Research Paper on Juvenile Myasthenia Gravis (JMG): Causes, Symptoms & Treatment

Introduction

Juvenile Myasthenia Gravis (JMG) is an autoimmune neuromuscular infection in children. Myasthenia gravis frequently affects adults but it affects children too. There are three types of Myasthenia Gravis (MG), Transient neonatal Myasthenia Gravis, Juvenile Myasthenia Gravis, and Congenital Myasthenia Gravis. The disorder causes muscle weakness due to impaired neuromuscular transmission. The inappropriate communication between nerves and muscles is caused by abnormal antibodies that break up neuromuscular connections. The Myasthenia Gravis have a direct effect on voluntary muscles also known as the skeletal muscles of the body causing muscular weakness. The condition mostly affects the throat, legs, eye, arms, and mouth. The muscles affected and the extent of muscle weakness caused range from one individual to another. The common cause of this disorder is an acquired immunological abnormality. However, sometimes it can be as a result of genetic malformation at the neuromuscular junction.

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Pathophysiology of Myasthenia Gravis

In neuromuscular transmission, acetylcholine (ACh) is released by the normal neuromuscular junction in quanta packages from the motor nerve terminal. After the release, acetylcholine discrete package moves across synaptic cleft through the diffusion process to bind to numerous receptors found on the membrane of the overlapped muscle endplate (Kulikova, 2015). Once the motor nerves have been stimulated, it releases a lot of acetylcholine discrete packages that modify specific muscle's endplate causing the targeted muscle to contract. In the case of acquired immunological abnormality, there is a deformation of the postsynaptic membrane of the muscle because it loses its normal shape due to the effects of acetylcholine (ACh).

Acetylcholine receptors concentration decreases on the membrane of the muscle end-plate causing the attachment of the antibodies to the membrane. In this case, there is a normal secretion of Ach, however, it does not intensely affect the post-synaptic membrane. As a result, the post junction membrane becomes numb to acetylcholine (Ach) (Mishra, 2018). Therefore, reduced acetylcholine functionality in the synapses reduces proper nerve impulses transmission in the muscles leading to muscle fatigue and weakness (Hirata et al., 2016).

Signs and Symptoms of Juvenile Myasthenia Gravis

According to Kulikova (2015), juvenile myasthenia gravis form varies on how it manifest as sometimes it imitates other infections, causing numerous challenges in its early diagnosis. For instance, noticing antibodies in children's blood is more challenging than in adults. Some of the most common symptoms of Juvenile myasthenia gravis include mild to severe muscle weakness, double vision, and drooping eyelids. Both double vision and drooping eyelids are caused by an earlier form called ocular myasthenia, damaging muscles around the eye. Additional signs and symptoms include difficulty in swallowing or speaking, fatigue, clumsiness, falling easily due to difficulty in walking, and titled facial expression. However, not all patients exhibit all the listed signs and symptoms (Hirata et al., 2016). Symptoms do vary from time to time depending on the extent of the disease.

Patient and Parent Education

Mishra argued that it is important to carry out awareness of the dangers of myasthenia gravis among children to enable parents to identify, report and immediately seek medication to minimize the adverse effects of the impending health crisis (2018). When the affected person has some infection, myasthenia gravis (MG) symptoms may be worsened. In hot weather or hot environment, victims usually experience mild exasperation of weakness. It is important to note that women with severe myasthenia gravis have higher chances of giving birth to children with congenital abnormality (arthrogryposis multiplex) (Hirata et al., 2016). Additionally, neonates children born to parents with myasthenia gravis have a higher chance to experience respiratory failure, therefore, they should always be under intense care for the first two weeks after being born. Parents should also be aware of how they use some of the immunosuppressant drugs during pregnancy since some of these drugs contain teratogen which can cause congenital malformation.

Nursing Interventions

For effective nursing intervention, the nurses need to take both subjective and objective data of the patient. Using both data, the nurse should first provide cholinesterase inhibitors in time and proper dosage. Administration of these inhibitors will help in maximizing acetylcholine functionality at the nerve synapses thus accelerates impulse conduction within the affected muscles (Hirata et al., 2016). Respiratory condition of the patient should always be monitored to prevent respiratory distress. Additionally, due to ptosis and eye muscle weakness, eye care should be considered to reduce eye irritation. Proper nutrition, patient and parents education regarding triggers plays a crucial role in ensuring patients stay safe (Hirata et al., 2016). When the nurse observes all the appropriate interventions, problems such as cholinergic crisis and myasthenic crisis can be minimized.

Medications and Side Effects

Myasthenia gravis cannot be cured, however, numerous medications can be administered to assist manage symptoms and help strengthen the patient's muscle (Kulikova, 2015). Depending on the parent and child's particular needs and medical conditions, one or more treatments may be recommended, either non-surgical or surgical treatment (Hirata et al., 2016). There are two main types of non-surgical medications, provision of acetylcholine esterase inhibitors, and immunosuppressive therapy. The provision of acetylcholine esterase inhibitors increases the level of acetylcholine that helps in nerve-muscle communication. Pyridostigmine also referred to as Mestinon is the common prescription as it is most effective for acute symptoms. Muscle cramping and diarrhea are some of the side effects associated with this medication. In immunosuppressive therapy, intravenous immunoglobulin and or oral steroids are used to stabilize the patient. In this medication, the patient's exposure to these drugs is regulated to reduce side effects. Surgical treatment, on the other hand, focuses on the thymus removal to help minimize medication usage and trigger remission.

Developmental Considerations

The development of this disorder often begins during pregnancy when maternal acetylcholine receptor (AChR) antibodies are transferred to the fetus across the placenta (Mishra, 2018). This causes neuromuscular transmission abnormality in the neonate. At birth, the baby appears to be normal. Thereafter, the newborn begins to exhibit signs and symptoms such as weak cry, the floppy baby syndrome also known as hypotonia, ptosis and facial fatigue, and difficulty in breathing demanding mechanical ventilation.

References

Hirata, Y., Inoue, M., Nabatame, S., Okumura, M., & Ozono, K. (2016). Multidisciplinary treatment for prepubertal juvenile myasthenia gravis with crisis. Pediatrics International, 58(8), 772-774. https://doi.org/10.1111/ped.12957

Kulikova, S. (2015). OP43 - 2438: Generalized juvenile myasthenia gravis: Long-term outcome. European Journal Of Paediatric Neurology, 19, S14. https://doi.org/10.1016/s1090-3798(15)30044-1

Mishra, S. (2018). Juvenile Myasthenia Gravis: A Short Review. Progressing Aspects In Pediatrics And Neonatology, 2(1). https://doi.org/10.32474/papn.2018.02.000127