Myasthenia gravis (MG) is an autoimmune disease characterized by dysfunction of the neuromuscular junction resulting in skeletal muscle weakness. It is equally prevalent in males and females, but debuts at a younger age in females and at an older age in males.
Ptosis, diplopia, facial bulbar weakness, and limb weakness are the most common symptoms. MG can be classified based on the presence of serum autoantibodies. Acetylcholine receptor (AChR) antibodies are found in 80%-85% of patients, muscle-specific kinase (MuSK) antibodies in 5%-8%, and <1% may have low-density lipoprotein receptor-related protein 4 (Lrp4) antibodies. Approximately 10% of patients are seronegative for antibodies binding the known disease-related antigens. In patients with AChR MG, 10%-20% have a thymoma, which is usually detected at the onset of the disease. Important differences between clinical presentation, treatment responsiveness, and disease mechanisms have been observed between these different serologic MG classes.
Besides the typical clinical features and serologic testing, the diagnosis can be established with additional tests, including repetitive nerve stimulation, single fiber EMG, and the ice pack test.
Treatment options for MG consist of symptomatic treatment (such as pyridostigmine), immunosuppressive treatment, or thymectomy. Despite the treatment with symptomatic drugs, steroid-sparing immunosuppressants, intravenous immunoglobulins, plasmapheresis, and thymectomy, a large proportion of patients remain chronically dependent on corticosteroids (CS). In the past decade, the number of treatment options for MG has considerably increased. Advances in the understanding of the pathophysiology have led to new treatment options targeting B or T cells, the complement cascade, the neonatal Fc receptor or cytokines. In the future, these new treatments are likely to reduce the chronic use of CS, diminish side effects, and decrease the number of patients with refractory disease.

Paraneoplastic Neurologic Disorders (Volume 200) (Handbook of Clinical Neurology, Volume 200)

Bruno Giometto; Sean J. Pittock

Academic Press, Incorporated

Morgan Kaufmann Publishers

Brooks/Cole

United States, United States of America

1, PT, 2024

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Paraneoplastic Neurological Disorders, Volume 200 covers new, recent descriptions of autoantibodies against neuronal cell surface antigens (only partly associated with paraneoplastic disorders) and the introduction of new immuno-oncological drugs that can contribute to diseases that are pathogenetically indistinguishable from paraneoplastic syndromes. Besides investigating the pathogenetic mechanisms of these disorders, this book also explores what the introduction of new drugs used in immune checkpoint inhibitor cancer therapy are teaching us in terms of immunopathogenesis. Following sections present individual syndromes (phenotypical clinical manifestations) of the CNS, PNS, NMJ, and muscle. Finally, the book investigates the role of antibody markers that differentiate by cytoplasmic, nuclear, and neuronal cell surface reactivities. Side effects of the new immuno-oncological drugs increasingly used in cancer therapy are also covered.