Pathophysiology of Childhood-Onset Myasthenia: Abnormalities of Neuromuscular Junction and Autoimmunity and Its Background

The pathophysiology of myasthenia gravis (MG) has been largely elucidated over the past half century, and treatment methods have advanced. However, the number of cases of childhood-onset MG is smaller than that of adult MG, and the treatment of childhood-onset MG has continued to be based on researc...

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Main Author: Masatoshi Hayashi
Format: Article
Language:English
Published: MDPI AG 2023-12-01
Series:Pathophysiology
Subjects:
Online Access:https://www.mdpi.com/1873-149X/30/4/43
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author Masatoshi Hayashi
author_facet Masatoshi Hayashi
author_sort Masatoshi Hayashi
collection DOAJ
description The pathophysiology of myasthenia gravis (MG) has been largely elucidated over the past half century, and treatment methods have advanced. However, the number of cases of childhood-onset MG is smaller than that of adult MG, and the treatment of childhood-onset MG has continued to be based on research in the adult field. Research on pathophysiology and treatment methods that account for the unique growth and development of children is now desired. According to an epidemiological survey conducted by the Ministry of Health, Labour and Welfare of Japan, the number of patients with MG by age of onset in Japan is high in early childhood. In recent years, MG has been reported from many countries around the world, but the pattern of the number of patients by age of onset differs between East Asia and Western Europe, confirming that the Japanese pattern is common in East Asia. Furthermore, there are racial differences in autoimmune MG and congenital myasthenic syndromes according to immunogenetic background, and their pathophysiology and relationships are gradually becoming clear. In addition, treatment options are also recognized in different regions of the world. In this review article, I will present recent findings focusing on the differences in pathophysiology.
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spelling doaj.art-a8a8093f2c3346bc93f48090b6d02ad82023-12-22T14:31:08ZengMDPI AGPathophysiology1873-149X2023-12-0130459961710.3390/pathophysiology30040043Pathophysiology of Childhood-Onset Myasthenia: Abnormalities of Neuromuscular Junction and Autoimmunity and Its BackgroundMasatoshi Hayashi0Department of Pediatrics, Uwajima City Hospital, Uwajima 798-8510, JapanThe pathophysiology of myasthenia gravis (MG) has been largely elucidated over the past half century, and treatment methods have advanced. However, the number of cases of childhood-onset MG is smaller than that of adult MG, and the treatment of childhood-onset MG has continued to be based on research in the adult field. Research on pathophysiology and treatment methods that account for the unique growth and development of children is now desired. According to an epidemiological survey conducted by the Ministry of Health, Labour and Welfare of Japan, the number of patients with MG by age of onset in Japan is high in early childhood. In recent years, MG has been reported from many countries around the world, but the pattern of the number of patients by age of onset differs between East Asia and Western Europe, confirming that the Japanese pattern is common in East Asia. Furthermore, there are racial differences in autoimmune MG and congenital myasthenic syndromes according to immunogenetic background, and their pathophysiology and relationships are gradually becoming clear. In addition, treatment options are also recognized in different regions of the world. In this review article, I will present recent findings focusing on the differences in pathophysiology.https://www.mdpi.com/1873-149X/30/4/43autoimmunitychildhood-onsetgenetic backgroundmyasthenia gravisneuromuscular junctionpathophysiology
spellingShingle Masatoshi Hayashi
Pathophysiology of Childhood-Onset Myasthenia: Abnormalities of Neuromuscular Junction and Autoimmunity and Its Background
Pathophysiology
autoimmunity
childhood-onset
genetic background
myasthenia gravis
neuromuscular junction
pathophysiology
title Pathophysiology of Childhood-Onset Myasthenia: Abnormalities of Neuromuscular Junction and Autoimmunity and Its Background
title_full Pathophysiology of Childhood-Onset Myasthenia: Abnormalities of Neuromuscular Junction and Autoimmunity and Its Background
title_fullStr Pathophysiology of Childhood-Onset Myasthenia: Abnormalities of Neuromuscular Junction and Autoimmunity and Its Background
title_full_unstemmed Pathophysiology of Childhood-Onset Myasthenia: Abnormalities of Neuromuscular Junction and Autoimmunity and Its Background
title_short Pathophysiology of Childhood-Onset Myasthenia: Abnormalities of Neuromuscular Junction and Autoimmunity and Its Background
title_sort pathophysiology of childhood onset myasthenia abnormalities of neuromuscular junction and autoimmunity and its background
topic autoimmunity
childhood-onset
genetic background
myasthenia gravis
neuromuscular junction
pathophysiology
url https://www.mdpi.com/1873-149X/30/4/43
work_keys_str_mv AT masatoshihayashi pathophysiologyofchildhoodonsetmyastheniaabnormalitiesofneuromuscularjunctionandautoimmunityanditsbackground