An Unusual Variant
But highest on Unia’s list of suspects was an unusual version of Guillain-Barré syndrome (G.B.S.) known as the Miller Fisher variant. G.B.S. is also an autoimmune disorder, usually triggered by an infection. Antibodies created to fight the infection mistakenly attack the nerves that control movement, usually starting with the legs and ascending up the body. In the Miller Fisher variant of G.B.S., the disease attacks the nerves controlling the muscles of the head and neck as well as those of the feet and legs, causing double vision and difficulty swallowing.
Unia ordered the blood test that looks for this version of G.B.S. But the results could take weeks. In the meantime, the neurologist decided to treat him even without this proof. Treatment involves suppressing the wayward immune system — first with steroids and then, if needed, with intravenous immunoglobulin (IVIg), an infusion of antibodies that block the destructive ones of G.B.S.
New Developments in Cancer Research
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Progress in the field. In recent years, advancements in research have changed the way cancer is treated. Here are some recent updates:
Chemotherapy. A quiet revolution is underway in the field of cancer treatment: A growing number of patients, especially those with breast and lung cancers, are being spared the dreaded treatment in favor of other options.
Leukemia. After receiving a new treatment, called CAR T cell therapy, more than a decade ago, two patients with chronic lymphocytic leukemia saw the blood cancer vanish. Their cases offer hope for those with the disease, and create some new mysteries.
Esophageal cancer. Nivolumab, a drug that unleashes the immune system, was found to extend survival times in patients with the disease who took part in a large clinical trial. Esophageal cancer is the seventh most common cancer in the world.
The patient had just finished his last day of treatment when the hospitalist Dmitry Opolinsky took over his care. Opolinsky asked him how he was feeling. Much better, the patient exclaimed, but then exploded into a paroxysm of coughing. Unia had warned Opolinsky that the patient was eager to feel better but that his exam had not really changed since his arrival. Still, it often takes a week or two for any improvement following the IVIg. They needed to give him time.
As he waited for his patient to start to get better, Opolinsky kept his eye on the results that were still trickling in. His vitamin B12 was normal. So were the other vitamin deficiencies he was tested for. The biggest disappointment came from the Mayo Clinic, where the neuroimmunology lab looks for evidence of any of a dozen paraneoplastic syndromes. They were all negative. This probably was G.B.S., though nearly a week after treatment, the patient was no better.
The next day Opolinsky got a text to call a number he didn’t recognize. The voice, deep with a hint of an Irish accent, identified himself as Dr. Andrew McKeon, co-director of the Mayo Clinic lab. “Oh, yes, we got those results yesterday,” Opolinsky told him. “All negative.” Actually, McKeon interjected, that result was wrong — or rather, incomplete. There was a newly discovered antibody identified a couple of years earlier by McKeon’s lab, still so new that it had not yet made it onto the automated result form used in testing. This patient had a very strong positive result for this antibody, which attacks something known as neuronal intermediate filaments in the brain. “If he’s a smoker,” McKeon predicted, “then he has small-cell lung cancer. If he’s not, he probably has Merkel cell skin cancer.”
The patient had never smoked, so Opolinsky focused on the possibility that he had this rare type of skin cancer. What Opolinsky remembered from his training was that Merkel cell carcinoma was an aggressive form of skin cancer caused by sun damage and had a much higher rate of spreading than most other forms of skin cancer. He looked at the images on the internet, which showed a bluish red nodule, usually found on the head or face. He didn’t find any of these odd growths, but he ordered a CT scan to look for metastases. There, deep in the patient’s left underarm, was an enlarged lymph node — about the size of a lime. Opolinsky hurried to the patient to feel for the mass, yet even knowing it was there, he couldn’t locate it. But the surgeons could, and removed a mass that tested positive for Merkel cell carcinoma. After his surgery, a PET scan showed that he was free of cancer.
It took some time, and an immune-suppressing medication, but slowly the patient began to recover. That was this past summer. These days he can walk, but only with a walker. And he still coughs a lot. But he’s hopeful that, come spring, he’ll be back on his tractor, even if he has to get onto it from the porch.
Lisa Sanders, M.D., is a contributing writer for the magazine. Her latest book is “Diagnosis: Solving the Most Baffling Medical Mysteries.” If you have a solved case to share, write her at [email protected]