RESUMEN
Myasthenia gravis (MG) is an autoimmune disease that affects the postsynaptic membrane at the neuromuscular junction. In MG, antibodies bind to acetylcholine receptors inducing muscle weakness. The weakness typically increases with exercise and repetitive muscle use. Improvement of muscular weakness after rest and/or administration of anticholinesterase drugs (edrophonium) are characteristic of MG. We report a patient with unexplained dysphagia, dysphonia, and dysarthria, whose diagnosis was suggested by high-resolution esophageal motility and edrophonium infusion. We highlight the importance of dysphagia as presenting or dominant symptom in MG and review the esophageal motility findings in this rare, but treatable disorder.
RESUMEN
Diffuse esophageal spasm (DES) is a motility disorder of undetermined etiology, typically presenting with chest pain, dysphagia or both. The aim of this paper is to provide a critical review of the prevalence, pathogenesis, diagnosis and therapy of DES. Data from referral centers indicates that this is a rare disorder with a prevalence of 4-7%. The diagnosis is based on the combination of typical symptoms, radiological findings and manometry (simultaneous contractions (SC) in the distal esophagus in > or = 20% of wet swallows mixed with normal peristalsis). The pathogenesis remains poorly understood. Recent evidence suggests that nitric oxide deficiency may explain the SC that characterizes this condition at manometry. Gastroesophageal reflux (GER) can coexist in DES and GER has also been implied in the pathogenesis of DES. Whether patients with DES and GER represent a subtype of DES with a different prognosis or outcome is unknown. We present a critical appraisal regarding different therapeutic approaches available for DES and conclude suggesting a management algorithm based on current available literature.