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* The exact pathogenesis of DES is not fully understood. However, current high-resolution manometric studies suggest impairment of inhibitory myenteric plexus neuron. These neurons use nitric oxide (NO) as neurotransmitter. Hence, these patients may also have dysregulation of endogenous NO synthesis or/and degradation<ref name="pmid4196712">{{cite journal| author=Orlando RC, Bozymski EM| title=Clinical and manometric effects of nitroglycerin in diffuse esophageal spasm. | journal=N Engl J Med | year= 1973 | volume= 289 | issue= 1 | pages= 23-5 | pmid=4196712 | doi=10.1056/NEJM197307052890106 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=4196712  }}</ref>. The final result is premature and rapidly propagated or simultaneous contraction of smooth muscles of distal esophagus.
* The exact pathogenesis of DES is not fully understood. However, current high-resolution manometric studies suggest impairment of inhibitory myenteric plexus neuron. These neurons use nitric oxide (NO) as neurotransmitter. Hence, these patients may also have dysregulation of endogenous NO synthesis or/and degradation<ref name="pmid4196712">{{cite journal| author=Orlando RC, Bozymski EM| title=Clinical and manometric effects of nitroglycerin in diffuse esophageal spasm. | journal=N Engl J Med | year= 1973 | volume= 289 | issue= 1 | pages= 23-5 | pmid=4196712 | doi=10.1056/NEJM197307052890106 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=4196712  }}</ref>. The final result is premature and rapidly propagated or simultaneous contraction of smooth muscles of distal esophagus.
References<references />
References<references />
== Natural History, Complications, and Prognosis ==
=== Natural History ===
* The symptoms of (disease name) usually develop in the first/ second/ third decade of life, and start with symptoms such as ___.
* The symptoms of (disease name) typically develop ___ years after exposure to ___.
* If left untreated, [#]% of patients with [disease name] may progress to develop [manifestation 1], [manifestation 2], and [manifestation 3].
=== Complications ===
* Common complications of [disease name] include:
** [Complication 1]
** [Complication 2]
** [Complication 3]
=== Prognosis ===
* Prognosis is generally excellent/good/poor, and the 1/5/10-year mortality/survival rate of patients with [disease name] is approximately [#]%.
* Depending on the extent of the [tumor/disease progression/etc.] at the time of diagnosis, the prognosis may vary. However, the prognosis is generally regarded as poor/good/excellent.
* The presence of [characteristic of disease] is associated with a particularly [good/poor] prognosis among patients with [disease/malignancy].
* [Subtype of disease/malignancy] is associated with the most favorable prognosis.
* The prognosis varies with the [characteristic] of tumor; [subtype of disease/malignancy] have the most favorable prognosis.
== References ==

Revision as of 17:11, 29 October 2017


Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Madhu Sigdel

Historical Perspective

Discovery

  • DES was first described by Osgood, in 1889 in 6 patients who presented with chest pain and dysphagia.
  • Creamer (1954) made the first manometric descriptions of DES.

Classification of DES

  • There is no established system for the classification of DES although it is categorized as one of the major disorders of peristalsis according to The Chicago Classification v.3.0.

Risk Factors

  • Common risk factors in the development of Diffuse Esophageal Spasm include Age (60-80 years), presence of GERD, Hypertension, anxiety or depression, and drinks (eg. red wine, very hot or cold liquid or fluid).

Pathophysiology

Pathogenesis

  • The exact pathogenesis of DES is not fully understood. However, current high-resolution manometric studies suggest impairment of inhibitory myenteric plexus neuron. These neurons use nitric oxide (NO) as neurotransmitter. Hence, these patients may also have dysregulation of endogenous NO synthesis or/and degradation[1]. The final result is premature and rapidly propagated or simultaneous contraction of smooth muscles of distal esophagus.

References

  1. Orlando RC, Bozymski EM (1973). "Clinical and manometric effects of nitroglycerin in diffuse esophageal spasm". N Engl J Med. 289 (1): 23–5. doi:10.1056/NEJM197307052890106. PMID 4196712.

Natural History, Complications, and Prognosis

Natural History

  • The symptoms of (disease name) usually develop in the first/ second/ third decade of life, and start with symptoms such as ___.
  • The symptoms of (disease name) typically develop ___ years after exposure to ___.
  • If left untreated, [#]% of patients with [disease name] may progress to develop [manifestation 1], [manifestation 2], and [manifestation 3].

Complications

  • Common complications of [disease name] include:
    • [Complication 1]
    • [Complication 2]
    • [Complication 3]

Prognosis

  • Prognosis is generally excellent/good/poor, and the 1/5/10-year mortality/survival rate of patients with [disease name] is approximately [#]%.
  • Depending on the extent of the [tumor/disease progression/etc.] at the time of diagnosis, the prognosis may vary. However, the prognosis is generally regarded as poor/good/excellent.
  • The presence of [characteristic of disease] is associated with a particularly [good/poor] prognosis among patients with [disease/malignancy].
  • [Subtype of disease/malignancy] is associated with the most favorable prognosis.
  • The prognosis varies with the [characteristic] of tumor; [subtype of disease/malignancy] have the most favorable prognosis.

References