| Parry-romberg syndrome and rasmussen encephalitis: possible association. Clinical and neuroimaging features. | |
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MedLine Citation:
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PMID: 19555404 Owner: NLM Status: In-Data-Review |
Abstract/OtherAbstract:
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Parry-Romberg syndrome (PRS) is a sporadic disease of unknown etiology with typical onset in childhood or in young adults. It is characterized by a slow and progressive atrophy affecting one side of the face, the skin, the subcutaneous tissue, the muscles, the cartilages, and the underlying bony structures. The neurological symptoms usually include focal epilepsy, migraine, and unilateral brain lesions on the same side as the atrophy. A common neuroimaging finding of the syndrome is white matter high signal intensity on brain magnetic resonance (MR) imaging. Rasmussen encephalitis (RE) is a rare and chronic inflammatory disease of the brain that begins in the first decade of life and more rarely in adolescents and adults. It usually involves one hemisphere with focal cortical inflammation. Neurologic symptoms are intractable seizures and progressive hemiplegia. Both PRS and RE are often associated with other inflammatory or autoimmune disorders and only 1 case of both syndromes has been reported in literature. We report the clinical and neuroradiological findings in a 6-year-old boy, presenting with focal hemifacial and arm motor seizures and progressive facial hemiatrophy. Serial MR imaging studies revealed progressive brain hemispheric signal alterations and atrophy. This would thus suggest acoexistence of PRS and RE. |
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Authors:
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Daniela Longo; Amalia Paonessa; Nicola Specchio; Luciana Nogueira Delfino; Dianela Claps; Lucia Fusco; Francesco Randisi; Elisabetta Genovese; Federico Vigevano; Giuseppe Fariello |
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Publication Detail:
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Type: Journal Article |
Journal Detail:
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Title: Journal of neuroimaging : official journal of the American Society of Neuroimaging Volume: 21 ISSN: 1552-6569 ISO Abbreviation: J Neuroimaging Publication Date: 2011 Apr |
Date Detail:
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Created Date: 2011-03-25 Completed Date: - Revised Date: - |
Medline Journal Info:
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Nlm Unique ID: 9102705 Medline TA: J Neuroimaging Country: United States |
Other Details:
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Languages: eng Pagination: 188-93 Citation Subset: IM |
Copyright Information:
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Copyright © 2009 by the American Society of Neuroimaging. |
Affiliation:
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From the Department of Radiology (DL, AP, LND, FR, GF), Division of Neurology (NS, DC, LF, FV), and Department of Occupational Health and Safety-Medical Physics, Bambino Gesù Children's Hospital IRCCS, Rome, Italy (EG). |
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