Assistance

18 6.6.21 Photo illustrations by Ina Jang

Diagnosis By Lisa Sanders, M.D.

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 fi lled with water, it sudden- ly 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 col- lapsing. ‘‘Mama, help me,’’ she called out.

The young woman had been to Griffi n Hospital, in nearby Derby, Conn., twice in the last two weeks. Since having bar- iatric surgery three months earlier, she had vomited several times a day, almost every day. She had been excited about the surgery but had no idea it would be this bad afterward. Sure, she lost weight: nearly 50 pounds. But she felt just awful since the operation, and lately the nausea and vomiting were even worse.

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.

In the E.R., the young woman wasn’t surprised to hear that her potassium was dangerously low or that a test of her kid- neys showed that she was severely dehy- drated. It’s what she was told the last two times she was there. This time, though, the decision was made to admit her.

The next morning the patient’s potassi- um was normal; so were her kidneys. But she was still too weak to get out of bed. And the world still looked blurry. Her mother demanded to know why she wasn’t better. The doctors weren’t sure. Potassium is one of the electrolytes muscles depend on to do their work. But once it was replenished, her strength should have returned. And so they called in the neurology team.

↓ Paralyzed Eyes

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

A few months after bariatric surgery, the young mother found herself cripplingly weak, her eyes unable to focus. Did the operation cause this?

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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 examina- tion? Or was this real weakness?

Despite her complaints of feeling weak, her arms and legs seemed strong. She could move her arms and legs even when he tried to hold her in place. Yet when he tapped her knees, ankles and the backs of her elbows, she had no refl exes. Tapping these locations causes a jerk because of a rapid-transit nerve circuit between the tapped tendon and the spinal cord. Loss of this refl exive jerk usually indicates an injury somewhere in the system.

All the tests they sent out once her potassium and fl uids were replaced came back normal. An M.R.I. of her brain and its blood vessels was also normal.

But clearly, her condition was not nor- mal. 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.), aff ects 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 infec- tion. This type of autoimmune disorder can be caused by antibodies created to fi ght off the infecting bug, which then, mistakenly, start to attack the nerves of the body. But this patient hadn’t had an infection.

Now deep into his career, Butler had seen many patients with G.B.S. as well as a few with M.F.S. Vomiting wasn’t part of the usual picture. But Butler recalled one patient he saw years earlier, when he was an intern, who had M.F.S. and who had come to the hospital with severe vomiting — just like this young woman. He never saw another case like it. But it’s good to be thor- ough. He ordered the blood test to look for the antibody associated with M.F.S.

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A Confounding Test Result

Butler’s associate, Yaniv Chen, took over at Griffi n later that week. He reviewed the patients his partner had been following and then went to see them. The young woman had gotten out of bed and taken a few wobbly steps. Her steps were pain- ful, even though her feet felt numb. ‘‘Am I going to die?’’ she asked the new doc- tor. Chen reassured her. He thought his partner’s suspicion that it might be M.F.S. was probably right. This disease, while serious, is not usually fatal, and over time most patients recover fully.

On exam, the patient had all three of the characteristic symptoms of M.F.S. Her eye muscles were still paralyzed. She still had no refl exes. And now that she was able to get out of bed, it was clear that her walk was abnormal.

Still, there were other possibilities. 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 diff erent type of autoim- mune 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 confi dent that the patient had M.F.S. He started her on a fi ve- day course of intravenous immunoglobu- lin — 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. This was a reliable test. So maybe it wasn’t M.F.S. after all. But what else could it be?

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Nutrient Deficiencies?

Bariatric surgery often results in nutri- tional defi ciencies. And she’d been vom- iting for months; that, too, depletes nutri- ents. Her presentation, with its strange eye paralysis and loss of strength and refl exes, wasn’t typical of any of the single nutritional defi ciencies Chen could think of. But perhaps she had multiple defi cien- cies and together they caused this unusual constellation of symptoms.

He ordered tests to assess the levels of the vitamins and minerals known to be aff ected by bariatric surgery that could cause weakness: vitamins B12, B1, C, D and E, zinc, copper. Once the tests were drawn, he started her on replacement levels of these vitamins. Those test results were also unsatisfying. The B12 and thiamine (B1) were normal. So were the zinc and copper levels. Her vitamin C was undetectable, and her vitamin A and D were low; she was already on a high-dose multivitamin, so those were covered. But he wasn’t cer- tain these vitamin defi ciencies would have caused her symptoms. And if they had, she should have gotten better once the vitamins were replaced. She didn’t.

And so, Chen found himself back where he started: Could this be M.F.S.? Every- thing 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.

The patient, now home, tells me that her recovery has been maddeningly slow. She had to relearn everything, from eat- ing with a fork and writing with a pen to simply walking. At this point, six months since returning home, her eyes only both- er her when she’s tired. She uses a walker less and less. She’ll feel like she’s really recovered, she says, when she can stand and pick up her child. She’s not there yet, but soon, she thinks. Very soon. �

Lisa Sanders, M.D.,

is a contributing writer for the magazine. Her latest book is ‘‘Diagnosis: Solving the Most Baffl ing Medical Mysteries.’’ If you have a solved case to share with Dr. Sanders, write her at Lisa [email protected].

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