| Cor pulmonale in a patient with Brown-Vialetto-Van Laere syndrome: a case report. | |
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MedLine Citation:
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PMID: 21055769 Owner: NLM Status: In-Process |
Abstract/OtherAbstract:
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Brown-Vialetto-Van Laere syndrome (BVVLS) is a rare neurological disease characterized by sensorineural hearing loss and multiple cranial nerve palsies, usually involving the VIIth and IXth to XIIth cranial nerves. We describe the clinical and pathological features of a 33-year-old woman with BVVLS. The patient developed progressive exertional dyspnea, with clinical and laboratory findings of right-sided heart failure and pulmonary hypertension. She developed status epilepticus in the setting of cardiac deterioration and respiratory infection, and died of cardiogenic and septic shock. Autopsy disclosed bilateral neuronal loss and gliosis in the inferior colliculi, locus coeruleus and facial and vestibular nuclei. Cor pulmonale is a complication of hypoventilation-induced hypoxia and hypercapnia and had not yet been reported in BVVLS. |
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Authors:
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Francisco Pereira da Silva-Júnior; Rafael de Deus Moura; Sérgio Rosemberg; Paulo Eurípedes Marchiori; Luiz Henrique Martins Castro |
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Publication Detail:
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Type: Journal Article Date: 2010-11-05 |
Journal Detail:
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Title: Journal of the neurological sciences Volume: 300 ISSN: 1878-5883 ISO Abbreviation: J. Neurol. Sci. Publication Date: 2011 Jan |
Date Detail:
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Created Date: 2010-12-24 Completed Date: - Revised Date: - |
Medline Journal Info:
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Nlm Unique ID: 0375403 Medline TA: J Neurol Sci Country: Netherlands |
Other Details:
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Languages: eng Pagination: 155-6 Citation Subset: IM |
Copyright Information:
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Copyright © 2010 Elsevier B.V. All rights reserved. |
Affiliation:
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Department of Neurology, Universidade de São Paulo, São Paulo, Brazil. franciscops@hotmail.com |
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From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine
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