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1.
Neuroradiology ; 53(2): 141-8, 2011 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-20959972

RESUMO

INTRODUCTION: Neurofibromatosis type 1 (NF1) is frequently associated with hyperintense lesions on T2-weighted images called "unidentified bright objects" (UBO). To better characterize the functional significance of UBO, we investigate the basal ganglia and thalamus using spectroscopic imaging in children with NF1 and compare the results to anomalies observed on T2-weighted images. METHODS: Magnetic resonance (MR) data of 25 children with NF1 were analyzed. On the basis of T2-weighted images analysis, two groups were identified: one with normal MR imaging (UBO- group; n = 10) and one with UBO (UBO+ group; n = 15). Within the UBO+ group, a subpopulation of patients (n = 5) only had lesions of the basal ganglia. We analyzed herein seven regions of interest (ROIs) for each side: caudate nucleus, capsulo-lenticular region, lateral and posterior thalamus, thalamus (lateral and posterior voxels combined), putamen, and striatum. For each ROI, a spectrum of the metabolites and their ratio was obtained. RESULTS: Patients with abnormalities on T2-weighted images had significantly lower NAA/Cr, NAA/Cho, and NAA/mI ratios in the lateral right thalamus compared with patients with normal T2. These abnormal spectroscopic findings were not observed in capsulo-lenticular regions that had UBO but in the thalamus region that was devoid of UBO. CONCLUSION: Multivoxel spectroscopic imaging using short-time echo showed spectroscopic abnormalities in the right thalamus of NF1 patients harboring UBO, which were mainly located in the basal ganglia. This finding could reflect the anatomical and functional interactions of these regions.


Assuntos
Gânglios da Base/metabolismo , Gânglios da Base/patologia , Imageamento por Ressonância Magnética , Espectroscopia de Ressonância Magnética/métodos , Neurofibromatose 1/metabolismo , Neurofibromatose 1/patologia , Tálamo/metabolismo , Tálamo/patologia , Adolescente , Biomarcadores/análise , Criança , Pré-Escolar , Feminino , Humanos , Masculino , Prótons , Estatística como Assunto
2.
Dev Neuropsychol ; 34(6): 736-48, 2009.
Artigo em Inglês | MEDLINE | ID: mdl-20183730

RESUMO

Learning disabilities represent the main childhood complication in neurofibromatosis type 1 (NF1). Patients frequently exhibit T2-weighted hyperintensities called unidentified bright objects (UBOs) on brain magnetic resonance imaging (MRI), with unclear relationship to such cognitive disabilities. This study aimed to determine whether thalamo-striatal UBOs correlate with cognitive disturbances. Thirty-seven NF1 children were studied: 24 with UBOs (18 of which were thalamo-striatal UBOs), and 13 without UBOs. NF1 subjects carrying thalamo-striatal UBOs had significantly lower IQs and visuospatial performances than those without UBOs in this location. These results suggest that UBOs may contribute to NF1 cognitive impairments through thalamo-cortical dysfunction.


Assuntos
Transtornos Cognitivos/diagnóstico , Corpo Estriado/patologia , Imageamento por Ressonância Magnética , Neurofibromatose 1/epidemiologia , Tálamo/patologia , Adolescente , Criança , Transtornos Cognitivos/epidemiologia , Feminino , Humanos , Masculino , Percepção Espacial , Percepção Visual
3.
J Clin Ultrasound ; 35(4): 216-20, 2007 May.
Artigo em Inglês | MEDLINE | ID: mdl-17354250

RESUMO

Pentalogy of Cantrell is a rare thoraco-abdominal disruption with a variable association of abdominal wall, diaphragm, pericardium, sternum, and heart defects. Diagnosis is feasible on antenatal sonography. However, because prognosis depends on the extent of the defect, anatomic assessment is crucial for optimal parental counseling and decision-making regarding the outcome of pregnancy. We report the use of 2-dimensional and 3-dimensional sonography in the diagnosis of pentalogy of Cantrell.


Assuntos
Doenças Fetais/diagnóstico por imagem , Ultrassonografia Pré-Natal , Adulto , Ecocardiografia , Feminino , Cardiopatias Congênitas/diagnóstico por imagem , Hérnia Diafragmática/diagnóstico por imagem , Hérnia Umbilical/diagnóstico por imagem , Humanos , Imageamento Tridimensional , Gravidez , Prognóstico , Esterno/anormalidades , Esterno/diagnóstico por imagem , Tetralogia de Fallot/diagnóstico por imagem
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