CC BY-NC-ND 4.0 · Neurology International Open 2018; 02(01): E6-E9
DOI: 10.1055/s-0043-120334
Review
Eigentümer und Copyright ©Georg Thieme Verlag KG 2018

Juvenile Myasthenia Gravis

Adela Della Marina
Myasthenie Zentrum für Kinder und Jugendliche, Abteilung für Neuropädiatrie, Kinderklinik I, Universitätsklinikum Duisburg- Essen
,
Ulrike Schara
Myasthenie Zentrum für Kinder und Jugendliche, Abteilung für Neuropädiatrie, Kinderklinik I, Universitätsklinikum Duisburg- Essen
› Author Affiliations
Further Information

Publication History

Publication Date:
08 January 2018 (online)

Abstract

Juvenile myasthenia gravis (JMG) is an autoimmune disorder of neuromuscular transmission caused by production of antibodies against the postsynaptic membrane of the neuromuscular junction. Clinical signs in young children and adolescents range from isolated ocular symptoms to general muscular weakness and respiratory insufficiency. Clinical presentation of JMG in young children and adolescents shows distinct features compared to adults. Young children may show generalized muscular weakness already during the first two years of life, and in this group specific antibodies can be only slightly increased. Because of existing therapeutic options, an early diagnosis is important. In case of negative specific antibodies and onset of the first symptoms during infancy or early childhood, the diagnosis of a congenital myasthenic syndrome (CMS) must be considered and is not always clear to differentiate. Clinical symptoms, diagnostic procedures and therapeutic strategies with consideration of specificities of this age group are discussed.