Document Detail

Manometric study in Kearns-Sayre syndrome.
MedLine Citation:
PMID:  11422310     Owner:  NLM     Status:  MEDLINE    
Although swallowing difficulties have been described in patients with Kearns-Sayre syndrome (KSS), the spectrum of manometric characteristics of dysphagia is not yet well known. Moreover, it is conceivable that a combination of various degrees of swallowing difficulties with different patterns in manometric studies exist, each playing a major role in the prognosis, natural history, and quality of life of KSS patients. An 18-year-old girl diagnosed at the age of 5 years with KSS (muscle biopsy) was admitted to our department with an upper respiratory tract infection and dysphagia. Clinical examination revealed growth retardation, external ophthalmoplegia, pigmentary retinopathy, impaired hearing, and ataxia. An electrocardiogram revealed cardiac conduction defects (long Q-T), and brain magnetic resonance imaging showed abnormalities in the cerebellar hemispheres. A manometric and motility study for dysphagia was conducted and the pharynx and upper esophageal sphincter (UES) resting pressures were similar to control group values, but the swallowing peak contraction pressure of the pharynx and the closing pressure of the UES were very low and could not promote effective peristaltic waves. Relaxation and coordination of the UES were not affected although pharyngeal and upper esophagus peristaltic waves proved to be very low and, consequently, were practically ineffective. The patient was started on treatment comprising a diet rich in potassium, magnesium, and calcium, and oral administration of vitamin D and co-enzyme Q10 100 mg daily; she was discharged 6 days later with apparent clinical improvement.
K H Katsanos; D Nastos; V Noussias; D Christodoulou; A Kappas; E V Tsianos
Publication Detail:
Type:  Case Reports; Journal Article    
Journal Detail:
Title:  Diseases of the esophagus : official journal of the International Society for Diseases of the Esophagus / I.S.D.E     Volume:  14     ISSN:  1120-8694     ISO Abbreviation:  Dis. Esophagus     Publication Date:  2001  
Date Detail:
Created Date:  2001-06-25     Completed Date:  2001-09-20     Revised Date:  2009-11-19    
Medline Journal Info:
Nlm Unique ID:  8809160     Medline TA:  Dis Esophagus     Country:  Australia    
Other Details:
Languages:  eng     Pagination:  63-6     Citation Subset:  IM    
Department Hepato-Gastroenterology, Medical School, University of Ioannina, Ioannina, Greece.
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MeSH Terms
Deglutition Disorders / etiology,  pathology*,  physiopathology*
Esophagus / pathology,  physiopathology
Kearns-Sayre Syndrome / complications,  pathology*,  physiopathology*
Pharynx / pathology,  physiopathology

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