Document Detail

Linear scleroderma associated with progressive brain atrophy.
MedLine Citation:
PMID:  12536035     Owner:  NLM     Status:  MEDLINE    
Linear scleroderma (LS) is characterized by scleroatrophic lesions affecting limbs and legs, unilaterally. Neurological involvement may be associated with ipsilateral facial and skull involvement in disorders referred to clinically as LS 'en coup de sabre', and Parry-Romberg syndrome. We report a child with LS presenting with a severe neurological disorder characterized by epilepsy, progressive mental deterioration and a rapid process of atrophy involving the ipsilateral cerebral hemisphere, but not associated with an overlying facial structure involvement. Functional brain studies showed a reduction in the diameter of the left internal carotid and of the left middle cerebral artery. Our observations suggest that neuroimaging studies should be considered in all patients with linear scleroderma, and such studies become necessary when neurological symptoms occur.
Salvatore Grosso; Antonella Fioravanti; Giovanni Biasi; Elvira Conversano; Roberto Marcolongo; Guido Morgese; Paolo Balestri
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Publication Detail:
Type:  Case Reports; Journal Article    
Journal Detail:
Title:  Brain & development     Volume:  25     ISSN:  0387-7604     ISO Abbreviation:  Brain Dev.     Publication Date:  2003 Jan 
Date Detail:
Created Date:  2003-01-21     Completed Date:  2003-04-04     Revised Date:  2009-11-03    
Medline Journal Info:
Nlm Unique ID:  7909235     Medline TA:  Brain Dev     Country:  Netherlands    
Other Details:
Languages:  eng     Pagination:  57-61     Citation Subset:  IM    
Department of Pediatrics, Obstetrics and Reproductive Medicine, University of Siena, Viale M. Bracci - Le scotte, 53100 Siena, Italy.
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MeSH Terms
Brain Diseases / complications*,  pathology*,  physiopathology,  radiography
Carotid Artery, Internal / pathology
Cerebral Angiography
Diagnosis, Differential
Facial Hemiatrophy / pathology
Magnetic Resonance Imaging
Middle Cerebral Artery / pathology
Scleroderma, Localized / complications*,  pathology,  physiopathology*
Tomography, Emission-Computed, Single-Photon
Tomography, X-Ray Computed

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