease is a very rapid mental deterioration, which usually occurs within a few months. Symptoms also include:
- Memory loss.
- Personality changes.
- Blurred vision or blindness.
- Insomnia.
- Altered thoughts.
- incoordination
- speech difficulties
- swallowing problems
- sudden jerky movements
These symptoms worsen as the disease progresses. Most patients eventually go into a coma, and death usually occurs within a year of diagnosis.
How can Creutzfeldt-Jakob disease be contracted?
The percentage of people who get the disease is very low. It can mainly develop in three ways:
- Sporadically. Most patients with Creutzfeldt-Jakob disease develop it for no apparent reason, and it is called spontaneous Creutzfeldt-Jakob disease.
- Genetic. This is the case in less than 15% of people with the disease, who have a family history, or a genetic mutation related to the disease. This type is called hereditary Creutzfeldt-Jakob disease.
- Contamination. A small percentage of people have developed the disease after being exposed to infected human tissue during medical procedures. This can occur during corneal or skin transplants but also after undergoing brain surgery with contaminated instruments, as standard cleaning methods do not destroy the abnormal prions. This variant is known as iatrogenic Creutzfeldt-Jakob disease. A very low percentage of people have developed the pathology after eating contaminated beef, mainly from eating meat infected with mad cow disease (bovine spongiform encephalopathy).
Relationship of Creutzfeldt-Jakob disease to contaminated meat: popular cases in the 1990s
Creutzfeldt-Jakob disease became popular in the 1990s precisely because of the last case mentioned above, when some people contracted a variant of this disease in the United Kingdom after eating contaminated meat from diseased cattle, which became known as mad cow disease. However, classical Creutzfeldt-Jakob disease is not related to contaminated meat, but this is a variant of the pathology.
Does Creutzfeldt-Jakob disease have a treatment or cure?
To date, there is no treatment that can cure or significantly improve the symptomatology. The only possible treatment aims to alleviate the symptoms as far as possible, and thus improve the patient’s quality of life.