Neural Science and Vision – Intermission

We’ll take a break from extracting the visual highlights in Principles of Neural Science to look at a neuro case in today’s New York Times Sunday Magazine column on Diagnosis by Lisa Sanders, M.D. The print version begins: “A few months after bariatric surgery, the young mother found herself cripplingly weak, her eyes unable to focus. Did the operation cause this?”

Quoting from the online version: “The 21-year-old woman stood at the sink washing the bottles her baby went through that day. She was exhausted. As a plastic bottle filled with water, it suddenly felt strangely heavy. Her hands began to shake from the strain. She felt her legs threaten to buckle beneath her and grabbed the counter to keep from collapsing. “Mama, help me,” she called out …

Her mother persuaded her to go back to the hospital. She helped support her daughter’s weight as they walked to the car. Along the way, her daughter said she could barely see the ground. Every object was fuzzy, the whole world out of focus …

Dr. James Butler was the neurologist seeing patients in the hospital that week. The young woman looked tired; her face, impassive. She was slow to respond to the doctor’s questions, but when she did, her answers were reasonable. She wasn’t confused, just tired, and — based on what she’d told the E.R. doctor — depressed by how awful she felt since her surgery.

Butler’s examination of the patient focused on her nervous system. Three abnormalities concerned him. First, she didn’t seem to be able to move her eyes. “Follow the light with your eyes,” he instructed her as he moved a penlight to the right and the left, up and down. But she didn’t. She couldn’t, she told him. And when he asked her to smile — a way of testing facial-muscle strength — it was a pale whisper of a smile. Was she too depressed to participate in the examination? Or was this real weakness …

Butler was particularly struck by the eye exam. You could have paralysis of one of the six muscles that move the eye; but for all of them to be paralyzed, so that virtually no movement was possible? That would be extremely unusual. But he couldn’t believe this was a manifestation of her depression. He had seen patients whose depression made them feel that they couldn’t move their arms or legs — but eyes? Never.

There was another possibility, though an unlikely one. A rare disease called Miller Fisher syndrome (M.F.S.), an autoimmune disease that is a variant of Guillain-Barré syndrome (G.B.S.), affects the muscles of the eyes as well as those of the face, arms and legs, the usual target of G.B.S. Like G.B.S., it is often linked to a recent infection. This type of autoimmune disorder can be caused by antibodies created to fight off the infecting bug, which then, mistakenly, start to attack the nerves of the body. But this patient hadn’t had an infection.”

According to EyeWiki, Miller Fisher syndrome (M.F.S.), also known as Fisher syndrome and the Miller Fisher Variant of Guillain-Barré syndrome, is an autoimmune, antibody mediated neurologic disorder. M.F.S. typically presents as a self-resolving triad of ophthalmoplegia, ataxia, and areflexia, and is closely related to other diseases such as Guillain-Barré syndrome, Bickerstaff brainstem encephalopathy, and acute ophthalmoparesis, collectively known as the anti-GQ1b syndromes.

Butler’s associate, Yaniv Chen, took over the case from his partner and reviewed the record. Upon his examination, the patient had all three of the characteristic symptoms of M.F.S. Her eye muscles were still paralyzed. She still had no reflexes. And now that she was able to get out of bed, it was clear that her gait was abnormal.

Still, there were other possibilities as Dr. Sanders notes: “Was it some version of Lyme disease? This was Connecticut, after all. Or was it one of the several viruses that cause encephalitis? Could it be a different type of autoimmune disease — maybe myasthenia gravis? Although also rare, this frequently starts in young women. He looked for each of these. But even before the results came back, Chen was pretty confident that the patient had M.F.S. He started her on a five-day course of intravenous immunoglobulin — antibodies taken from donors — the usual treatment for both G.B.S. and M.F.S. Most of the results came in over the next few days. It wasn’t Lyme or one of the viruses that attack the brain. It wasn’t myasthenia gravis. But it was weeks before the test for M.F.S. came in. And that test was also negative. That surprised Chen … Everything favored that diagnosis except the test. The test is accurate 85 to 90 percent of the time. He suspected a false negative, but it was impossible to know for sure.

In any case, she had been treated for M.F.S., and over the course of her time in the hospital she started, slowly, to improve. Finally she was discharged to a rehabilitation hospital … She had to relearn everything, from eating with a fork and writing with a pen to simply walking. At this point, six months since returning home, her eyes only bother her when she’s tired.”