| Eponym : Rasmussen syndrome. | |
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MedLine Citation:
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PMID: 20177700 Owner: NLM Status: In-Process |
Abstract/OtherAbstract:
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Rasmussen's syndrome (RS) is a rare acquired progressive inflammatory encephalopathy characterized by drug-resistant partial seizures and cognitive deterioration resulting from a gradual impairment and a subsequent atrophy of a single brain hemisphere. It was firstly described by Theodore Rasmussen in 1958. The original etiopathogenic hypothesis of a chronic viral infection no longer holds. Today, the presumed mechanism is a complex autoimmune process comprising an active role of both cytotoxic T lymphocytes and circulating autoantibodies, activating the subunit 3 of the ionotropic glutamate receptor (GluR3Ab). Several medical options, such as antiepileptic drugs and immunomodulatory therapies, deserve consideration to face epileptic manifestations and to slow neurologic regression. Yet, all these therapies turn out to be almost always just temporizing measures. Surgical disconnection of the affected hemisphere ("functional hemispherectomy") is the best therapeutic choice to achieve the arrest of the disease, a good seizure control, the block of neurologic decline, and the improvement of patient's quality of life. |
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Authors:
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Mario Mastrangelo; Rosanna Mariani; Alessandra Menichella |
Publication Detail:
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Type: Journal Article Date: 2010-02-23 |
Journal Detail:
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Title: European journal of pediatrics Volume: 169 ISSN: 1432-1076 ISO Abbreviation: Eur. J. Pediatr. Publication Date: 2010 Aug |
Date Detail:
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Created Date: 2010-06-23 Completed Date: - Revised Date: - |
Medline Journal Info:
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Nlm Unique ID: 7603873 Medline TA: Eur J Pediatr Country: Germany |
Other Details:
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Languages: eng Pagination: 919-24 Citation Subset: IM |
Affiliation:
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Child Neurology Division, Department of Pediatrics, La Sapienza-University of Rome, Viale Regina Elena, 324 00161 Rome, Italy. mari0mastrangelo@tiscali.it |
Export Citation:
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From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine
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