An Injury At Work Led To The Death Of A Woman From A Rare Disease After 8 Years

An injury at work led to the death of a woman from a rare disease after 8 years

The victim worked as a technician in a laboratory that studied prion diseases.

Photo: CC0 Public Domain

An incident in a French laboratory led to the illness and death of a woman 7.5 years later, scientists say. New England Journal of Medicine talks about unexpected development of extremely rare prion pathology - a variant form of Creutzfeldt-Jakob disease - after an injury at work

Prions are proteins with an irregular structure. They are infectious agents because they can transmit their "irregularity" to normal proteins. They cause several diseases in humans and animals.

The victim was a young woman (then 24) who worked as a laboratory technician on a prion project in 2010. She pricked her thumb with curved forceps, which were used to transfer fragments of the brain infected with prions. Despite wearing two pairs of protective gloves, the tool pierced her skin.

The pathological symptoms associated with this injection appeared 7.5 years later. The disease began with a burning sensation in the right shoulder and neck. The woman's condition worsened during the year: her memory began to be impaired, visual hallucinations appeared, and muscle spasm on the right side developed. She died 19 months after the onset of the disease. Diagnostics performed both before and after death confirmed variant Creutzfeldt-Jakob disease.

Prion diseases are very rare, and genetics influences the likelihood of their development even after infection. Scientists believe that another method of infection - not related to work - in this case was unlikely. Infection is possible when eating meat. But, given the genetic characteristics of a woman, with this route of transmission, the disease should have developed for more than 10 years.

Scientists write that this case “highlights the need to improve prevention of prion disease in laboratories and medical institutions specializing in neurosurgery.