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Posted by Dr. Sushrut Jangi July 19, 2013 05:00 PM
This is the answer to the diagnostic mystery I posted earlier this week about a patient named Wendy.
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. Consequently, patients with meningitis may have pounding headaches, a stiff neck, light sensitivity, and high fevers. 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, which was found in 5 percent of mosquitoes last year and caused a record number of 33 human cases in a single season.
Numerous other viruses can cause encephalitis too, but many of these are difficult to find in the spinal fluid - our testing isn't perfect. So lots of cases of viral encephalitis end up going undiagnosed. 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. Had a neurologist not asked for a repeat MRI, viral encephalitis probably would have been her diagnosis.
The MRI of Wendy's brain illustrated white hyperintensities, a finding that can also be seen in normal people. 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 feel 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 was read. Just two days after the steroids were given, she opened her eyes.
Despite the suddenness of the onset of ADEM in Wendy (this illness causes dramatic decline in a matter of days) the effects of the illness lasted far longer. While Wendy recovered her ability to speak, and 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 - with great effort, attempt a jog down the hospital corridors.
A sudden illness - one that began in a matter of days - reached its grasp around Wendy's life weeks, months, even years after it first began. The impact has had such resonance on Wendy's perspective that she has catalogued her story in a book, titled To Get Back Home. The disease has erased the memory of the first few days she fell ill and much of the time she spent in the hospital; the details of her story have been reconstructed through her interviews with her doctors, family, and loved ones. I've noticed stories of illness are rarely told by only one narrator; whenever I walk into a hospital room and ask the patient what happened, family often jumps in - "No, mom, tell them about your hand," or "That's not what happened, Dad!" Like memory, the impact of disease is shared. It is this sharing that can make illness so terrible and recovery so meaningful.
Wendy Chapin Ford recounts the entire story of her illness in the book she has authored, "To Get Back Home: A Mysterious Disease: A Fight for Life."
The author is solely responsible for the content.