A woman in Massachusetts puzzled doctors when her dementia caused a frighteningly rapid deterioration.
In just five months, the 78-year-old went from living independently to struggling to form sentences and needing help with basic tasks such as bathing and eating.
A relative had died weeks before the deterioration, so the woman’s daughter attributed the symptoms to grief – until it became clear something was deeply wrong.
Doctors struggled to reach a diagnosis, which meant the woman was repeatedly sent for tests at some of the finest hospitals – including Massachusetts General Hospital, Harvard Medical School, the University of Pittsburgh and the University of Calgary.
The 78-year-old woman began experiencing unexplainable rapid and severe cognitive decline (stock photo)
She was eventually found to have undiagnosed lung cancer that had spread to her lymph nodes, as well as a toxic build up of amyloid proteins in her arteries.
The woman was diagnosed with paraneoplastic encephalomyelitis (PEM), inflammation in the brain and spinal cord that leads to confusion, brain bleeds, cognitive decline and difficulty with thinking and speaking – similar to Alzheimer’s.
PEM is a rare neurological disorder that affects fewer than one in 100,000 people each year.
It develops because of the presence of cancer somewhere in the body.
The patient’s case was detailed in The New England Journal of Medicine.
Doctors said her symptoms began with a ‘flat affect,’ which the patient’s daughter had attributed to grief after the death of the woman’s sister.
‘More severe memory issues’ then developed and she was admitted to the hospital after a frightening episode that saw her struggle to speak.
An MRI revealed areas of her brain tissue were damaged from a repeated lack of blood supply, indicating she may have experienced a stroke.
But with no conclusive answers, doctors sent her home. Just 10 days later, the patient was becoming more impulsive, confused and more forgetful.
Her primary care doctor prescribed medication to treat ‘possible depression.’
Over the next several weeks, the woman’s condition continued to rapidly deteriorate and she began needing more help with daily life and bathing, as well as being reminded to eat.
The above brain scans are suggestive of a stroke, brain tissue damage and narrowing of the brain’s vessels
Her daughter, once again, brought her to the hospital about four months later where she underwent more extensive testing, including blood work, a spinal tap, X-rays and another MRI.
Testing revealed the woman had brain abnormalities, with damage to brain tissue and brain vessels, affecting the way blood and oxygen are supplied to the organ.
After her formal diagnosis, the patient underwent treatment with steroids but did not receive chemotherapy due to her family’s concern about her poor memory and confusion.
She did undergo immunotherapy, however, for six months.
The patient was also given a drug meant to treat seizures and the authors of the case report wrote her confusion decreased slightly and her awareness improved.
She was discharged from the hospital to a nursing facility on a course of steroids. However, she became paranoid and confused and her memory worsened.
Once her steroid treatment concluded, she developed respiratory distress and pneumonia and was admitted to the intensive care unit. As her condition began to deteriorate, the family chose to forego intervention and offer end-of-life comfort care instead.
The woman died at a hospice facility.
