Mysterious neurologic symptoms may have saved this patient’s life
Medical Mysteries is a column about the diagnosis and treatment of complicated medical cases at Brigham and Women’s Hospital (BWH).
At some point, Jim Valdes’s immune system began attacking his spinal cord. The repeated assaults from his own antibodies weakened his nervous system’s ability to send messages from his brain to his legs.
This condition went on silently as Jim, a 64-year-old semi-retired engineer, consulted part-time at the Woods Hole Oceanographic Institution, where he’d spent four decades designing underwater submersibles, an exciting career that had taken him to the North and South Poles. A lifelong athlete, Jim rode his bike about 50 miles a week on Cape Cod.
Jim Valdes, a cycling enthusiast, bikes 50 miles a week. One morning, he woke up numb from the chest down.
One morning, in the summer 2015, the damage to Jim’s spinal cord hit a perilous tipping point.
“I woke up and was numb from the chest down,” he says.
Jim’s wife drove him to the local emergency room. The doctors discharged him hours later with a medication for Lyme disease.
The next morning, Jim could barely feel his legs. At the ER, an MRI showed an inflamed spinal cord. He was rushed to Brigham and Women’s Hospital (BWH) in Boston.
Summer in the neurology clinic
Jim spent July and August in BWH’s Department of Neurology, as doctors tried to find out why he’d lost sensation in his legs.
Tests ruled out Lyme and common infections, such as Herpes and varicella zoster virus. Routine blood work was normal.
However, the fluid in Jim’s brain and spinal cord showed high levels of white blood cells, suggestive of an inflammatory condition. More blood tests confirmed that his immune cells were fighting something.
Radiologist spots a thickening in the esophagus
A diagnosis eluded doctors until a radiologist examined a CAT scan and spotted a slight thickening in Jim’s esophagus, the tube that connects the throat to the stomach. There appeared to be a mass in the lower part of his esophagus.
This prompted an upper endoscopy, a procedure that allows a gastroenterologist to visualize and biopsy the inside of the esophagus.
The fluid in Jim’s brain and spinal cord showed high levels of white blood cells, suggestive of an inflammatory condition.
A biopsy confirmed adenocarcinoma, a cancer of the esophagus that affects 18,000 Americans a year, according to the American Cancer Society.
“Up to that point, my only health problem had been high cholesterol. I could barely feel my legs. And now I had cancer,” says Jim.
The good news was that the cancer hadn’t left the esophagus. However, doctors still didn’t have a connection between Jim’s tumor and his leg weakness.
A rare syndrome with ‘quirky’ symptoms
“The neurological symptoms were strange in the context of esophageal cancer. We began to suspect a paraneoplastic syndrome,” says Daniel Wiener, MD, a thoracic surgeon at Brigham, who specializes in treating cancers of the lung and esophagus.
A paraneoplastic syndrome occurs when a patient’s own cancer-fighting antibodies target healthy cells. It’s an autoimmune reaction, like multiple sclerosis, and it can cause “quirky” symptoms, says Dr. Wiener, including nerve and muscle damage.
Jim was suffering from a type of paraneoplastic syndrome known as transverse myelitis, a neurological disorder that damages the insulation covering nerve cells.
The harmful antibody responsible for the damage targets a receptor that is expressed in high amounts on the surface of spinal cord nerve cells.
Transverse myelitis can disrupt messages from the spinal cord nerves to the body, causing pain, muscle weakness, paralysis, sensory problems, or bladder and bowel dysfunction.
A canary in the coal mine
While devastating, the paraneoplastic syndrome was an early warning sign of cancer. Had Jim’s immune system not overreacted, the tumor in his esophagus might have spread to his lymph nodes, liver or lungs.
“His prognosis could have been very different had the tumor grown for another 6-12 months,” says Dr. Wiener.
Had Jim’s immune system not overreacted, the tumor in his esophagus might have spread.
Now that doctors had diagnosed the inflammatory condition and the cancer, they turned their attention to treatment.
Treat the tumor, treat the autoimmunity
“Jim’s an engineer, so he asked a lot of questions,” says Dr. Wiener. “I valued that, because he wanted to understand the rationale behind our decisions.”
The doctors first tried to blunt the immune response with a course of steroids, but Jim’s legs went numb two weeks later.
“When Jim didn’t respond to our next treatment, where we removed the harmful antibodies from his blood, we decided to treat the tumor directly,” says Dr. Wiener.
When Jim didn’t respond to our next treatment, we decided to treat the tumor directly.
Since the cancer hadn’t metastasized, and conventional cancer treatments could have caused further neurological damage, a multidisciplinary team of surgeons, oncologists and radiologists chose to skip chemotherapy and radiation and surgically remove the tumor.
Jim underwent an six-hour-long surgical procedure, known as a esophagectomy, where a portion of Jim’s esophagus was removed and reconnected to the stomach.
After surgery, Jim spent several days in the intensive care unit, and two weeks in the hospital, hooked to a feeding tube.
Learning how to walk again
“Jim’s nerves weren’t going to start working immediately after we removed the tumor,” says Dr. Wiener. “But at least they wouldn’t be continually injured by the antibodies.”
Jim’s blood slowly cleared of the harmful antibodies, and his neurological symptoms began to fade.
He focused on physical therapy, regaining the use of his legs. Each day, he walked a mile up and down Brigham’s halls. Using a walker, he says his movements consisted of “Move foot, move foot, move walker, move foot, move foot.”
Jim was discharged after making a “remarkable recovery,” says Dr. Wiener.
Dr. Wiener asked Jim to contact him so that he could monitor his healing. It was a uniquely close doctor-patient relationship, which Dr. Wiener says was rewarding.
Getting the nerves to find new pathways
Back on Cape Cod, Jim threw himself into in-home physical therapy. The activity encouraged his damaged spinal nerves to find alternate pathways.
Jim’s neurologist, Tamara Kaplan, MD, likened the process to navigating through traffic in rush hour. A straight path through traffic takes a long time, she said, but backroads are faster.
Jim returned to the gym and began cycling again. He went back to Woods Hole Oceanographic Institution to work part-time on an instrument he designed in the 90’s.
Jim’s cancer is in remission. The odds that it will reoccur are relatively low.
He still has symptoms, including weakness and tingling in his legs, but they haven’t prevented him from resuming his daily activities.
“In terms of prognosis, transverse myelitis is typically a relapsing condition, so I can’t provide exact odds on Jim making a full neurologic recovery, but his cancer is in remission and the odds that it will reoccur are relatively low,” says Dr. Wiener.
They follow-up every four months for scans and blood work.
A personal milestone
A year after his ordeal, Jim was riding his bike 50 miles a week. After racking up 75 miles, with one day left in the week, he kept riding and achieved 100 miles. He emailed a selfie to Dr. Wiener. “I called it my centennial week,” he says.
- By Dustin G.