| Mitral valve prolapse and ophthalmoplegia: a progressive, cardioneurologic syndrome. | |
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MedLine Citation:
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PMID: 6992676 Owner: NLM Status: MEDLINE |
Abstract/OtherAbstract:
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Disorders characterized by both neurologic (ataxia, ophthalmoplegia, ptosis, neuromyopathy) and cardiologic (heart block, cardiomyopathy) abnormalities have been previously called the "ophthalmoplegia plus" syndromes. Most are not due to a specific enzyme defect or metabolic abnormality and thus may be similar phenotypic expressions of diverse causes. We studied seven patients with progressive external ophthalmoplegia and variable ataxia, with mitral valve prolapse and mitral regurgitation that progressed in severity as did the neuromuscular manifestations. Abnormal skeletal muscle biopsies showed "ragged-red" fibers or congenital fiber type disproportion; serum alanine levels were elevated; in-vivo and in-vitro tests of pyruvate metabolism gave abnormal results; C4 complement was decreased; and the patients' fibroblasts bound immunoglobulin when incubated with autologous serum. These data suggest a distinct neuromuscular disorder with metabolic and immunologic features associated with mitral valve prolapse and progressive mitral regurgitation. |
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Authors:
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J R Darsee; C L Miklozek; S B Heymsfield; L C Hopkins; N K Wenger |
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Publication Detail:
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Type: Case Reports; Journal Article; Research Support, U.S. Gov't, P.H.S.; Retracted Publication |
Journal Detail:
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Title: Annals of internal medicine Volume: 92 ISSN: 0003-4819 ISO Abbreviation: Ann. Intern. Med. Publication Date: 1980 Jun |
Date Detail:
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Created Date: 1980-08-25 Completed Date: 1980-08-25 Revised Date: 2006-11-15 |
Medline Journal Info:
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Nlm Unique ID: 0372351 Medline TA: Ann Intern Med Country: UNITED STATES |
Other Details:
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Languages: eng Pagination: 735-41 Citation Subset: AIM; IM |
Export Citation:
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APA/MLA Format Download EndNote Download BibTex |
| MeSH Terms | |
Descriptor/Qualifier:
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Adult Amino Acids / blood Echocardiography Humans Male Mitral Valve Insufficiency / complications Mitral Valve Prolapse / complications*, diagnosis, immunology, metabolism, pathology Muscles / pathology Myocardium / metabolism Ophthalmoplegia / complications*, immunology, metabolism, pathology Pyruvates / metabolism Syndrome |
| Chemical | |
Reg. No./Substance:
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0/Amino Acids; 0/Pyruvates |
| Comments/Corrections | |
Retraction In:
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Ann Intern Med. 1983 Aug;99(2):275-6
[PMID:
6349461
]
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From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine
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