J Korean Child Neurol Soc.  2017 Mar;25(1):1-8. 10.0000/jkcns.2017.25.1.1.

Subgroup Classification and Therapeutic Guidance for Myasthenia Gravis

• Lee YJ

• KMID: 2385206
• DOI: http://doi.org/10.0000/jkcns.2017.25.1.1

Abstract

Myasthenia gravis (MG) is a chronic autoimmune disease of neuromuscular blockade, characterized by muscle weakness and fatigue, and is associated with the production of autoantibodies against the skeletal muscle acetylcholine receptor, muscle-specific kinase (MuSK), low-density lipoprotein receptor-related protein 4 (LRP4), and other muscle endplate proteins. In addition, MG may be classified according the location of the affected muscles (ocular vs. generalized), patient age at symptom onset, and thymic pathology. Subgroup classification based on serum antibodies and clinical features include early-onset, late-onset, thymoma-associated, MuSK and LRP4 antibody-negative, and ocular forms of MG, and can help with therapeutic decisions and prognosis. Pyridostigmine is the chosen symptomatic treatment. For patients who do not adequately respond to symptomatic therapy, corticosteroids, other immunomodulating agents, and thymectomy are the first-line immunosuppressive treatments. The treatment of MG is highly individualized and depends on the age of the patient, the type and severity of the disease, and the pace of progression.

Keyword

Myasthenia gravis; Child; Adult; Therapy; Thymectomy

MeSH Terms

Acetylcholine
Adrenal Cortex Hormones
Adult
Antibodies
Autoantibodies
Autoimmune Diseases
Child
Classification*
Fatigue
Humans
Lipoproteins
Muscle Weakness
Muscle, Skeletal
Muscles
Myasthenia Gravis*
Neuromuscular Blockade
Pathology
Phosphotransferases
Prognosis
Pyridostigmine Bromide
Thymectomy
Acetylcholine
Adrenal Cortex Hormones
Antibodies
Autoantibodies
Lipoproteins
Phosphotransferases
Pyridostigmine Bromide