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Autonomic dysfunction presenting as orthostatic intolerance in patients suffering from mitochondrial cytopathy.
MedLine Citation:
PMID:  20960537     Owner:  NLM     Status:  In-Process    
BACKGROUND: Disturbances in autonomic nervous system function have been reported to occur in patients suffering from mitochondrial cytopathies. However, there is paucity of literature on the occurrence of orthostatic intolerance (OI) in these patients. We report on a series of patients diagnosed with mitochondrial cytopathy who developed features of autonomic dysfunction in the form of OI.
METHODS: This was a single-center report on a series of 6 patients who were followed in our clinic for orthostatic intolerance. All of these patients had a diagnosis of mitochondrial cytopathy on the basis of muscle biopsy and were being followed at a center specializing in the treatment of mitochondrial disorders. This study was approved by our local institutional review board. Each of the patients had suffered from symptoms of fatigue, palpitations, near syncope, and syncope. The diagnosis of OI was confirmed by head-up tilt test. Collected data included demographic information, presenting symptoms, laboratory data, tilt-table response, and treatment outcomes.
RESULTS: Six patients (3 females) were identified for inclusion in this report. The mean age of the group was 48 ± 8 years (range, 40-60 years). All of these patients underwent head-up tilt table testing and all had a positive response that reproduced their clinical symptoms. Among those having an abnormal tilt-table pattern, 1 had a neurocardiogenic response, 1 had a dysautonomic response, and 4 had a postural orthostatic tachycardia response. All but 1 patient reported marked symptom relief with pharmacotherapy. The patient who failed pharmacotherapy received a dual-chamber closed-loop pacemaker and subsequently reported marked improvement in her symptoms with elimination of her syncope.
CONCLUSIONS: Orthostatic intolerance might be a significant feature of autonomic nervous system dysfunction in patients suffering from mitochondrial cytopathy.
Khalil Kanjwal; Beverly Karabin; Yousuf Kanjwal; Bilal Saeed; Blair P Grubb
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Publication Detail:
Type:  Journal Article    
Journal Detail:
Title:  Clinical cardiology     Volume:  33     ISSN:  1932-8737     ISO Abbreviation:  Clin Cardiol     Publication Date:  2010 Oct 
Date Detail:
Created Date:  2010-10-20     Completed Date:  -     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  7903272     Medline TA:  Clin Cardiol     Country:  United States    
Other Details:
Languages:  eng     Pagination:  626-9     Citation Subset:  IM    
Copyright Information:
© 2010 Wiley Periodicals, Inc.
Section of Electrophysiology, Division of Cardiology, Department of Medicine, The University of Toledo Medical Center, Toledo, Ohio 43614, USA.
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