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Amyloidosis mimics achalasia's effect on lower esophageal sphincter

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Abstract

Esophageal involvement in systemic amyloidosis is common. Manometric studies have been few in number and have revealed a variety of findings compatible with deposition of amyloid in myopathic as well as neuropathic patterns. This report describes a patient with primary amyloid whose esophageal dysfunction was limited to the lower esophageal sphincter, with both a hypertensive sphincter and impaired relaxation after swallows. Pharmacologic testing confirmed a mild abnormality of the inhibitory pathway to the LES with an intact excitatory pathway and sphincter muscle. This case suggests that amyloidosis, like idiopathic achalasia and carcinoma of the lower esophagus, can produce dysphagia by selective impairment of the inhibitory neural pathway to the lower esophageal sphincter.

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Authors and Affiliations

  1. Division of Gastroenterology-Hepatology, Department of Medicine, State University of New York at Stony Brook, USA

    Jeffrey R. Lefkowitz MD,  Douglas L. Brand MD, Michael D. Schuffler MD & William R. Brugge MD

  2. Departments of Medicine, Pacific Medical Center and the University of Washington, Seattle, Washington

    Jeffrey R. Lefkowitz MD,  Douglas L. Brand MD, Michael D. Schuffler MD & William R. Brugge MD

Authors
  1. Jeffrey R. Lefkowitz MD
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  2. Douglas L. Brand MD
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  3. Michael D. Schuffler MD
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  4. William R. Brugge MD
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Lefkowitz, J.R., Brand, D.L., Schuffler, M.D. et al. Amyloidosis mimics achalasia's effect on lower esophageal sphincter. Digest Dis Sci 34, 630–635 (1989). https://doi.org/10.1007/BF01536343

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  • DOI: https://doi.org/10.1007/BF01536343

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