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BOSTON MEDICAL MYSTERIES

Why was Wendy suddenly paralyzed?

MRI similar to Wendy’s, which shows white lesions in the brain.

MRI similar to Wendy’s, which shows white lesionsin the brain.

Solve a diagnostic puzzle: This is the case of a real patient treated at a Boston-area hospital

One Monday morning, 42-year-old Wendy woke up and found she couldn’t walk. Wendy and her husband, Bruce, lived in one of those pretty oceanside towns that dot the rocky arc of the North Shore of Massachusetts. They had a wonderful, happy life. Wendy worked downtown, her office high up in the glassy Hancock Tower that gave her an expansive view of Boston. A summertime tennis player, a wintertime skier, and a year-round active mom (she had a 7-year-old son and 3-year-old-daughter) Wendy considered herself lucky, blessed, healthy.

But Bruce had seen something befall Wendy that weekend that had begun to trouble him. At first, she had come down with some kind of flu. “I spent that weekend resting,” Wendy would say later, but Bruce noted that she had barely gotten out of bed at all. It wasn’t like her. He thought it would pass, but by Sunday night, things had only gotten worse. She had developed intermittently high fevers, her forehead was clammy, and Wendy had begun to say things to Bruce that were increasingly nonsensical. He wondered if she was hallucinating. “If you’re not better by tomorrow,” Bruce had said, “we’re taking you to the hospital.”

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On Monday, after her legs became paralyzed, Wendy called to her husband, in shock at the inability to coordinate her legs. Devotedly, Bruce helped her to dress. Then, he lifted her out of bed onto his back and carried her downstairs. As he carried his wife out to the driveway, his concern doubled. “Is that a new car?” Wendy asked him, when she laid eyes on the old Buick they had owned for years. He drove her straight away to the hospital downtown.

Wendy was rushed into the emergency room, where physicians inserted a long needle into the space between her lower vertebrae to test the fluid around her spinal cord. They were hunting for the source of her fevers and paralysis, wondering if her brain harbored infection. Soon enough, the laboratory called back with the results. Her spinal fluid was full of white blood cells – markers of inflammation — but in Wendy’s case, the doctors found no bacteria. Nevertheless, they started her on antibiotics. “Which ones?” Wendy mumbled to the doctor, who replied: “All of them.”

Over the next few days, despite the cocktail of antibiotics, Wendy’s condition deteriorated. When her name was called, she woke only briefly, babbling incoherently. Soon, she stopped following instructions altogether. Her lower legs remained immobile. Four days since that Monday morning, she stopped opening her eyes, unable to wake.

As the doctors stood around Wendy’s bed in the intensive care unit, they had reached a standstill: Her diagnosis was in question, treatments had no demonstrated effect, and Wendy’s life hung in the balance. Such standstills, which occur often in medicine, require reframing the story. Crazy suggestions are entertained. Old clues are reviewed under new light. At the suggestion of one of the neurologists, the team decided to repeat an MRI of her head. What had been an ordinary scan the first time around had transformed into a new film: A brain scattered with white blotches that heralded a promise of a diagnosis and a change in Wendy’s treatment.

The diagnosis:

Some years ago at the hospital where I worked, a crying woman came into the emergency room with her husband, who had woken up in the middle of the night with high fevers and chills. By morning, he had developed a purplish rash that spread down his legs. When he arrived, he was clammy, delirious, and unresponsive. We started him on intravenous fluids and antibiotics, but within hours of reaching the emergency room, he was dead. I had never before seen a man die so precipitously from illness. The blood drawn from his veins grew out a bacterium called Neisseria meningitidis, an organism that can enter the spinal fluid and cause a swift, and sometimes fatal, meningitis.

Did meningitis explain Wendy’s sudden decline? Probably not. Wendy was confused, and people with meningitis usually have a normal mental state because the disease affects only the tissues surrounding the brain.

But if the infection spreads into the tissue of the brain, a person may begin to behave bizarrely, hallucinating, shouting, or becoming lethargic or comatose. We call this encephalitis, which can be caused by a number of viruses, the most famous culprit in Massachusetts being the West Nile virus.

So did Wendy have some kind of encephalitis? The picture almost fits – encephalitis can cause problems with motor function, potentially explaining the immobility of the lower legs.

The MRI of Wendy’s brain illustrated white hyperintensities, a finding that can also be seen in people who are not ill. However, in patients with symptoms similar to encephalitis, such brain lesions can represent a rare illness, called acute disseminated encephalomyelitis, or ADEM. Unlike meningitis or encephalitis, ADEM is not directly caused by an infectious agent. Instead, ADEM is the result of damage caused by the body’s own immune cells, which target the tissue of the brain. Nobody really knows why the body’s immune system goes awry in ADEM, although some believe a prior infection, or even vaccination, may incite the immune cells to attack. Treating ADEM means shutting down the angry immune system, usually accomplished through the administration of steroids. That’s what Wendy got soon after the MRI. Just two days after the steroids were given, she opened her eyes.

Despite the suddenness of the onset of ADEM in Wendy the effects of the illness lasted far longer. While Wendy recovered her ability to speak, breathe, and eat on her own, the disease had caused significant damage to her brain and spinal cord, rendering her quadriplegic at the time of her discharge from the hospital. It was only following prolonged and aggressive treatment at Spaulding Rehabilitation Hospital, that nearly two months after the illness had begun, she was able to stand, walk, and – attempt a jog down the hospital corridors.

Wendy has recounted her story in a book, titled “To Get Back Home.” The disease erased the memory of the first few days she was ill and much of the time she spent in the hospital; the details of her story have been reconstructed through interviews with her doctors, family, and loved ones.

Do you have your own medical mystery? Dr. Sushrut Jangi of Beth Israel Deaconess Medical Center can be reached at sushrut.jangi@gmail.com.
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