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Neonatal myasthenia gravis is uncommon and life threatening. We describe the use of intravenously administered immunoglobulin, in addition to conventional modalities, in a neonate with severe neonatal myasthenia gravis. However, despite this aggressive management, the child had a prolonged period of weakness requiring intensive care.
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Transient neonatal myasthenia gravis is a postsynaptic neuromuscular transmission defect occurring in 21% of infants born to women with active (and, less commonly, in remission) acquired myasthenia gravis. Although passive-transfer acetylcholine receptor (AChR) antibodies are found in the majority of these newborns, their pathogenic role is questionable because only some infants are symptomatic. Pathogenesis in infants without AChR antibodies is unknown. There is still no biologic marker for prenatal identification of this subpopulation of newborns, although HLA typing may be a promising tool. Sucking, swallowing, and respiratory difficulties are the most common presenting signs in the first day of life. Final diagnosis is done when administration of acetylcholinesterase agents transiently corrects the neuromuscular transmission defect. Serum AChR antibody titers follow the same pattern as their mothers. Supportive management and anticholinesterase agents prior to feedings are necessary in about 80% of patients. In the majority of infants the condition resolves spontaneously.
BACKGROUND: The exact mechanism for the development of neonatal myasthenia gravis is unclear, and the occurrence of neonatal myasthenia gravis cannot be predicted. CASE: A case of normal duration of fetal forearm extention with return to flexion in response to vibroacoustic stimulation, and the pertinent details concerning neonatal myasthenia gravis and vibroacoustic stimulation test are described. CONCLUSIONS: Inasmuch as fetal acoustic stimulation test results in a fetal reaction resembling the startle response of the newborn, we think that it will worthwhile to perform this test antenatally in the next encountered fetuses of myasthenic mothers in order to assess its role in the prediction of neonatal myasthenia gravis.
A female infant with neonatal myasthenia gravis remained weak for an excessively long period compared to the usual situation in this disease process. At 71 days of age, electrophysiological studies revealed evidence of a defect in neuromuscular transmission that improved with intravenous Tensilon therapy. Repeat study at 183 days of age was normal. This case represents an unusually long time for an affected infant to have neonatal myasthenia gravis. Electrophysiological studies are of value in the diagnosis and management of patients with neonatal myasthenia gravis.
A preterm newborn had transient neonatal myasthenia gravis and was mechanically ventilated for 9 days. In addition to the usual supportive care, she was treated with neostigmine and underwent two exchange transfusions. High-dose intravenous immunoglobulin therapy (2 gm/kg) was used for the first time in transient neonatal myasthenia gravis to the best of our knowledge. The clinical and laboratory responses are presented.
An infant with transient neonatal myasthenia gravis had a double-blood-volume exchange transfusion because of maternal-fetal blood group incompatibility. This seemed to accelerate both decline in antiacetylcholine antibody titer and clinical improvement.
Neonatal myasthenia gravis has been described as a transient condition affecting only a small percent of neonates. We report a twin gestation in a seronegative mother with myasthenia gravis, in which only one twin was affected.
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Neonatal autoimmune diseases are thought to be due to the transfer of maternal autoantibodies. However, there is a puzzling lack of correlation between maternal autoantibody titres and disease in the neonate. So far, no factor reliably predictive of neonatal disease has been found. Agalactosyl IgG is a variable feature of normal IgG. Preliminary studies indicated that the percentage of agalactosyl IgG is lower in the serum of normal neonates, than in the serum of the mother at delivery. Since raised % agalactosyl IgG is often associated with autoimmune disease we sought to determine whether this relationship holds true in a neonatal autoimmune disease. We measured the % agalactosyl IgG in paired maternal-cord sera from patients with myasthenia gravis, some of whom had offspring with neonatal myasthenia gravis. We found that the percentage of agalactosyl IgG was significantly higher in affected than in unaffected neonates. Moreover % agalactosyl IgG was higher in sera of affected neonates than in serum from their mothers, while unaffected infants of mothers with myasthenia had %Gal(0) lower than their mothers, mimicking the normal situation. This suggests that in affected neonates a high proportion of the IgG is synthesised by the baby itself rather than derived from the mother. This agrees with previous evidence based on the presence of idiotypes not found in the mother which implied that the neonates with neonatal myasthenia gravis produce their own autoantibodies.
A case of neonatal myasthenia gravis is reported in the infant of an asymptomatic thymectomized mother with comparably elevated acetylcholine receptor (AChR) antibody titers. The mother remained asymptomatic despite elevated antibody titers while the infant became asymptomatic in association with the disappearance of the AChR antibody. It is suggested that the AChR antibody plays an essential role in the development of neonatal myasthenia gravis. It is also suggested that a thymic factor is necessary for the development of clinical symptomatology accounting for the lack of correlation between the clinical state of the mother and infant.
A premature infant with neonatal myasthenia gravis is presented to illustrate the utility of electrodiagnosis. The patient, born to a mother with myasthenia gravis, suffered additional problems, including hypoxia and subependymal hemorrhage which potentially contributed to hypotonia and poor respiratory effort, thus complicating the diagnosis. Standard testing with edrophonium originally was negative which cast doubt on the diagnosis; however, a repetitive motor nerve stimulation test demonstrated a significant decremental response which was consistent with neonatal myasthenia gravis. This decremental response was corrected following intravenous infusion of edrophonium. In the newborn with suspected myasthenia gravis, repetitive motor nerve stimulation may be a more reliable diagnostic procedure than the more frequently recommended pharmacologic tests. Use of this electrodiagnostic procedure in combination with pharmacologic testing may improve diagnostic accuracy in the premature infant and lead to earlier treatment.
Neonatal myasthenia gravis (NMG) with mild arthrogryposis was observed at birth in the newborn infant of a myasthenic mother. Anti-acetylcholine receptor (anti-AChR) antibodies were present in the serum of both mother and child, in umbilical cord serum and in the amniotic fluid. The presence of anti-AChR antibodies in the amniotic fluid may be one of the causes of the NMG.
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Transient neonatal myasthenia gravis (MG) is a human model of passively transferred MG. In an effort to understand the characteristics of the most pathogenic antibodies in MG, we studied the fine antigenic specificities of anti-AChR antibodies in sera from 21 MG mothers (nine of which had transiently transferred the disease) and 17 of their infants. Although in a few cases significant differences in antibody specificities were observed between mothers and infants, whether myasthenic or not, generally the antigenic specificities of the antibodies in sera from infants were very similar to those of their mothers. Furthermore, no characteristic differences were detected between the antibody repertoires of mothers who transferred the disease and those who did not.
Authors reported about their experiences with newborn infants, who had transient myasthenia gravis; one disease developed in the fetal, others 10 in the early neonatal age. Direct correlation was found between the development of maternal polyhydramnios and the severity symptoms in newborns: risk of neonatal myasthenia gravis increased at these infants. Specific treatment included blood exchange transfusions and pyridostigmin (Mestinon) medication for 2-10 weeks. Five transient myasthenia gravis responded readily to blood exchange transfusions. Authors pointed out that in the early neonatal period the aetiology of an obscure respiratory inadequacy and hypoventilation might be regarded as transient myasthenia gravis.