Transient Neonatal Myasthenia Gravis: A Case Report.

Transient neonatal myasthenia gravis (TNMG) is a neuromuscular disorder that occurs in infants born from mothers with myasthenia gravis (MG) due to transplacental transfer of antibodies against the acetylcholine receptor. TNMG is a rare form occurring in 10-15% of infants born from mothers with MG. We present a case of a newborn with TNMG with generalized hypotonia and respiratory distress. The newborn shows symptoms of hypotonia, weakened reflexes, poor crying, difficult sucking and potentiated tachydyspnea after 24 hours of birth and needs of assisted mechanical ventilation. Based on the mother's positive history of MG and the high titer of mother's (8.43nmol/l) and newborn's (9.088nmol/l) anti-AChR antibodies, TNMG was diagnosed. The baby was treated with assisted mechanical ventilation and neostig-mine until the anti-AChR antibody titer was negative. Adequate management of the newborn resulted in a positive outcome and evident withdrawal of the symptoms. Although TNMG is one of the rare neuromuscular disorders in newborns that can be treated, a multidisciplinary approach in the management of pregnant women with MG and newborns through timely diagnosis and early appropriate treatment, results in successful resolution of this condition.

Myasthenia gravis and pregnancy.

Myasthenia gravis (MG) is an autoimmune disorder with bimodal age of presentation, occurring in young women of reproductive age and at an older age in men. Occasionally, MG is diagnosed during pregnancy. Management of MG includes symptomatic treatment with cholinesterase inhibitors and immunosuppressive therapy for controlling the disease activity. Treatment of MG in women of reproductive age, who may be contemplating pregnancy, requires discussion regarding the choice of medication as well as the understanding of risks/adverse effects involved with various treatments. During the peripartum period, it is essential to ensure careful monitoring of the disease state along with the well-being of the mother and fetus and to coordinate neonatal monitoring overseen by a multidisciplinary team comprising a high-risk maternal fetal medicine specialist, a neurologist familiar with these complex issues, and a neonatologist.

[Neonatal hyperthyroidism: A sometimes challenging diagnosis]. / L'hyperthyroïdie néonatale : un diagnostic parfois difficile.

Graves disease complicates two pregnancies out of 1000 and when it is known before pregnancy, it warrants careful monitoring of the fetus and the newborn. We report on a case of neonatal hyperthyroidism, which revealed a previously unknown maternal thyroid disease. In this situation, neonatal signs can be misinterpreted, delaying the diagnosis. Neonatal hyperthyroidism is, however, a therapeutic emergency because of the risk of cardiac and neurological complications. The neonatologist must identify thyroid disease in the absence of a maternal history in order to promptly start therapy.

Marked hypotonia in an infant of a mother with Devic disease.

A full-term female neonate was born with severe hypotonia and weakness. Her mother had been treated for neuromyelitis optica (Devic disease) for 6 years. Her previous son, born 10 years earlier and before she developed the disease, also had marked hypotonia that gradually improved over several weeks. A suspicion of neonatal myasthenia gravis arose, as a search of the literature revealed the occasional detection of anti-acetylcholine receptor antibodies in patients with Devic disease. A neostigmine test was mildly positive in the baby, but anti-acetylcholine receptor antibodies were elevated. Aquaporin 4 antibodies typical of neuromyelitis optica were not detected in the infant. Because of clinical deterioration, intravenous immunoglobulin was administered with substantial improvement. Anti-acetylcholine antibodies were markedly elevated in the mother's serum, although she showed no clinical signs of myasthenia gravis. It is very likely that her previous baby also had unrecognized transient myasthenia gravis.

Myasthenia gravis in pregnancy and birth: identifying risk factors, optimising care.

Women with myasthenia gravis (MG) have increased risk of pregnancy complications and an adverse pregnancy outcome. This study examined risk factors for such complications in order to improve the care for pregnant MG women. Through the Medical Birth Registry of Norway, 73 MG mothers with 135 births were identified. Their obstetrical and clinical records were examined. Data on pregnancy, delivery and the newborn were combined with information on mother's disease. The risk for neonatal MG was halved if the mother was thymectomized (P = 0.03). Children with neonatal MG were more likely to display signs of foetal distress during delivery (P = 0.05). Only in one-third of the pregnancies did the patient see a neurologist during pregnancy. These patients used MG medication more often during pregnancy (P = 0.001), and were more likely to be thymectomized (P = 0.007). They also had a higher rate of elective sections (P = 0.009). Thymectomy may have a protective effect against neonatal MG. Neonatal MG can cause foetal distress during delivery. Most MG women benefit from being examined by a neurologist during pregnancy, to minimize risks and select the best delivery mode in collaboration with obstetricians.

Miastenia gravis: embarazo e impacto perinatal / Myasthenia gravis: pregnancy and perinatal impact

Se analizan 19 embarazos en 9 pacientes con miastenia gravis. Hubo 2 exacerbaciones de la enfermedad, una de esta asociada al uso de aminoglicósidos en el tratamiento de pielonefritis aguda. En un caso la enfermedad debutó a las 24 semanas de gestación. Hubo tres casos de miastenia gravis neonatal en la misma gestante, 2 de los recién nacidos fallecieron a los 29 y 25 días respectivamente, pese al tratamiento. Hubo un recién nacido de pretérmino y 6 casos (35 por ciento) de restricción del crecimiento intrauterino.

[The therapeutic strategy in transient neonatal myasthenia gravis]. / Principi lecenja transitorne miastenije gravis kod novordjenceta.

The article deals with 17 pregnant women of whom 16 were treated for juvenile myasthenia gravis since childhood or adolescence, and one of them since she was 22 years old. Four of them (23%) gave birth to babies with transient neonatal myasthenia gravis: three of them had one ill child and one two diseased children. The significance of ultrasound follow-up of the strength of foetal movements of foetuses in risk for transient neonatal myasthenia gravis is stressed. Frequent controls of antibodies titers to acetylcholine receptors in myasthenic pregnant patients are very important whithout regard to their previous condition, whether they were thymectomized or not, and whether they used anticholinesterase medicaments or not during pregnancy. If high antibodies titar to acethylcholinesterase receptors is detected in the mother, especially in combination with slowed foetal movements, the therapy of choice is plasma exchange. Treatment principles of babies with transient neonatal myasthenia gravis are discussed; they are based on the data from literature, and our personal experience.