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Titolo:
Clinical and neuroradiological follow-up in mucopolysaccharidosis type III(Sanfilippo syndrome)
Autore:
Barone, R; Nigro, F; Triulzi, F; Musumeci, S; Fiumara, A; Pavone, L;
Indirizzi:
Univ Catania, Inst Paediat, Div Paediat Neurol, Pediat Clin, I-95125 Catania, Italy Univ Catania Catania Italy I-95125 , Pediat Clin, I-95125 Catania, Italy Hosp San Raffaele, Dept Paediat, I-20132 Milan, Italy Hosp San Raffaele Milan Italy I-20132 Dept Paediat, I-20132 Milan, Italy Hosp San Raffaele, Dept Neuroradiol, I-20132 Milan, Italy Hosp San Raffaele Milan Italy I-20132 Neuroradiol, I-20132 Milan, Italy Univ Sassari, Dept Paediat, I-07100 Sassari, Italy Univ Sassari Sassari Italy I-07100 Dept Paediat, I-07100 Sassari, Italy
Titolo Testata:
NEUROPEDIATRICS
fascicolo: 5, volume: 30, anno: 1999,
pagine: 270 - 274
SICI:
0174-304X(199910)30:5<270:CANFIM>2.0.ZU;2-Q
Fonte:
ISI
Lingua:
ENG
Soggetto:
BRAIN;
Keywords:
mucopolysaccharidosis type III; MRI; follow-up;
Tipo documento:
Article
Natura:
Periodico
Settore Disciplinare:
Clinical Medicine
Life Sciences
Citazioni:
11
Recensione:
Indirizzi per estratti:
Indirizzo: Barone, R Univ Catania, Inst Paediat, Div Paediat Neurol, Pediat Clin, VleA Doria 6, I-95125 Catania, Italy Univ Catania Vle A Doria 6 Catania Italy I-95125 Catania, Italy
Citazione:
R. Barone et al., "Clinical and neuroradiological follow-up in mucopolysaccharidosis type III(Sanfilippo syndrome)", NEUROPEDIAT, 30(5), 1999, pp. 270-274

Abstract

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.

ASDD Area Sistemi Dipartimentali e Documentali, Università di Bologna, Catalogo delle riviste ed altri periodici
Documento generato il 26/11/20 alle ore 09:05:08