Tragic Medical Mystery: How Did One of The World's Top Cancer Researchers Succumb To a Rare Brain Infection?

“My father, Dr. Baselga, was a fighter and a believer in science. That’s why, when he was diagnosed with Creutzfeldt-Jakob disease (CJD), a rapidly progressing, fatal neurodegenerative disease, our lungs dropped to our stomachs,” she wrote. “The science behind CJD is a mystery and there are no treatments, which rendered his fighting spirit useless.”

Baselga’s death was confirmed by AstraZeneca, where he had been serving as executive vice president for research and development in oncology.

In a statement, AstraZeneca Chief Executive Officer Pascal Soriot described Baselga as an “outstanding scientific leader.”

“José leaves a lasting legacy in the scientific community and here at AstraZeneca. He set our Oncology R&D (research and development) function on a remarkable trajectory, and his visionary leadership, deep scientific expertise and strategic insight have delivered so many achievements that we can all be proud of and that will benefit patients' lives in the years to come.”

At AstraZeneca, Baselga’s work included “transformative collaborations” with pharmaceutical company Daiichi-Sankyo on the clinical development of Enhertu (trastuzumab deruxtecan) for breast cancer and datopotamab deruxtecan for non-small cell lung cancer.

Prior to AstraZeneca, Baselga served as Physician In Chief at Memorial Sloan Kettering Cancer Center in New York. He resigned from that position after a New York Times/Pro Publica investigation showed Baselga failed to disclose millions of dollars in payments from drug and healthcare companies. Baselga said at the time the lack of disclosure was “unintentional.”

What is Creutzfeldt-Jakob disease (CJD)?

CJD is a very rare prion disease that affects around one person per million per year, according to the Creutzfeldt-Jakob Disease Foundation.

A prion is a type of protein that can trigger normal proteins in the brain to fold abnormally.  Prion diseases occur when this protein becomes abnormal and clumps in the brain, causing brain damage.   85% of CJD cases are “sporadic” meaning they have no known cause.

Dr. Andrew Zillgitt, a neurologist at Beaumont Hospital in Royal Oak, Michigan, tells SurvivorNet that in his 10 years of practice he’s only seen five cases.

Other types of CJD include genetic CJD, which is passed down in families, and acquired CJD, which can occur through contamination during a medical procedure or through exposure to BSE (mad cow disease) through infected beef. It is unclear what type of CJD Baselga had.

Diagnosis of CJD can occur via a spinal fluid test, electroencephalogram (EEG) and MRI.

“Symptoms can vary from person-to-person, but the most common symptom is a rapidly progressive dementia, or rapidly progressive memory impairments,” Dr. Zillgitt says.

Other symptoms include:

• Difficulty walking
• Hallucinations
• Muscle twitching and stiffness
• Confusion
• Fatigue
• Difficulty speaking

There is no known cure for CJD, and following diagnosis, doctors focus their efforts on easing the patient’s symptoms and discomfort.

“Most therapies revolve around supportive care,” Dr. Zillgitt explains. “For example, if myoclonus (involuntary movements of the arms and legs) is present, seizure medications like, levetiracetam, clonazepam, or valproate could be beneficial. Psychiatric symptoms could be treated with medications like, quetiapine or risperidone. Overall, the care is palliative and there is no definitive treatment or cure.”

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