Primary Motility  Disorders of the  Esophagus
 The Esophageal
 Esophagogastric  Junction

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Volume: Primary Motility Disorders of the Esophagus
Chapter: Transitional forms

Those between corkscrew esophagus and achalasia have long been known

J. Janssens, G. Vantrappen (Leuven)

Primary esophageal motility disorders constitute a spectrum of conditions composed of achalasia, diffuse esophageal spasm and intermediate types. This is particularly true in patients with severe functional dysphagia: in a series of 156 consecutive patients with a severe degree of dysphagia that required treatment by pneumatic dilatations, the nature of the underlying motility disorders was analysed on the basis of simple manometric criteria, i.e. presence or absence of peristaltic contractions and presence or absence of normal sphincteric relaxations [1]. Theoretically, 4 different combinations are possible in these patients: absence of peristalsis and absence of normal sphincter relaxations (achalasia), severe esophageal motor disorders with preservation of some peristaltic contractions and some normal sphincter relaxations (diffuse esophageal spasm), and two intermediate types in which either some peristalsis or some sphincter relaxation is preserved. Of the 156 patients, 70 p. cent had typical achalasia, 11 p. cent had typical diffuse esophageal spasm and 19 p. cent had intermediate types.

The existence of such a spectrum of primary esophageal motility disorders already suggests the possibility of a transition from one entity into the other.

The transition from symptomatic diffuse esophageal spasm to achalasia was first described by Kramer et al. [2], and similar case reports have been published since. However, it is generally believed to be a rather exceptional evolution. In only 6 of the 156 patients described above, we have observed a transition from a condition that may be characterized as « severe esophageal motility disturbances with peristalsis and sphincter relaxations after some swallows» to a condition « without peristalsis and without relaxations ». Two patients initially presented with a stage which should be called (vigorous) diffuse spasm; and 4 patients initially had non vigorous diffuse esophageal contractions (vigorous being defined as by the presence of triple or more peaked waves and/or giant contractions). The final esophageal motility disorders corresponded to vigorous achalasia in 4, and classical achalasia in 2 patients.

These 6 cases may therefore be considered as transition from (non-vigorous or vigorous) diffuse esophageal spasm to (vigorous or non-vigorous) achalasia.


1. Vantrappen G, Janssens J, Hellemans J, Coremans G (1979) Achalasia, diffuse esophageal spasm, and related motility disorders. Gastroenterology ; 76 : 450-458.

2. Kramer P, Harris LD, Donaldson RM (1967) Transition from symptomatic diffuse spasm to cardiospasm. Gut 8 : 115-119.

Publication date: May 1991 OESO©2015