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Introduction: The course of double-seronegative myasthenia gravis (DSNMG) during and after pregnancy has not been well described., Objective: To assess the course of DSNMG during pregnancy and within 6 months postpartum., Methods: A retrospective cohort study of women with DSNMG seen in the Duke Myasthenia gravis (MG) Clinic after 2003., Results: Review of the Duke MG Clinic Registry and electronic medical record identified 8 patients who became pregnant after MG onset; the mean age at disease onset was 17.6 (SD = 10.0) years. Increased MG symptoms were observed in the first and third trimester and, most commonly, postpartum in 6 of 18 pregnancies. Except for 1 infant who developed respiratory distress that required neonatal intensive care admission, all the newborns were healthy at birth., Conclusions: As in seropositive MG, increased MG symptoms during pregnancy and within 6 months postpartum is also seen in women with DSNMG., Competing Interests: The authors report no conflicts of interest., (Copyright © 2023 Wolters Kluwer Health, Inc. All rights reserved.)

Purpose of Review: This article provides an overview of neuromuscular disorders in pregnancy, with a focus on diagnosis and management., Recent Findings: Neuromuscular disorders with issues that occur in pregnancy include conditions that are acquired (including autoimmune) or genetic; each requires a unique approach to management and treatment prepartum, peripartum, and postpartum. Guidance in the literature regarding management and treatment options is predominantly from case series and retrospective reviews. Treatment can be complex, particularly in autoimmune neuromuscular diseases, because of the risks of side effects of the treatments that may affect the patient and fetus., Summary: This article summarizes expectations, diagnosis, and management for a wide range of neuromuscular disorders in pregnancy., (Copyright © 2022 American Academy of Neurology.)

Purpose of Review: Myasthenia gravis (MG) is an acquired autoimmune disorder characterized by fluctuating ocular, limb, or oropharyngeal muscle weakness due to an antibody-mediated attack at the neuromuscular junction. The female incidence of MG peaks in the third decade during the childbearing years. A number of exacerbating factors may worsen MG, including pregnancy. When treatment is needed, it must be carefully chosen with consideration of possible effects on the mother with MG, the pregnancy, and the fetus., Recent Findings: Decisions are complex in the treatment of women with MG contemplating pregnancy or with presentation during pregnancy. While data is largely observational, a number of characteristic patterns and issues related to risk to the patient, integrity of the pregnancy, and risks to the fetus are recognized. Familiarity with these special considerations when contemplating pregnancy is essential to avoid potential hazards in both the patient and the fetus. Use of immunosuppressive agents incurs risk to the fetus. Deteriorating MG with respiratory insufficiency poses risk to both the mother and the fetus., Summary: This article reviews available information regarding expectations and management for patients with MG in the childbearing age. Treatment decisions must be individualized based on MG severity, distribution of weakness, coexisting diseases, and welfare of the fetus. Patient participation in these decisions is essential for successful management.

As with several autoimmune diseases, myasthenia gravis (MG) occurs frequently in young women in their childbearing years. The treatment of MG in women therefore poses unique and challenging issues to neurologists, obstetricians, and neonatologists as the safety of both mother and fetus needs to be carefully considered when choosing a therapeutic plan. The severity of generalized weakness and the potential for respiratory insufficiency and myasthenic crises in the mother should dictate how aggressive a treatment plan should be. The potential effects of immunosuppressant medications on the fetus should always be weighed against the risk of myasthenic crises and its potential to endanger both mother and fetus. Successful management of MG during pregnancy and in the postpartum period is possible in many cases but requires collaboration between the obstetrician, the neurologist, and a well-informed patient. The neurologist should be able to counsel women and discuss treatment options and pregnancy risks based on the best current knowledge, so that women will be able to make an informed decision and successfully complete pregnancy.

We report the use of single fiber electromyography (SFEMG) to demonstrate changes in the physiologic abnormality of myasthenia gravis (MG) during pregnancy. A 23-year-old became pregnant 15 months after the onset of mild ocular weakness. On initial evaluation, SFEMG jitter measurements demonstrated a slight abnormality of neuromuscular transmission. There was no change in severity of clinical disease or jitter measurements until the third trimester, when she improved. Jitter measurements at that time were normal. Labor was normal and she delivered a normal male. Three days postpartum, myasthenic weakness recurred temporarily and jitter measurements showed worsening. At 16 days and 6 weeks postpartum, she had only minimal medial rectus weakness and jitter studies were normal. Three months postpartum, ocular symptoms recurred and jitter measurements were slightly abnormal. She continued to worsen, developing limb muscle and severe ocular muscle weakness at 4 months postpartum. She was treated with plasma exchange and thymectomy. Prednisone was added 2 months after thymectomy due to continued worsening and development of oropharyngeal weakness. Three years postpartum she was taking prednisone 10 mg every other day and had only slight weakness of neck flexors, and jitter studies were again normal.