4.5 Article

Myasthenia Gravis and Lambert-Eaton Myasthenic Syndrome: New Developments in Diagnosis and Treatment

Journal

NEUROPSYCHIATRIC DISEASE AND TREATMENT
Volume 18, Issue -, Pages 3001-3022

Publisher

DOVE MEDICAL PRESS LTD
DOI: 10.2147/NDT.S296714

Keywords

Myasthenia Gravis; Lambert-Eaton Myasthenic Syndrome; disorders of neuromuscular transmission

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Myasthenia Gravis (MG) and Lambert-Eaton myasthenic syndrome (LEMS) are the most common disorders involving defective neuromuscular transmission in clinical practice. Patients often experience fatigable weakness and fluctuation in symptoms, regardless of whether the etiology is autoimmune, paraneoplastic, genetic, or toxic. Autoimmune MG is the most prevalent neuromuscular transmission disorder in adults, while LEMS is comparatively rare but requires familiarity with its clinical presentation, diagnosis, and management.
Myasthenia Gravis is, like it or not, the neurologist's disease! (Thomas Richards Johns II, MD Seminars in Neurology 1982). The most common disorders in clinical practice involving defective neuromuscular transmission are myasthenia gravis (MG) and Lambert-Eaton myasthenic syndrome (LEMS). The hallmark of weakness related to malfunction of the neuromuscular junction (NMJ) is variability in severity of symptoms from minute to minute and hour to hour. Fatigable weakness and fluctuation in symptoms are common in patients whether the etiology is autoimmune, paraneoplastic, genetic, or toxic. Autoimmune MG is the most common disorder of neuromuscular transmission affecting adults with an estimated prevalence of 1 in 10,000. While LEMS is comparatively rare, the unique clinical presentation, the association with cancer, and evolving treatment strategies require the neurologist to be familiar with its presentation, diagnosis, and management. In this paper we provide a summary of the meaningful recent clinical developments in the diagnosis and treatment of both MG and LEMS.

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