Esophageal contractions in patients with chronic progressive external ophthalmoplegia

Chronic progressive external ophthalmoplegia is a mitochondrial myopathy that causes muscular or multisystem symptoms and has dysphagia as one manifestation.

To evaluate esophageal contractions in patients with chronic progressive external ophthalmoplegia.

We studied 14 patients with chronic progressive external ophthalmoplegia and 16 asymptomatic volunteers. The diagnosis of the disease was established by the clinical picture and by mitochondrial DNA analysis in skeletal muscle. We used the manometric method with a perfusion catheter that recorded the esophageal contractions at 2, 7, 12, 17, and 22 cm from the lower esophageal sphincter (LES). All subjects performed in the supine position 20 swallows of a 5-ml bolus of water at room temperature, ten every 30 s and ten every 10 s.

The amplitude, duration, and area under the curve of contractions at 17 and 22 cm from the LES were lower in patients than in volunteers for swallows performed at 10-s and 30-s intervals (P<0.01). There was no difference in contractions at 7 and 2 cm, except for the contractions at 2 cm after swallows performed at 30-s intervals. The interval between the onset of contractions between 7 and 2 cm and between 22 and 2 cm was lower in patients than in volunteers, with swallows performed every 10 s and every 30 s.

There is impairment of esophageal contractions in patients with chronic progressive external ophthalmoplegia, mainly in the proximal esophageal body.