Myasthenia gravis in children; Issues and challenges

Abstract

Autoimmune myasthenia gravis (MG) occurs in children, as well as in adults. Childhood myasthenia gravis is characterized by its distinct clinical features. Epidemiological studies have shown the high frequency of onset before the age of 5 years in Japan. Similar results have been reported from China. Clinical types are divided into pure ocular, latent generalized and generalized. In general, childhood myasthenia gravis is milder, mainly with ocular symptoms. Anti-acetylcholine receptor antibody is often negative (seronegative). The responsiveness to treatment is generally good, and almost 90% of patients can achieve complete remission. Early diagnosis and adequate treatments are important.

Diagnostic dilemmas and challenges in the management of myasthenia in infants and toddlers: A case report

“…[10][11][12] AchR antibodies are often negative in young children with autoimmune myasthenia, which leads to difficulties in diagnosis. 1,10 The diagnosis is therefore either delayed or the patients are misdiagnosed. It is essential to distinguish between congenital and autoimmune myasthenia in young children due to the differing treatment options and prognosis.…”

“…Myasthenia is a neuromuscular junction disorder, characterized by muscle weakness and fatigability. 1 In children, three types of myasthenia have pathophysiological distinct mechanisms: transient neonatal myasthenia, congenital myasthenic syndrome (CMS), and juvenile myasthenia gravis (JMG). 2,3 CMS is a group of genetic disorders resulting from mutations in genes that code for proteins at the neuromuscular junction.…”

Approach to the unraveled questions over neuromuscular junction diseases

“…Finally, Nomura wrote a review on childhood MG. 5 Little is known about this area, because there are a small number of patients, and neurologists do not have many chances to examine them. However, it is noteworthy that, unlike in Caucasians, there is a certain proportion of childhood MG patients among Asian patients, especially with onset age between 0 and 5 years.…”