1 40 1 Text A resource consisting primarily of words for reading. Examples include books, letters, dissertations, poems, newspapers, articles, archives of mailing lists. Note that facsimiles or images of texts are still of the genre Text. Citation List Month Backlog URL Address <a href="http://doi.org/10.1055/s-2007-973503" target="_blank" rel="noreferrer">http://doi.org/10.1055/s-2007-973503</a> Dublin Core The Dublin Core metadata element set is common to all Omeka records, including items, files, and collections. For more information see, http://dublincore.org/documents/dces/. Title A name given to the resource Clinical and neuroradiological follow-up in mucopolysaccharidosis type III (Sanfilippo syndrome) Publisher An entity responsible for making the resource available Neuropediatrics Date A point or period of time associated with an event in the lifecycle of the resource 1999 Subject The topic of the resource Child; Female; Humans; Male; Disease Progression; Magnetic Resonance Imaging; Preschool; infant; Q3 Literature Search; Atrophy; Brain/abnormalities/pathology; Mental Retardation/etiology; Mucopolysaccharidosis III/pathology/physiopathology; Psychomotor Disorders/diagnosis/etiology Creator An entity primarily responsible for making the resource Barone R; Nigro F; Triulzi F; Musumeci S; Fiumara A; Pavone L Description An account of the resource Mucopolysaccharidosis type III (Sanfilippo syndrome) is an autosomal recessive disorder characterised by progressive nervous system involvement with mental retardation, behavioural problems and seizures. Three patients, of 20 months to 12 years of age, were followed up for 3 years both clinically and by using brain magnetic resonance imaging (MRI). Our results suggest that in MPS III patients MRI findings, including atrophy and abnormal or delayed myelination, may precede the onset of overt neurological symptoms. The increasing neurological morbidity is accompanied by different degrees of progressive atrophic changes, mainly affecting the cerebral cortex and the corpus callosum. However, it appears that, across subjects, the rate of MRI changes is unrelated to the severity of the clinical phenotype. On this basis it could be argued that in MPS III the worsening of the neurological symptoms might not necessarily reflect only the progressive cerebral abnormalities detectable by MRI. 1999 Identifier An unambiguous reference to the resource within a given context <a href="http://doi.org/10.1055/s-2007-973503" target="_blank" rel="noreferrer">10.1055/s-2007-973503</a> Rights Information about rights held in and over the resource Article information provided for research and reference use only. PedPalASCNET does not hold any rights over the resource listed here. All rights are retained by the journal listed under publisher and/or the creator(s). Type The nature or genre of the resource Journal Article 1999 Atrophy Backlog Barone R Brain/abnormalities/pathology Child Disease Progression Female Fiumara A Humans Infant Journal Article Magnetic Resonance Imaging Male Mental Retardation/etiology Mucopolysaccharidosis III/pathology/physiopathology Musumeci S Neuropediatrics Nigro F Pavone L Preschool Psychomotor Disorders/diagnosis/etiology Q3 Scoping Review Results Triulzi F