The pacemaker implanted only two weeks earlier was sending out its regular signals, but doing nothing to the heart. Instead, the patient's right arm was twitching 100 times a minute. Earlier, he'd had abdominal spasms like hiccups, one per second, for hours on end.
That was the symptom that had brought this 75-year-old man to the emergency room at The Miriam Hospital, affiliated with Brown University's medical school in Providence, R.I.
An X-ray showed the cause: Twiddler's syndrome.
Every once in a while, fiddling with the bump created by a pacemaker will turn the tiny machine around and around, wrapping it up in its own wires. They pull away from the heart. If they're pulled high enough, a nerve leading to the diaphragm picks up the impulses. Even higher, and they hit a nerve to the arm.
It's not common. A letter in the April 24 issue of the New England Journal of Medicine, describing the incident, listed five earlier reports starting in 1968.
Dr. Hugh Calkins, a cardiologist at Johns Hopkins, said he has seen one case in 17 or 18 years. During that time, he said, he has implanted thousands of pacemakers and defibrillators, and followed thousands of additional patients who already had those devices.
"It's one of these curiosities," he said.
When the man described in the journal came in about a year ago, the chief residents on ER duty at The Miriam about a year ago couldn't figure out what the X-ray was showing them. They called in Dr. Peter Tilkemeier, who recognized it from a similar incident during his own cardiology training in the late 1980s. He'd had to call in a senior doctor, too.
"It gets a paragraph in a cardiovascular textbook - and that's a two-volume textbook," said Tilkemeier. "It's not something you see every day, but when you see it, it's important to recognize what it is."
This man was a rarity with a rare condition, Calkins said - usually, the wires just break, rather than making other parts of the body twitch or jump. Twiddler's syndrome is also rarer now than it was when the devices were much bigger, he said.
Elderly and obese patients seem to be at most risk, because loose tissue under the skin lets them spin the pacemaker in the little pocket created for it in the chest muscles, the journal report said.
Chemotherapy infusion pumps and implantable defibrillators also have caused Twiddler's syndrome, noted Tilkemeier and Drs. William J. Nicholson, now at Emory University, and Kathryn A. Tuohy, now at Brigham and Women's Hospital.
Making the pocket as small as possible and sewing the pacemaker to a nearby sheet of tissue will help prevent it, they said.
Calkins said he didn't think the problem was big enough to make sewing pacemakers in the standard procedure. It is standard to make the pocket just big enough for the device, and to tack down the wires, he said.
This man's wires were put back in place, and the pacemaker sewn in more securely.
New England Journal of Medicine: http://nejm.org
Brown Medical School: http://bms.brown.edu/med/