Review of the Diagnostic Challenges of Lambert-Eaton Syndrome Revealed Through Three Case Reports

被引:2
作者
Angel Merino-Ramirez, Miguel [1 ,2 ]
Bolton, Charles F. [3 ]
机构
[1] Hosp Univ Ribera, Dept Clin Neurophysiol, Carretera Corbera Km 1, Valencia 46600, Spain
[2] CEU Cardenal Herrera Univ, Dept Biomed Sci, Valencia, Spain
[3] Queens Univ, Dept Med, Div Neurol, Etherington Hall, Kingston, ON, Canada
关键词
Lambert-Eaton syndrome; Myasthenia gravis; Diaphragm; Heart rate variability; Autoimmune disease; REPETITIVE NERVE-STIMULATION; CALCIUM-CHANNEL AUTOANTIBODIES; SYMPATHETIC SKIN-RESPONSE; SINGLE-FIBER EMG; MYASTHENIC-SYNDROME; AUTONOMIC DYSFUNCTION; CLINICAL CORRELATION; AAEM MINIMONOGRAPH; GRAVIS; PATIENT;
D O I
10.1017/cjn.2016.268
中图分类号
R74 [神经病学与精神病学];
学科分类号
摘要
Lambert-Eaton syndrome (LES) is a rare immune-mediated disorder characterized by proximal leg weakness, autonomic symptoms and hypoactive tendon reflexes. The paraneoplastic form is associated with small-cell lung cancer in 50-60% of cases, whereas the remaining cases are found in younger adults with a higher likelihood of coexisting autoimmune disease. The early recognition of LES is crucial for improving clinical outcomes but remains a major challenge. In this review, we analyze the clinical characteristics and diagnostic considerations in treating LES through a series of three case studies, one of which showed definitive response to pyridostigmine and corticosteroid combination therapy, followed by spontaneous remission. Patients were assessed by image-based screening, serological testing and electrophysiological evaluations, which included respiratory and autonomic testing. A better understanding of the common pitfalls in the clinical, serological and neurophysiologic diagnosis of LES through assessment of typical LES dysfunction throughout the nervous system should enable improved recognition and treatment of this syndrome.
引用
收藏
页码:635 / 647
页数:13
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