Distal esophageal spasm is a rare motility disorder presenting principally with nonobstructive dysphagia and noncardiac chest pain. In symptomatic patients, the manometric diagnosis is made when >10% of the wet swallows have simultaneous and/or premature contractions intermixed with normal peristalsis. We characterize manometry and barium as complementary diagnostic approaches, and given the intermittent nature of the disorder, one should be always aware that it is almost impossible to rule out spasm. Treatment is difficult; we propose an approach beginning with the least invasive intervention.

Previous studies have not been able to correlate manometry findings with bolus perception. The aim of this study was to evaluate correlation of different variables, including traditional manometric variables (at diagnostic and extreme thresholds), esophageal shortening, bolus transit, automated impedance manometry (AIM) metrics and mood with bolus passage perception in a large cohort of asymptomatic individuals.

High resolution manometry (HRM) was performed in healthy individuals from nine centers. Perception was evaluated using a 5-point Likert scale. Anxiety was evaluated using Hospitalized Anxiety and Depression scale (HAD). Subgroup analysis was also performed classifying studies into normal, hypotensive, vigorous, and obstructive patterns.

One hundred fifteen studies were analyzed (69 using HRM and 46 using high resolution impedance manometry (HRIM); 3.5% swallows in 9.6% of volunteers were perceived. There was no correlation of any of the traditional HRM variables, esophageal shortening, AIM metrics nor bolus transit with perception scores. There was no HRM variable showing difference in perception when comparing normal vs extreme values (percentile 1 or 99). Anxiety but not depression was correlated with perception. Among hypotensive pattern, anxiety was a strong predictor of variance in perception (R² up to .70).

Bolus perception is less common than abnormal motility among healthy individuals. Neither esophageal motor function nor bolus dynamics evaluated with several techniques seems to explain differences in bolus perception. Different mechanisms seem to be relevant in different manometric patterns. Anxiety is a significant predictor of bolus perception in the context of hypotensive motility.

To cover the diagnosis and management of diffuse esophageal spasm, nutcracker esophagus/spastic nutcracker and hypertensive lower esophageal sphincter. An outline of the presentation and manometric features of these conditions will precede a discussion of therapies. All of these diagnoses are made manometrically, even though they may be suspected by presentation and by findings at video fluoroscopic swallow or gastroscopy testing.

The advent of high-resolution manometry testing has allowed a better understanding of these motility disorders, and the ability to standardize the diagnoses by the use of the Chicago Classification is a major step forward. Recent developments show that botulinum toxin and perioral myotomy can be an effective treatment for some patients. This should bring more therapies to the fore in the future, but at present there is still the need for more prospective study of best therapies.

The important point to remember for all of these conditions is that unlike achalasia, there is no definite pathological correlation to the manometrically observed abnormalities. This therefore makes the management challenging and means that treatment pathways are not as well set out as for some other upper gastrointestinal motility problems.

The concept of esophageal spastic disorders encompasses spastic achalasia, distal esophageal spasm, and jackhammer esophagus. These are conceptually distinct in that spastic achalasia and distal esophageal spasm are characterized by a loss of neural inhibition, whereas jackhammer esophagus is associated with hypercontractility. Hypercontractility may also occur as a result of esophagogastric junction outflow obstruction or inflammation. The diagnosis of jackhammer esophagus as a primary motility disorder is based on the characteristic manometric findings after ruling out mechanical obstruction and eosinophilic esophagitis. Despite the differences in pathophysiology among the esophageal spastic disorders, their management is similar.

Distal oesophageal spasm is a rare and under-investigated motility abnormality. Recent studies indicate effective bolus transit in varying percentages of distal oesophageal spasm patients.

Explore functional aspects including contraction onset velocity and contraction amplitude cut-off values for simultaneous contractions to predict complete bolus transit.

We re-examined data from 107 impedance-manometry recordings with a diagnosis of distal oesophageal spasm. Receiver operating characteristic analysis was conducted, regarding effects of onset velocity on bolus transit taking into account distal oesophageal amplitude and correcting for intra-individual repeated measures.

Mean area under the receiver operating characteristic curve for saline and viscous swallows were 0.84±0.05 and 0.84±0.04, respectively. Velocity criteria of >30 cm/s when distal oesophageal amplitude>100 mmHg and 8 cm/s when distal oesophageal amplitude<100 mmHg for saline and 32cm/s when distal oesophageal amplitude>100 mmHg and >7 cm/s when distal oesophageal amplitude<100 mmHg for viscous had a sensitivity of 75% and specificity of 80% to identify complete bolus transit. Using these criteria, final diagnosis changed in 44.9% of patients. Abnormal bolus transit was observed in 50.9% of newly diagnosed distal oesophageal spasm patients versus 7.5% of patients classified as normal. Distal oesophageal spasm patients with distal oesophageal amplitude>100 mmHg suffered twice as often from chest pain than those with distal oesophageal amplitude<100 mmHg.

The proposed velocity cut-offs for diagnosing distal oesophageal spasm improve the ability to identify patients with spasm and abnormal bolus transit.

Diffuse esophageal spasm (DES) is a rare primary motility disorder of unknown cause, that can be found in patients complaining of chest pain and dysphagia and in whom ischemic heart disease and GERD have been excluded. The manometric hallmark of DES is the presence of simultaneous contractions in the distal esophagus alternating with a normal peristalsis. Even at specialized esophageal motility laboratories, DES is considered an uncommon diagnosis. In this review, the authors discuss the clinical and diagnostic aspects of this disease, as well as the possible therapeutic options (medical, endoscopic or surgical therapy). Surgery (esophageal myotomy performed through a thoracotomy or with a thoracoscopic access) seems to have a better outcome than medical or endoscopic treatment, and it is considered "the last resource" in these patients. However, satisfactory results are reported, from highly skilled centers, in only about 70% of treated cases, certainly inferior to those achieved in other esophageal disorders. The role of surgery in this disease requires therefore further study, even if controlled trials are probably difficult to perform, due to the rarity of the disease.

Diffuse esophageal spasm (DES) remains insufficiently understood. Here we aimed to summarize the demographic, clinical, radiographic, and manometric features in a large cohort of patients with DES. We identified all consecutive patients diagnosed with DES from 2000 to 2006 at Mayo Clinic Florida. The computerized records of these patients were reviewed to extract relevant information. We performed 2654 esophageal motilities during that period. There were 108 patients with esophageal spasm, and 55% were female. Median age was 71 years. The most common leading symptom was dysphagia in 55, followed by chest pain in 31. Weight loss occurred in 28 patients. The median of time from onset of symptoms to diagnosis was 48 months (range 0-480), with a median of time from the first medical consultation to diagnosis of 8 months (range 0-300). The most frequent comorbidities were hypertension and psychiatric problems. At presentation, 81 patients were taking acid-reducing medications, and 49 patients were taking psychotropic drugs. An abnormal esophagogram was noted in 46 of 76 patients with this test available, but most radiographic findings were nonspecific with the typical 'corkscrew' appearance seen in only three patients. Gastroesophageal reflux disease (GERD) was diagnosed by pH testing or endoscopy in 41 patients. We did not find any difference between the rate of simultaneous contractions or esophageal amplitude between patients with a leading symptom of dysphagia and those with chest pain. DES is an uncommon motility disorder that often goes unrecognized for years. Physicians should be aware of the clinical heterogeneity of DES and consider motility testing early in the course of unexplained esophageal symptoms. Given the high prevalence of GERD in DES, the role of GERD and the impact of acid-reducing therapy in DES deserve further study.

Esophageal high-resolution manometry (HRM) improves the management of patients with nonobstructive dysphagia. It has increased the diagnostic yield for detecting achalasia and defined three clinically relevant achalasia subtypes. Esophagogastric junction (EGJ) outflow obstruction, defined as an impaired EGJ relaxation in association with some preserved peristalsis, might also represent an achalasia variant in some cases. Using the concept of distal latency, the criteria for defining distal esophageal spasm, have been revised as the occurrence of premature distal contractions. Finally, the combination of HRM and impedance monitoring allows for a functional definition of weak peristalsis associated with incomplete bolus transit.

The manometric diagnosis of distal esophageal spasm (DES) uses "simultaneous contractions" as a defining criterion, ignoring the concept of short latency distal contractions as an important feature. Our aim was to apply standardized metrics of contraction velocity and latency to high-resolution esophageal pressure topography (EPT) studies to refine the diagnosis of DES.

Two thousand consecutive EPT studies were analyzed for contractile front velocity (CFV) and distal latency to identify patients potentially having DES. Normal limits for CFV and distal latency were established from 75 control subjects. Clinical data of patients with reduced distal latency and/or rapid CFV were reviewed.

Of 1070 evaluable patients, 91 (8.5%) had a high CFV and/or low distal latency. Patients with only rapid contractions (n = 186 [17.4%] using conventional manometry criteria; n = 85 [7.9%] using EPT criteria) were heterogeneous in diagnosis and symptoms, with the majority ultimately categorized as weak peristalsis or normal. In contrast, 96% of patients with premature contraction had dysphagia, and all (n = 24; 2.2% overall) were ultimately managed as spastic achalasia or DES.

The current DES diagnostic paradigm focused on "simultaneous contractions" identifies a large heterogeneous set of patients, most of whom do not have a clinical syndrome suggestive of esophageal spasm. Incorporating distal latency into the diagnostic algorithm of EPT studies improves upon this by isolating disorders of homogeneous pathophysiology: DES with short latency and spastic achalasia. We hypothesize that prioritizing measurement of distal latency will refine the management of these disorders, recognizing that outcomes trials are necessary.

Diffuse esophageal spasm (DES) has been reported as a potential cause of dysphagia or chest pain; however, the patho-physiology of DES is unclear. The aim of this study was to examine the manometric correlates of dysphagia and chest pain in this patient population. All patients undergoing manometry at our institution are entered into a prospectively maintained database. After institutional review board approval, the database was queried to identify patients meeting criteria for DES (≥20% simultaneous waves with greater than 30 mm Hg pressure in the distal esophagus). The patient-reported symptoms and manometric data, along with the results of a 24-hour pH study (if done), were extracted for further analysis. Out of 4923 patients, 240 (4.9%) met the manometric criteria for DES. Of these, 217 patients had complete manometry data along with at least one reported symptom. Of the patients with DES, 159 (73.3%) had dysphagia or chest pain as a reported symptom. Patients reporting either dysphagia or chest pain had significantly higher lower esophageal sphincter (LES) pressure than patients without these symptoms (P= 0.007). Significant association was noted between reported dysphagia and percentage of simultaneous waves. Chest pain did not correlate with percent of simultaneous waves, mean amplitude of peristalsis, or 24-hour pH score. The origin of reported chest pain in patients with DES is not clear but may be related to higher LES pressure. Simultaneous waves were associated with reported dysphagia. Using current diagnostic criteria, the term DES has no clinical relevance.

 Diffuse esophageal spasm (DES) is characterized on manometry by a combination of simultaneous contractions and normal swallows. The aim of this study was to examine the manometric characteristics of simultaneous and 'normal' swallows in patients with DES patients compared with normal controls.

Manometric studies from 69 patients with DES and 20 controls were analysed to determine the proportion of normal, hypertensive, ineffective and simultaneous contractions, and the velocity of propagation along the esophagus, the duration and amplitude of contraction and the relaxation characteristics (nadir and duration) of the lower esophageal sphincter.

 The propagation velocity was the only significant difference between normal swallows and simultaneous contractions in DES patients (middle third: 49.2 VS 101.2 mm s(-1), P ≤ 0.001 lower third: 44.1 VS 88.7 mm s(-1), P ≤ 0.001). 'Normal' swallows in patients with DES had a greater velocity of propagation than those in age-matched control subjects (middle third: 49.2 VS 37.0 mm s(-1), P = 0.02, lower third: 44.1 VS 23.3 mm s(-1), P ≤ 0.001).

 As expected, simultaneous contractions of DES patients differ from 'normal' swallows in DES patients mainly regarding the velocity of propagation of contraction but are similar in amplitude, however 'normal' swallows of DES patients are also more rapidly propagated along the esophagus than normal swallows of a control group suggesting that all swallows in DES are affected to some degree by the same process.

We studied the spectrum of motor dysphagia in a northern Indian tertiary referral center.

In this retrospective study, consecutive patients with motor dysphagia referred to the Gastrointestinal Pathophysiology and Motility Laboratory from 2002 to 2007 were evaluated clinically and with eight-channel water-perfusion manometry. Causes of dysphagia were diagnosed using standard criteria.

Of 250 patients (age 41.3 [15.0] years, 146 men), 193 (77%) had achalasia cardia (AC) and 57 (23%) had other causes (11, 4.4%: diffuse esophageal spasm [DES]; 9, 3.6%: hypertensive lower esophageal sphincter [Hy LES]); manometry was normal in 37 patients. Twenty-seven patients (14%) had vigorous AC. Duration of dysphagia at presentation was longer in those with AC and Hy LES than in normal manometry (NM) (21 months [1-180] vs. 6 [1-360], p = 0.000; 24 months [7-48] vs. 6 [1-360], p = 0.015). Regurgitation and bolus obstruction were more frequent in those with AC than in NM (89/154, 57.79% vs. 3/27, 11.11%, p = 0.000001). Heartburn was less frequent in patients with AC than in others (AC: 4/146, 2.73% vs. normal: 4/27, 14.8% [p = 0.02] and others: 3/15, 20% [p = 0.018]). Chest pain was reported by 74/135 (54.8%) classic and 12/19 (63.2%) vigorous AC (p = NS). Patients with NM had lower LES pressure than those with classic AC, Hy LES and vigorous AC (p < 0.0001 in each case). Patients with DES had lower LES pressure than in classic AC, Hy LES and vigorous AC (p = 0.043, p < 0.0001, and p = 0.002, respectively). Patients with classic AC had lower LES pressure than in Hy LES and vigorous AC (p = 0.024, p = 0.001, respectively).

Classic AC was the commonest cause of motor dysphagia in our center. AC was associated with higher LES pressure, longer duration of dysphagia, frequent regurgitation and bolus obstruction.

To evaluate the utility of selective esophageal manometry in clinical practice.

Retrospective data from 200 subjects was reviewed. Manometry was considered to be (1) high clinical utility when specific abnormality was identified resulting in therapeutic intervention, (2) low clinical utility when specific abnormality was identified without alteration of therapy, (3) limited clinical utility when manometry was normal. High, low, and limited clinical utility was noted in 47, 40, and 13% of instances. Manometry was of high utility in patients with dysphagia or non-cardiac chest pain (P < 0.05), and low utility in gastroesophageal reflux (P < 0.05).

(1) Esophageal manometry has high clinical utility in dysphagia after exclusion of structural disorders; and non-cardiac chest pain after careful exclusion of reflux. (2) Ineffective motility disorder has high association with gastroesophageal reflux disease but low clinical utility except in preoperative assessment for fundoplication. (3) Esophageal manometry is of high utility in clinical practice when used in conjunction with other diagnostic exclusions.

The current available methods for diagnosis of GORD are symptom questionnaires, catheter and wireless pH-metry, impedance-pH monitoring and Bilitec(@). Osophageal pH monitoring allows both quantitative analysis of acid reflux and assessment of reflux-symptom association. Impedance-pH monitoring detects all types of reflux (acid and non-acid) and allows assessment of proximal extent of reflux, a relevant parameter for understanding symptoms perception and extraoesophageal symptoms. Bilitec provides a quantitative assessment of duodeno-gastro-oesophageal reflux. Oesophageal motor abnormalities have been associated with GORD symptoms as well as chest pain and dysphagia. High-resolution manometry contributed to re-classify oesphageal motor disorders. However, barium swallows are still essential for evaluation of oesophageal anatomy and combined oesophageal manometry-impedance can assess oesophageal motility and bolus transit simultaneously in a non-radiological way. Still in experimental phase, high-frequency ultrasound allows monitoring of the oesophageal wall thickness and exaggerated longitudinal muscle contraction that might be associated to chest pain and dysphagia. This chapter provides a critical evaluation of the clinical application of these techniques.

Esophageal manometry is a specialized procedure used to evaluate lower and upper esophageal sphincter pressure, esophageal body contraction amplitude, and peristaltic sequence. The procedure is clinically useful in evaluation of a patient with nonstructural dysphagia, unexplained or noncardiac chest pain, a compendium of symptoms suggested because of gastroesophageal reflux disease, and in the preoperative evaluation for antireflux surgery. Manometric findings in 95 normal subjects evenly distributed across age groups were reported in 1987, and are the values still used in our and most laboratories today. The subsequent review will offer our "view" on the clinical utility of esophageal manometry, on the basis of years of experience and performance techniques that have remained constant over decades.

In 1987, Richter and colleagues published the results of an esophageal motility study conducted on 95 normal healthy volunteers between the ages of 22 and 79. In accordance with these results, abnormal esophageal motility was defined on the basis of the percentage of manometric normal, ineffective, and simultaneous swallows and on lower esophageal sphincter dynamics during liquid swallows. For example, Richter and colleagues found that the mean amplitude of contraction in the distal body of the esophagus >180 mm Hg in association with wet swallows was above the 95% confidence interval of normal. Richter's study also showed a wide variation among individuals and that the mean distal esophageal contractile amplitude increased with age without sex predominance. Likewise, as no subjects had >20% simultaneous contractions (though a considerable number, 4%, had 10% simultaneous contractions), esophageal spasm was defined at the >20% mark.

The purpose of this article is to review the clinical features, pathophysiology, diagnosis, and management of patients with diffuse esophageal spasm (DES). The PubMed database was searched with a focus on recent publications, using keywords "DES," plus "epidemiology," "prevalence," "diagnosis," "pathogenesis," "calcium channel blocker," "nitrates," "botulinum toxin," "antidepressants," "dilation," and "myotomy." The reference lists of papers identified in the initial PubMed search were reviewed for further relevant publications.

Motor abnormalities of the oesophagus are characterised by a chronic impairment of the neuromuscular structures that co-ordinate oesophageal function. The best-defined entity is achalasia, which is discussed in a separate chapter. Other motor disorders with clinical relevance include diffuse oesophageal spasm, oesophageal dysmotility associated with scleroderma, and ineffective oesophageal motility. These non-achalasic motor disorders have variable prevalence but they could be associated with invalidating symptoms such as dysphagia, chest pain and gastro-oesophageal reflux disease. New oesophageal diagnostic techniques, including high-resolution manometry, high-frequency intraluminal ultrasound and intraluminal impedance, allow (1) better definition of peristalsis and sphincter function, (2) assessment of changes in oesophageal wall thickness, and (3) evaluation of pressure gradients within the oesophagus and across the sphincters that can produce normal or abnormal patterns of bolus transport. This chapter discusses recent advances in physiology, pathophysiology, diagnosis and treatment of non-achalasic oesophageal motor disorders.

Every year more insight into the pathogenesis and treatment of esophageal motor disorders is obtained. This review highlights some interesting literature published in this area during the last year.

Longitudinal and circular muscle contractions act in a well coordinated fashion to allow normal peristalsis. Techniques such as intraluminal impedance, high-resolution manometry and intraluminal ultrasound provide useful additional information on esophageal function both in the normal and abnormal situation. The dynamics of the gastroesophageal junction can be studied with a newly developed probe, and the mechanism behind transient lower esophageal sphincter relaxations is still being unravelled. New manometric criteria for nutcracker esophagus have been proposed, whereas further evidence is reported supporting an association between diabetes mellitus and cardiovascular disease and esophageal dysmotility and spasm, respectively. Finally, several long-term follow-up results of surgical myotomy and pneumodilatation have been reported.

Due to the perfection of esophageal measuring techniques, our knowledge of esophageal function continues to increase. The studies reviewed here provide interesting information on the pathogenesis and treatment of several esophageal motor disorders.

Oesophageal spasm is a common empiric diagnosis clinically applied to patients with unexplained chest pain. In contrast it is an uncommon manometric abnormality found in patients presenting with chest pain and/or dysphagia and diagnosed by >or=20% simultaneous oesophageal contractions during standardized motility testing. Using Medline we searched for diagnostic criteria and treatment options for oesophageal spasm. While the aetiology of this condition is unclear, studies suggest the culprit being a defect in the nitric oxide pathway. Well-known radiographic patterns have low sensitivities and specificities to identify intermittent simultaneous contractions. Recognizing that simultaneous contractions may result from gastro-oesophageal reflux this diagnosis should be investigated or treated first. Studies have documented improvements with proton-pump inhibitors, nitrates, calcium-channel blockers and tricyclic antidepressants or serotonin reuptake inhibitors. Small case series reported benefits after botulinium toxin injections, dilatations and myotomies. Uncertainties persist regarding the optimal management of oesophageal spasm and recommendations are based on controlled studies with small numbers of patients or on case series. Acid suppression, muscle relaxants and visceral analgetics should be tried first. Botulinium toxin injections should be reserved for patients who do not respond. Pneumatic dilatations or myotomies represent rather heroic approaches for non-responding patients.

Diffuse esophageal spasm (DES) and achalasia share both clinical and manometric characteristics. Some reports support the notion of progression of DES to achalasia. However, there are currently no prospective data in support of this theory. To assess prospectively the rate of manometric progression of DES to achalasia. Manometry tracings of DES patients diagnosed between 1992 and 2003 were independently reviewed blindly and agreed on by two esophageal experts. Patients with DES who agreed to undergo repeat esophageal manometry constituted the study cohort. Follow-up manometry tracings were evaluated blindly and independently by the same two interpreters to determine the rate of manometric progression to achalasia. Predictors of manometric progression were assessed. A total of 32 patients were diagnosed with DES between 1992-2003. Twelve patients (9M/3F; median age 62 years) agreed to participate and underwent second manometry (mean +/- SD follow-up of 4.8 +/- 3.4 years). Achalasia was diagnosed on follow-up manometry in one patient (8%), seven (58%) patients continued to have DES, three (25%) had normal motility, and one (8%) had nutcracker esophagus. There were no predictors of progression to achalasia based on the initial manometry parameters. A subgroup of DES patients with initial low esophageal body amplitude developed increase in esophageal simultaneous contractions on follow-up similar to the patient who evolved to achalasia. Following were the results. 1) Progression from DES to achalasia is uncommon. 2) DES patients with low esophageal body amplitude may develop increased simultaneous contractions over time. 3) DES remains an elusive diagnosis clinically and manometrically.

Diffuse esophageal spasm (DES) is an uncommon condition that results in simultaneous esophageal contractions. Current medical treatment of DES is frequently unsatisfactory. We hypothesized that, as a smooth muscle relaxant, peppermint oil may improve the manometric findings in DES.

Eight consecutive patients with chest pain or dysphagia and who were found to have DES were enrolled during their diagnostic esophageal manometry. An eight-channel perfusion manometry system was used. Lower esophageal sphincter pressure and contractions of the esophageal body after 10 wet swallows were assessed before and 10 minutes after the ingestion of a solution containing five drops of peppermint oil in 10 mL of water. Each swallow was assessed for duration (seconds), amplitude (mm Hg), and proportion of simultaneous and multiphasic esophageal contractions.

Lower esophageal sphincter pressures and contractile pressures and durations in both the upper and lower esophagus were no different before and after the peppermint oil. Peppermint oil completely eliminated simultaneous esophageal contractions in all patients (p < 0.01). The number of multiphasic, spontaneous, and missed contractions also improved. Because normal esophageal contractions are characteristically uniform in appearance, variability of esophageal contractions was compared before and after treatment. The variability of amplitude improved from 33.4 +/- 36.7 to 24.9 +/- 11.0 mm Hg (p < 0.05) after the peppermint oil. The variability for duration improved from 2.02 +/- 1.80 to 1.36 +/- 0.72 seconds (p < 0.01). Two of the eight patients had chest pain that resolved after the peppermint oil.

This data demonstrates that peppermint oil improves the manometric features of DES.

The cause of diffuse esophageal spasm (DES) has not been clearly established, and effective treatment is lacking. To determine whether a psychosomatic approach can be effective in treating DES patients, nine patients and 26 healthy volunteers were studied. Esophageal manometry and psychological testing were performed in both groups. The psychological background of the DES patients was investigated. Psychiatric diagnoses were made according to the criteria outlined in the Diagnostic and Statistical Manual of Mental Disorders, 3rd edition, revised. The authors started psychosomatic treatment with isosorbide dinitrate for 1 month, then prescribed serotonin reuptake inhibitor antidepressants for an additional month. Anxiety and depression scores were substantially higher for the DES group than for the control group. Five of the nine DES patients (56%) were diagnosed as having major psychiatric disorders. Only one patient showed improvement with isosorbide dinitrate, and eight patients improved with antidepressants. These initial observations suggest that psychosomatic treatment with antidepressants may be effective in the treatment of DES.

We wanted to evaluate the clinical significance of the esophageal peristalsis that appears in some achalasia patients after treatment.

We prospectively investigated the reappearance of esophageal peristalsis in 106 achalasic patients treated with forceful dilatation under endoscopic control (86 metallic dilatations and 20 pneumatic dilatations) and followed up clinically and manometrically for 1 year. Patients were divided in two groups in accordance with the presence (n = 26) or persistent absence (n = 80) of postdilatation esophageal peristalsis.

Before treatment, clinical data and manometric findings were comparable in both groups except for esophageal wave amplitude, which was higher in patients with postdilatation peristalsis (36 +/- 5 mmHg versus 24 +/- 2 mmHg, P < 0.05). One year after dilatation manometric findings were similar in the two groups, but esophageal wave amplitude remained higher in the group with postdilatation peristalsis (46 +/- 4 mmHg versus 21 +/- 2 mmHg, P < 0.05). The proportion of patients with persistent dysphagia was similar in the two groups (15% versus 12.5%). However, 10 patients with postdilatation peristalsis (38%) complained of chest pain as opposed to only 5 patients (6%) in the group with aperistalsis (P < 0.01).

The appearance of esophageal peristalsis after forceful dilatation in achalasic patients is frequently associated with persistent or new chest pain.

There are no requirements concerning the amplitude of simultaneous contractions among the present criteria for the manometric diagnosis of diffuse esophageal spasm. The purpose of this investigation was to determine whether the current criteria effectively identify an appropriately homogenous patient population. Sixty consecutive motility tracings that met the criteria for diffuse esophageal spasm were evaluated. A bimodal distribution of the highest simultaneous esophageal contraction for each patient was observed. One group's (N = 29) highest simultaneous esophageal contractile amplitude was < or = 74 mm Hg, the other's (N = 31) highest simultaneous esophageal contractile amplitude was > or = 100 mm Hg. Group 1 had significantly decreased lower esophageal sphincter pressure, lower peristaltic amplitude, more aperistalsis, fewer simultaneous contractions, and fewer complaints of chest pain. These comparisons suggest that consideration be given to the amplitude of simultaneous esophageal contractions in the manometric diagnosis of diffuse esophageal spasm.

Esophageal function testing has an important place in the investigation of a significant proportion of patients with esophageal disorders. Appropriate application of these tests requires a proper understanding of their capabilities and limitations and careful primary assessment by other modalities. Esophageal manometry is most useful for assessing significant troublesome dysphagia in the absence of organic obstruction. Esophageal pH monitoring is an important adjunct to clinical assessment and endoscopy in the diagnosis of reflux disease. Although it is the gold standard for the measurement of esophageal acid exposure and assessment of the relationship of symptoms to reflux, there are weakness in both of these functions that should be understood when applying the test to the diagnosis of reflux disease.

As recently suggested, nitric oxide (NO) may play an important role in the regulation of esophageal motility, being partly responsible for the latency period and latency gradient between the onset of a swallow and contractions of esophageal circular smooth muscles. Diffuse esophageal spasm appears to be a classical example in which the mechanisms normally responsible for the physiologic timing of the contractions occurring in the esophageal body after swallowing are disturbed.

Five patients (one male and four female; age, 18-48 years) with symptomatic esophageal spasm were give glyceryl trinitrate (GTN) intravenously in gradually increasing doses or L-arginine on two separate occasions and underwent manometric measurements of esophageal motility after wet swallows, using a multilumen perfused catheter system (Synetics Medical, Stockholm, Sweden). The amplitude, duration, and propagation of the contractions and the latency period were analyzed, using specially designed software. Additionally, during the GTN infusion period arterial blood pressure was measured every 5 min,

GTN infusion given at a dose of 100 to 200 micrograms/kg-h intravenously caused the occurrence of and a dose-dependent elongation of the latency period after swallowing. The mean amplitude of the contractions did not show any significant alterations, whereas the mean duration of the contractions decreased significantly, from 11.2 +/- 4.8 sec to 5.4 +/- 0.8 sec. These effects were accompanied by significant alleviation of symptoms during swallowing. Interestingly, no adverse side effects such as headache or flush were observed at any dose of GTN. The blood pressure did not show any changes during the studies in any of the five patients. Administration of L-arginine (300 mg/kg-h intravenously) did not cause any significant alterations of motility pattern or alleviation of dysphagia.

1) NO may play an important role in the control of human esophageal motility, being involved in the mechanisms responsible for the timing of propulsive contractions in the body after swallowing; 2) GTN may to be of benefit in the treatment of diffuse esophageal spasm in symptomatic patients; and 3) patients with diffuse esophageal spasm may have a malfunction in endogenous NO synthesis and/or degradation.

Primary esophageal motility disorders (achalasia, diffuse esophageal spasm, and intermediate forms) are suggested to be caused by different degrees of inhibitory dysfunction; however, direct evidence for this hypothesis has never been presented in humans. The aim of this study was to measure the degree of inhibition that precedes deglutitive contractions in patients with primary motility disorders.

Deglutitive inhibition was examined in patients with primary motility disorders: 9 with achalasia, 6 with symptomatic diffuse esophageal spasm, and 5 with intermediate forms. An artificial high-pressure zone was created in the esophageal body by inflating a balloon to a critical level, and pressure changes were measured at the interface between the balloon and esophageal wall. Inhibition was visualized as a relaxation of the artificial high-pressure zone.

An inverse relationship was found between the degree of inhibition and the propagation velocity of the deglutitive contraction (r = 0.75; P < 0.001). Normally propagated contractions were preceded by an inhibition of 84.2% +/- 3.6%; fast-propagating contractions were preceded by partial inhibition of 40.6% +/- 6.2%; and, in case of simultaneous contractions, inhibition was absent, i.e., 2.6% +/- 1.6%.

The spectrum of primary motility disorders is an expression of a progressively failing deglutitive inhibition.

Patients with unexplained chest pain represent a major clinical dilemma for primary-care physicians, gastroenterologists, and cardiologists. References to this prevalent clinical problem date to more than 150 years ago; confusion about its pathophysiology has resulted in the use of a variety of descriptive terms such as "noncardiac," "atypical," and "angiographically negative" chest pain. Since none of these terms applies to all cases, the description "chest pain of undetermined origin" may be preferable. Because the esophagus has a similar location and innervation as the heart, an esophageal source for unexplained chest pain syndromes has been frequently suggested. Recent studies have emphasized the importance of gastroesophageal reflux as a likely component of esophageal pain. Moreover, "irritable esophagus" is an emerging concept that implies a generalized alteration in esophageal pain threshold, that is, abnormal nociception. The potential effects of stress or altered psychological states in this phenomenon must be considered, and the role of "panic attacks" in the production of pain in these patients needs to be clarified. In addition, stress may produce altered esophageal motility and lead to manometric abnormalities such as the "nutcracker esophagus" or a hypertensive lower esophageal sphincter. Finally, the precise contribution of the heart in producing pain in patients with normal coronary angiograms remains unclear because the precise role of microvascular angina has yet to be clarified.