He Had Insomnia, No Appetite and Started Conversing With Dead People. What Was Going On?
Started Conversing With Dead People. What Was Going On?
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The woman found her 68-year-old husband just as she’d left him, lying on the bed, staring straight up at the ceiling — awake but not aware. He’d been sick and confused since he came home from the hospital three days before. She called his name. He didn’t respond. She shook his shoulder — he was stiff.
When the E.M.T.s arrived, they found that the man’s blood-sugar level was dangerously low. The technicians quickly gave him some glucose. It was probably a seizure triggered by his low sugar, they thought.
In the emergency room of the Yale New Haven Hospital, the patient’s cubicle swirled with activity as IVs were placed, blood was drawn and information confirmed. Doctors came through, asking questions and examining the man. When all was finally quiet, a young physician introduced himself as Dr. Clark Fisher, a resident in his first year of training and the doctor assigned to the man’s care.
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A Bad Year
The electronic medical record showed that almost exactly a year earlier, the patient developed a cough and a sharp pain when he took a deep breath. A CT scan revealed a mass a little smaller than a hockey puck in his upper chest. It was a thymoma, a tumor in the thymus gland. This gland, located just below the neck, is essential in childhood, helping the immune system develop. By puberty, the gland’s work is done, and it shrinks. A tumor in this gland is rare, usually affecting people in their 60s. The patient had completed his cancer treatment with chemotherapy, surgery and radiation a few months earlier.
The patient was asleep when Fisher arrived. He responded to questions, but his answers were short and often uninformative. His wife filled in the gaps, explaining that she and her husband retired the year before, expecting to spend the remaining years of their lives traveling and seeing family and friends.
That was still their plan, but it had been a terrible year, she told Fisher. Things had finally been getting better — until three weeks earlier, when her husband ended up in the hospital with a hugely distended abdomen. Imaging suggested that his intestines were blocked. When the problem persisted, the decision was made to operate. But in the operating room, the surgeons couldn’t find any type of blockage. The patient had what’s known as an ileus — a bowel that temporarily stops working — usually in response to severe illness. It wasn’t clear why her husband had developed one.
A Different Man
In the hospital, her husband wouldn’t eat or sleep. He talked to people who were long dead. And yet, if you asked him, as the doctors often did, where he was and why he was there, he could tell you that he was in the hospital because he was sick. He also knew the day and the year. The doctors sent him home, telling his wife that he would probably do better in a familiar setting. But at night he wandered through the house, combing through closets, drawers and cupboards. She asked him what was he looking for. He didn’t know. She brought him back to bed a dozen times each night.
When she returned with her husband to the hospital three days later, the doctors said that his sugar was probably low because he was still taking his usual medications for diabetes but wasn’t eating. Why, she wanted to know, wasn’t he eating? And why was he acting so strange?
They’d figure it out, Fisher assured the distressed woman. On exam, the man’s heart was racing. His blood pressure was erratic — one moment high, the next low. On his abdomen, there was a long surgical wound from the search for the obstruction the week before. The staples holding the edges of the wound together looked like a zipper, but one was open an inch at the bottom. To Fisher, infection seemed the likeliest cause of his symptoms.
More Symptoms
After discussions with the rest of his team, Fisher started the patient on broad-spectrum antibiotics. If this was an infection, it might be one picked up during his last hospital stay, and those bugs could be resistant to the usual drugs. The surgeons were confident that the wound wasn’t infected and recommended keeping it covered.
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Over the next 48 hours, the man developed other symptoms: Tiny muscles in his face and legs began to twitch in what is known as fasciculations, causing him to make strange expressions. He’d slur his words as if his tongue had forgotten how to speak. But moments later, his speech would be clear. He was agitated at night, then sleepy all day. It could be delirium, Fisher reasoned, but it was the strangest version he ever saw. Still, he was just halfway through his first year of training. There was plenty he didn’t know.
At home, on his day off, Fisher found himself worrying about the patient. He turned to the internet. When trying to diagnose, one trick Fisher was taught was to focus on the most unusual symptoms. These would have the fewest number of causes, and if you could find some overlap in the causes for two of the rarer symptoms, then the diagnosis would most likely lie in that region of overlap. Fisher started with the mysterious ileus and added the strange twitches he saw. But nothing fit.
Fisher added the thymus tumor to the search. And yes! Several autoimmune disorders linked to thymomas could cause neurological symptoms. Myasthenia gravis was the most common — a disease in which muscles weaken quickly because of an antibody that interferes with the nerves’ ability to tell the muscles to move. But this wasn’t the patient’s problem, so Fisher kept looking. Finally, he found one that seemed to include all the man’s symptoms. It was a rare disorder known as Morvan syndrome, often associated with a tumor in the thymus gland. At the end of the 19th century, the French physician Augustin Marie Morvan described four patients with neurological abnormalities, caused, he hypothesized, by an overcharged nervous system, which could result in many of the symptoms the patient experienced: a rapid heart rate, wildly variable blood pressure, fasciculations, insomnia, hallucinations, ileus and loss of appetite. This syndrome is now understood to be a disease in which errant antibodies interfere with the on-off switch of certain nerve cells.
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Confirming a Hunch
Fisher was excited by his find but knew that his team would be skeptical. Residents were often reminded to look for horses rather than zebras when hearing hoofbeats. He recalled that neurology was consulted about the patient’s muscle twitches when he was last in the hospital. Fisher reviewed the consultants’ note. They, too, had thought of a thymoma-triggered disorder. They’d sent blood to the Mayo Clinic to look for proof. Fisher called the Mayo Clinic lab and found that the tests were positive for the antibodies linked to Morvan. Positive results were so unusual that they had to double-check them before the answer could be officially reported.
That was enough for Fisher. The neurologists were consulted again. Even without the final results, they recommended action. The way to treat a disease caused by wayward antibodies is, paradoxically, to add more antibodies. Certain autoimmune diseases can be slowed or even stopped by the infusion of antibodies culled from other people’s blood, a treatment known as intravenous immunoglobulin, or IVIG. It isn’t clear why this works, but it does.
Over the next few months, the patient got six courses of IVIG. And slowly he started to recover. First, he was able to sleep at night. He became less confused. The twitching muscles stopped. His appetite came back. Finally, by the end of the treatment course, he was back to normal.
The patient’s wife considers his recovery a full-on miracle. They are planning to go on a family cruise in the spring, the first, she hopes, of many. In the meantime, the patient has taken up tai chi to help get his body into peak traveling form.
Lisa Sanders, M.D., is a contributing writer for the magazine and the author of “Every Patient Tells a Story: Medical Mysteries and the Art of Diagnosis.” If you have a solved case to share with Dr. Sanders, write her at Lisa.Sandersmd@gmail.com.
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