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

被引:8
|
作者
Pascuzzi, Robert [1 ]
Bodkin, Cynthia [1 ]
机构
[1] Indiana Univ Sch Med, Indiana Univ Hlth, Indianapolis, IN 46202 USA
关键词
Myasthenia Gravis; Lambert-Eaton Myasthenic Syndrome; disorders of neuromuscular transmission; DOUBLE-BLIND; MYCOPHENOLATE-MOFETIL; RANDOMIZED-TRIAL; PLASMA-EXCHANGE; NATURAL COURSE; THYMECTOMY; RITUXIMAB; 3,4-DIAMINOPYRIDINE; AUTOANTIBODIES; INSTRUMENT;
D O I
10.2147/NDT.S296714
中图分类号
R74 [神经病学与精神病学];
学科分类号
摘要
"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.
引用
收藏
页码:3001 / 3022
页数:22
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