title: Review of the Diagnostic Challenges of Lambert-Eaton Syndrome Revealed Through Three Case Reports.
abstract: 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.
authors.affiliation: 1Department of Clinical Neurophysiology,Hospital Universitario de la Ribera,Alzira,Valencia,Spain.
authors.forename: Miguel Ángel
authors.initials: MÁ
authors.lastname: Merino-Ramírez
authors.affiliation: 3Department of Medicine,Division of Neurology,Queen's University,Etherington Hall,Kingston,Ontario,Canada.
authors.forename: Charles F
authors.initials: CF
authors.lastname: Bolton
mesh_terms: D000200:Action Potentials / Q000502:physiology; D000328:Adult; D000368:Aged; D004558:Electric Stimulation; D004576:Electromyography; D019054:Evoked Potentials, Motor / Q000502:physiology; D005260:Female; D006801:Humans; D015624:Lambert-Eaton Myasthenic Syndrome / Q000175:diagnosis* / Q000503:physiopathology*; D008297:Male; D008875:Middle Aged; D018908:Muscle Weakness / Q000209:etiology; D009431:Neural Conduction / Q000502:physiology; D012120:Respiration Disorders / Q000175:diagnosis / Q000209:etiology
keywords: Autoimmune disease; Diaphragm; Heart rate variability; Lambert–Eaton syndrome; Myasthenia gravis