Case Report Describes NMOSD Rebound During Pregnancy

The rebound led to disability despite treatment, labor induction

Margarida Maia, PhD avatar

by Margarida Maia, PhD |

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An illustration shows a pregnant woman touching her stomach with one hand and holding a teddy bear in the other.

A young woman had a rebound of neuromyelitis optica spectrum disorder (NMOSD) toward the end of her pregnancy, for which she underwent plasma exchange and took steroids, according to a report from researchers in the U.S.

Because treatment with plasma exchange and steroids did not work well enough, the woman was offered an induction of labor to have her baby sooner and be cleared to start taking rituximab, an antibody that sometimes is used off-label in NMOSD.

Despite treatment with rituximab, the woman continued to experience weakness in her right hand that affected her ability to carry on with regular activities; she also had painful, sudden spasms that affected how she walked.

While plasma exchange may be a “viable option” for the treatment of NMOSD in pregnant women, “the optimal treatment of NMOSD during pregnancy is still unknown and likely unique to each patient,” the researchers wrote.

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The report, “Neuromyelitis optica spectrum disorder relapse during the third trimester of pregnancy,” was published in the journal Neuroimmunology Reports.

NMOSD is associated with loss of myelin, a fatty substance that forms a sheath around nerve cells, and with damage to the spinal cord and the optic (eye) nerves.

The disease occurs more often in women than in men. People with NMOSD may experience symptoms that include eye pain, poor eyesight, muscle weakness, loss of coordination, and changes in sensation. Some also experience transverse myelitis, which is when the spinal cord becomes inflamed.

The symptoms can worsen quickly and can be recurring. What triggers a recurring attack is unclear and can look different for every patient, but women with NMOSD are more likely to have a rebound soon after they give birth or end a pregnancy.

It’s not known how safe most of the treatments for NMOSD are for the fetus, and many women will stop taking their regular medication as soon as they plan to become pregnant. And, while being on immunosuppressants during pregnancy may reduce the risk of relapse, some women may see their symptoms return during pregnancy.

Case study detailed

Now, the researchers reported the case of a 20-year-old pregnant woman who visited the hospital with a worsening abnormal sensation in her right arm. She also was experiencing weakness in the left side of the body.

Two years before, she had received a diagnosis of NMOSD. Its first manifestations were transverse myelitis and attacks of nausea, vomiting, and hiccups, which are collectively referred to as “area postrema syndrome” in the context of NMOSD.

Treatment was with intravenous immunoglobulin (IVIG) to keep the immune system in check, and high doses of steroids to reduce inflammation. The  woman then was placed on maintenance treatment with rituximab, sold as Rituxan in the U.S. and MabThera in Europe and also available as biosimilars, for one year. During this time, she did not have any relapse. However, when she became pregnant, rituximab was stopped and instead she was started on monthly IVIG.

When she visited the hospital, she was 34 weeks into her pregnancy. A physical examination revealed decreased sensation to pinprick and temperature sensation on the left side of the body. The right side was overactive, which can occur when there’s muscle twitching.

An MRI scan revealed damage to the spinal cord at the neck’s level.

Doctors started her on five cycles of plasma exchange, also known as plasmapheresis. On the days she didn’t undergo plasma exchange, she received high doses of steroids. This eased her symptoms.

However, “careful monitoring of maternal and fetal health is needed during and post-treatment,” the researchers wrote. In this case, the fetus was monitored closely during plasma exchange and for six hours after, plus twice daily on the days the mother did not undergo treatment.

Inducing labor

After plasma exchange, the plan was to induce labor at 37 weeks of gestation to prepare the woman to start treatment with rituximab.

However, five days after the last session of plasma exchange, the woman returned to the hospital with worsening weakness in her right hand. So, to be able to start on rituximab, the woman was offered an induction of labor at 36 weeks and 6 days of gestation. The baby was born healthy.

Treatment with rituximab and steroids was started immediately after delivery. However, the woman developed an infection of the placenta and the amniotic fluid (chorioamnionitis) and preeclampsia, which is characterized by high blood pressure, protein in urine, and other symptoms. Moreover, two weeks after delivery, she complained of debilitating weakness in her right hand, and painful, sudden spasms that had spread to her legs and affected how she walked.

Stopping maintenance treatment during pregnancy may put women with NMOSD at risk of a relapse, the researchers concluded. This means that “accurate selection of short-term immunomodulatory agents is imperative to prevent the accumulation of severe disability.”