Pathology of the Gastrointestinal Tract

2017 Edition
| Editors: Fátima Carneiro, Paula Chaves, Arzu Ensari

Lewy Bodies, Achalasia

  • Manuela MafraEmail author
Reference work entry


Achalasia cardiae; Cardiospasm; Esophageal achalasia; Esophageal aperistalsis; Megaesophagus


Achalasia is an esophageal motility disorder involving the smooth muscle layer of the esophagus and the lower esophageal sphincter (LES).

Though the disease was first described more than 300 years ago (Willis 1674), its exact pathogenesis still remains poorly understood. Pathophysiologically, achalasia is caused by loss of inhibitory ganglion in the myenteric plexus of the esophagus. In the initial stage, degeneration of inhibitory nerves in the esophagus results in unopposed action of excitatory neurotransmitters, such as acetylcholine, resulting in high amplitude non-peristaltic contractions (vigorous achalasia). Progressive loss of cholinergic neurons over the time results in dilation and low-amplitude simultaneous contractions in the esophageal body (classical achalasia).

The most common form of achalasia is primary, which has no underlying cause. Since the initial...

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References and Further Reading

  1. Dichson, D., Fujishiro, H., DelleDonne, A., Menke, J., Ahmed, Z., Klos, K., Josephs, K., Frigerio, R., Burnett, M., Parisi, J., & Ahlskog, J. (2008). Evidence that incidental Lewy body disease is pre-symptomatic Parkinson’s disease. Acta Neuropathologica, 115, 437–444.CrossRefGoogle Scholar
  2. Ghosthal, U., Daschakraborty, S., & Singh, R. (2012). Pathogenesis of achalasia cardia. World Journal of Gastroenterology, 18(24), 3050–3057.CrossRefGoogle Scholar
  3. Giuli R, McCallum R. W., & Skinner, D. B. (1991). Primary motility disorders of the esophagus. Achalasia (hypomotility) is the best known entity.
  4. Marques, O., & Outeiro, T. F. (2012). Alpha-synuclein: From secretion to dysfunction and death. Cell Death and Disease, 3(7), e350.CrossRefGoogle Scholar
  5. Qualman, S. J., Haupt, H. M., Yang, P., & Hamilton, S. R. (1984). Esophageal Lewy bodies associated with ganglion cell loss in achalasia. Similarity to Parkinson’s disease. Gastroenterology, 87(4), 848–856.PubMedGoogle Scholar
  6. Wakabayashi, K., Tanji, K., Odagiri, S., Miki, Y., & Takahashi, H. (2013). The Lewy body in Parkinson’s disease and related neurodegenerative disorders. Molecular Neurobiology, 47(2), 495–508. (Epub ahead of print in 2012).CrossRefGoogle Scholar
  7. Wakabayashi, K., Mori, F., Tanji, K., Orimo, S., & Takahashi, H. (2010). Involvement of the peripheral nervous system in synucleinopathies, tauopathies and other neurodegenerative proteinopathies of the brain. Acta Neuropathologica, 120(1), 1–12.CrossRefGoogle Scholar
  8. Willis, T. (1674). Pharmaceutice Rationalis Sive Diatribe de Medicamentorum Operationibus in Human Corpore. London, England: Hagae Comitis.Google Scholar

Copyright information

© Springer International Publishing AG 2017

Authors and Affiliations

  1. 1.Centro Hospitalar Lisboa Central-H.S.JLisbonPortugal