achalasia is a primary esophageal motor disorder of unknown etiology characterized by selective loss of inhibitory neurons in the esophageal wall , resulting in insufficient relaxation of the lower esophageal sphincter and loss of esophageal peristalsis.1,2 jackhammer esophagus is a newly introduced term to describe patients with at least 20% of the swallows with distal contractile integral ( dci )  8000 mmhg.sec.cm.3 several reports have described patients with diffuse esophageal spasm ( des ) , non - specific esophageal motor disorder ( nsemd ) , nutcracker esophagus , and gastroesophageal reflux disease ( gerd ) progressing to achalasia.49 although no causal relationship has been identified , these reports suggest that the different esophageal motor disorders represent a spectrum rather than unique and stable disorders .
we describe , for the first time , a case of a patient who progressed from jackhammer esophagus to type ii achalasia .
a 66 year - old woman with a history of atrial fibrillation , hypertension , gastritis , and hypothyroidism presented to our clinic in july 2014 with progressive dysphagia for solids and liquids over 18 months .
her symptoms occurred daily and resulted in an 11-kg weight loss over this period of time .
she denied heartburn , regurgitation , choking or coughing during eating , chest pain , vomiting , abdominal pain , or change in bowel habits .
the barium swallow showed a mildly dilated esophagus with tertiary contractions , delayed emptying of the esophagus , and a narrowed gastroesophageal junction ( fig .
1 ) . the upper endoscopy demonstrated mild - moderate antral gastritis , normal appearing esophageal mucosa , and it was noted that the patient might have a tight gastroesophageal junction .
unfortunately , after completing these tests she missed 3 follow - up appointments and was seen in our clinic only one year later .
given her progressive dysphagia symptoms and weight loss , a high resolution esophageal manometry ( hrem ) was performed ( fig .
the study showed a normal median integrated residual pressure of 8 mmhg , normal mean resting pressure of 21 mmhg , normal mean residual pressure of 7 mmhg , and mean dci of 10 770 mmhg sec  cm .
of the 10 swallows , 30% were hypercontractile ( > 8000 mmhg sec  cm ) , 50% were normal and 20% simultaneous .
overall , the findings were consistent with jackhammer esophagus based on the chicago classification of motility disorders.3 pantoprazole was increased to 40 mg twice daily with plan to add a pain modulator if her symptoms recurred .
unfortunately , she was lost to follow - up again until she was seen in our clinic a year later in june 2015 .
she continued to have worsening dysphagia , lost an additional 7 kg , and reported new onset of occasional post - prandial chest tightness . at this point ,
3 ) . this study demonstrated diffuse esophageal dilatation with retained secretions and barium with only 020% change in esophageal volume after 5 minutes .
in addition , tertiary peristaltic waves within the distal esophagus , limited emptying of the contrast into the stomach and an air - fluid level were also noted .
4 ) revealing a median integrated residual pressure of 71.5 mmhg , mean resting pressure of 97 mmhg , and mean residual pressure of 90 mmhg .
there was panesophageal pressurization with every swallow and 80% of the swallows demonstrated prolonged pan - pressurization , all consistent with type ii achalasia .
therapeutic options were discussed with the patient , and she elected to undergo heller myotomy .
the patient was referred to general surgery but presented urgently to gi clinic 2 weeks later for worsening dysphagia , dehydration , weakness , inability to tolerate oral intake , and an additional 7-kg weight loss .
the patient was admitted to the hospital and was noted to develop acute kidney injury .
in addition , a nasogastric tube was placed and she was started on tube feeds to optimize her nutritional status . a week after discharge the patient underwent heller myotomy with dor fundoplication . on a follow - up visit with her general surgeon , she reported that she was tolerating liquids without any difficulties .
to our knowledge , this is the first case that describes progression of jackhammer esophagus to achalasia over a period of about one year .
smart et al5 reported 5 patients with gerd who subsequently developed achalasia over a period of 210 years .
robson et al4 reported a patient with gerd who developed des one year after initial diagnosis and achalasia one year later .
several reports describe the progression of des to achalasia.68 khatami et al10 conducted the first prospective cohort study with 12 patients .
they observed the progression of des to achalasia in only one patient after 10.6 years .
they also noted that low esophageal body amplitude contractions was a predictor of this progression .
fontes et al11 conducted the largest prospective study to date to assess the progression of des to achalasia .
patients with gerd confirmed by ph monitoring or systemic diseases that may affect the esophagus were excluded .
five ( 14% ) of the patients progressed to achalasia at a mean follow - up of 2.1 years .
although demographic characteristics were not predictive of the transition to achalasia , the authors observed that amplitude contractions of less than 50 mmhg was a predictive factor ( p = 0.002 ) .
anggianash et al9 described the progression of nutcracker esophagus to achalasia in one patient after 3 years .
vantrappen et al13 described six patients with nsemd who progressed to achalasia . while our patient presented with symptoms that could be considered classic for achalasia , her initial conventional manometry was consistent with nsemd .
only the second hrem as well as the timed barium esophagram were consistent with achalasia .
this progression within one year of an esophageal motor disorder was faster than what has been documented in previous reports .
however , this case suggests that all esophageal motility disorders represent a spectrum of disorders , where patients may progress over time from one to another .
this case also demonstrates the importance of following patients who were diagnosed with non - achalasia motility disorders with a repeat hrem in one year to assess for progression or a shift to a different esophageal motility disorder . because most of the studies documenting progression of a motility disorder to achalasia were case reports using conventional manometry ,
long - term studies evaluating this progression with hrem are needed . perhaps with the current availability of hrem
, researchers may be able to identify predictive factors associated with progression of certain esophageal motility disorders to achalasia .
 it has been suggested that patients with certain motility disorders may progress overtime to develop achalasia . 
 we describe a 66 year - old woman who presented with dysphagia for solids and liquids for a period of 18 months . 
 her initial workup showed normal endoscopy and non - specific esophageal motility disorder on conventional manometry . 
 six months later , due to persistence of symptoms , the patient underwent a high resolution esophageal manometry ( hrem ) demonstrating jackhammer esophagus . 
 the patient was treated with a high dose proton pump inhibitor but without resolution of her symptoms . during the last year , the patient reported repeated episodes of food regurgitation and a significant weight loss . 
 a repeat hrem revealed type ii achalasia . 
 multiple case reports , and only a few prospective studies have demonstrated progression from certain esophageal motility disorders to achalasia 
 . however , this report is the first to describe a case of jackhammer esophagus progressing to type ii achalasia . 
Introduction
Case Report
Discussion