A woman dies of rare dementia contracted after pregnancy



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A woman died of a rare form of dementia after decades of infection by her own baby while she was pregnant.

Her husband died of sporadic Creutzfeldt-Jakob disease two decades ago – but her own genes were previously unaffected by responsible mutation, reports the Daily Mail.

However, the unidentified woman, originally from Denmark, died of the same disabling illness as her late husband when she was seventy years old.

His son, whose identity was also concealed, but who is known to be 53 years old and is himself a father, is now showing sporadic CJD symptoms.

Doctors now believe that the son inherited the mutated gene responsible for the disease before passing it on to his mother while in the womb.

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Fetal cells containing the toxic proteins are believed to have passed through the placenta into the bloodstream before lodging in the brain.

This deadly disease causes irreversible brain damage, caused by abnormal proteins called prions, which gradually destroy brain cells.

The rare case was discovered by a team of doctors from the Danish Reference Center for Prion Diseases at the University Hospital of Copenhagen.

The woman was diagnosed with sporadic CJD before the disorder was linked to her deceased husband or son.

CJD is caused by abnormal proteins known as prions, which damage nerve cells.

Prions can appear spontaneously, be inherited or transmitted on contaminated surgical equipment.

There is no cure.

The treatment aims to relieve symptoms and put patients at ease with painkillers and antidepressants.

Ausrine Areskeviciute, one of the doctors who stumbled on the case, told the Times that it was a "very sad story".

She said, "We already know that when a woman is pregnant, the baby's cells cross the placenta and move around her body to lodge in various organs.

"However, in this case, the fetus was carrying the mutation for misfolded proteins, and its cells could also have had misfolded proteins when they entered the mother's body."

Ms. Areskeviciute added that this may have triggered the process that led to her death years later.

The case of CJD, which is considered a type of dementia, has been published in the Journal of Neuropathology & Experimental Neurology.

CJD, which has four main types, is a prion disease, which derives its name from "protein" and "infectious".

It is defined by the proteins of the nervous system taking an unusual form, which then propagate as a domino to cause damage to the brain.

Some 85% of cases occur at random, while 10% to 15% are inherited. In less than one percent of cases, CJD is acquired.

Mad cow disease is also a prion disease, which occurs when a person eats meat from cattle with a similar disease.

According to NHS figures, sporadic CJD affects at least one in a million people each year in the UK.

This follows research published earlier this year that suggests that prion diseases can spread during operations via poorly cleaned surgical equipment.

What is Creutzfeldt-Jakob Disease?

Creutzfeldt-Jakob Disease (CJD) is a rare and fatal disease that causes irreversible brain damage.

The symptoms, which worsen rapidly over time, include:

Loss of intellect and memory
Personality changes
Loss of balance and coordination
Speech disorders
Vision problems and blindness
Abnormal jerky movements
Loss of mobility

Most victims die in the year following the onset of symptoms, usually because of an infection caused by immobility.

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