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1.
Dermatol Reports ; 13(3): 9240, 2021 Nov 17.
Artigo em Inglês | MEDLINE | ID: mdl-35003569

RESUMO

The management of difficult-to-treat periocular basal cell carcinoma (BCC) becomes very challenging in cases of delayed diagnosis, leading to the development of locally advanced BCC. The aim of this study was to evaluate the outcomes of Hedgehog pathway inhibitors (vismodegib and sonidegib) treatment in patients affected by periocular locally advanced BCC. We focused on the common adverse events and their correlation with the administration schedule, to determine a management protocol specific for the periocular area. This observational prospective study included a single-center case series with patients who were histologically confirmed to have periocular or orbital locally advanced BCC, treated with Hedgehog pathway inhibitors. All patients benefitted in terms of regression or stabilization of the neoplasm. In the first months of treatment, the HPIs were well tolerated, and the first important side effects appeared after about 5 months of continuous use of the drug. These data could lead to a new type of therapeutic scheme where neoadjuvant therapy could be followed by pulse therapy as an adjuvant to surgery.

2.
Radiol Med ; 125(12): 1311-1321, 2020 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-32367321

RESUMO

In the context of the increasing spread of cardiac active implantable heart devices (CIEDs) in the population and of the wide diagnostic/therapeutic utility of magnetic resonance (MRI) examinations, the goal of this paper is to provide the experience of the Santa Maria Nuova Hospital of the USL Tuscany Center in Florence and to report an organizational proposal to perform, in the hospital settings, MRI examinations on patients carrying CIED. This report is intended to show the operational choices of a Radiology Department which organizes this activity in accordance with the new Italian regulatory framework in the field of safety of MR sites (Ministero della Salute in Decreto Ministeriale 10 agosto 2018 Determinazione degli standard di sicurezza e impiego per le apparecchiature a risonanza magnetica, 2018).


Assuntos
Abdome , Desfibriladores Implantáveis , Eletrodos Implantados , Imageamento por Ressonância Magnética/métodos , Marca-Passo Artificial , Abdome/diagnóstico por imagem , Adulto , Idoso , Idoso de 80 Anos ou mais , Articulação do Tornozelo/diagnóstico por imagem , Encéfalo/diagnóstico por imagem , Segurança de Equipamentos , Europa (Continente) , Humanos , Consentimento Livre e Esclarecido , Itália , Legislação Médica , Imageamento por Ressonância Magnética/estatística & dados numéricos , Pessoa de Meia-Idade , Modelos Organizacionais , Pelve/diagnóstico por imagem , Medição de Risco , Coluna Vertebral/diagnóstico por imagem , Coxa da Perna/diagnóstico por imagem
3.
J Magn Reson Imaging ; 30(1): 11-7, 2009 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-19557841

RESUMO

PURPOSE: To investigate the impact of cerebrospinal fluid (CSF) contamination on metabolite evaluation in the superior cerebellar vermis with single-voxel (1)H-MRS in normal subjects and patients with degenerative ataxias. MATERIALS AND METHODS: Twenty-nine healthy volunteers and 38 patients with degenerative ataxias and cerebellar atrophy were examined on a 1.5 Tesla scanner. Proton spectra of a volume of interest placed in the superior vermis were acquired using a four TE PRESS technique. We calculated N-acetyl aspartate (NAA)/creatine (Cr), choline (Cho)/Cr, and NAA/Cho ratios, T(2) relaxation times and concentrations of the same metabolites using the external phantom method. Finally, concentrations were corrected taking into account the proportion of nervous tissue and CSF, that was determined as Volume Fraction (VF). RESULTS: In healthy subjects, a significant difference was observed between metabolite concentrations with and without correction for VF. As compared to controls, patients with ataxias showed significantly reduced NAA/Cr and NAA concentrations, while only corrected Cr concentration was significantly increased. The latter showed an inverse correlation with VF. CONCLUSION: CSF contamination has a not negligible effect on the estimation of brain metabolites. The increase of Cr concentration in patients with cerebellar atrophy presumably reflects the substitutive gliosis which takes place along with loss of neurons.


Assuntos
Encéfalo/metabolismo , Ataxia Cerebelar/líquido cefalorraquidiano , Ataxia Cerebelar/metabolismo , Espectroscopia de Ressonância Magnética/métodos , Adulto , Análise de Variância , Ácido Aspártico/análogos & derivados , Ácido Aspártico/líquido cefalorraquidiano , Ácido Aspártico/metabolismo , Encéfalo/patologia , Ataxia Cerebelar/patologia , Cerebelo/metabolismo , Cerebelo/patologia , Colina/líquido cefalorraquidiano , Colina/metabolismo , Creatina/líquido cefalorraquidiano , Creatina/metabolismo , Feminino , Humanos , Processamento de Imagem Assistida por Computador/métodos , Masculino , Pessoa de Meia-Idade , Variações Dependentes do Observador , Tamanho do Órgão , Imagens de Fantasmas , Reprodutibilidade dos Testes
4.
J Comput Assist Tomogr ; 31(4): 574-80, 2007.
Artigo em Inglês | MEDLINE | ID: mdl-17882035

RESUMO

OBJECTIVE: To concurrently investigate with magnetic resonance (MR) the brain activation and regional brain atrophy in patients with Huntington disease (HD). METHODS: Nine symptomatic HD patients and 11 healthy subjects underwent an MR study including functional MR acquisition during finger tapping of the right hand and high-resolution T1-weighted images. Functional and structural data were analyzed using Statistical Parametric Mapping 2 software. RESULTS: As compared with control subjects, HD patients showed decreased activation in the left caudate nucleus and medial frontal and anterior cingulate gyri and increased activation in the right supplementary motor area and supramarginal gyrus and left intraparietal sulcus. The pattern of atrophy included thinning of the gray matter (GM) in the insula, inferior frontal gyrus, caudate, lentiform nucleus, and thalamus, bilaterally, in the left middle frontal, middle occipital, and middle temporal gyri, and of periventricular, subinsular, right temporal lobe, and left internal capsule white matter. Only the decreased activation in the caudate nucleus correlated topographically with the caudate GM loss. CONCLUSION: The cortical areas of functional changes do not correspond to those of GM atrophy in patients with HD and are likely to reflect decreased output of the motor basal ganglia-thalamo-cortical circuit and compensatory recruitment of accessory motor pathways.


Assuntos
Encéfalo , Doença de Huntington/diagnóstico , Imageamento por Ressonância Magnética/métodos , Atrofia , Encéfalo/patologia , Encéfalo/fisiopatologia , Córtex Cerebral/patologia , Córtex Cerebral/fisiopatologia , Feminino , Humanos , Masculino , Pessoa de Meia-Idade
5.
J Neurol Neurosurg Psychiatry ; 78(9): 1001-4, 2007 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-17702784

RESUMO

OBJECTIVE: To explore with neuroimaging techniques the anatomical and functional correlates of sporadic hyperekplexia. METHODS: Two elderly women with sporadic hyperekplexia underwent neurophysiological assessment, MRI of the brain and proton magnetic resonance spectroscopy (1H-MRS) of the brainstem and frontal lobes. Regional cerebral blood flow was investigated with single photon emission tomography (SPECT) during evoked startles and at rest. RESULTS: Both patients showed excessively large and non-habituating startle responses. In both patients, MRI showed impingement of the brainstem by the vertebrobasilar artery, lack of frontal or brainstem abnormalities on 1H-MRS and hyperperfusion in the dorsal pons and cingulate cortex, and superior frontal gyrus at SPECT during evoked startles. CONCLUSIONS: In our patients with hyperekplexia, the vertebrobasilar arteries were found to impinge on the brainstem. Neurophysiological findings and neurofunctional imaging of evoked startles indicated a pontine origin of the movement disorder modulated by activation in cortical, especially frontal, areas. The neurofunctional correlates of evoked startles in human sporadic hyperekplexia are similar to those observed for the startle circuit in animals.


Assuntos
Encéfalo/patologia , Encéfalo/fisiopatologia , Circulação Cerebrovascular/fisiologia , Reflexo Anormal/fisiologia , Reflexo de Sobressalto/fisiologia , Idoso , Encéfalo/diagnóstico por imagem , Feminino , Humanos , Cintilografia
6.
Radiology ; 241(2): 492-500, 2006 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-17057070

RESUMO

PURPOSE: To prospectively compare brain magnetic resonance (MR) imaging and hydrogen 1 (1H) MR spectroscopy findings and to use functional MR imaging to explore the patterns of brain activation in men and women with Fabry disease (FD). MATERIALS AND METHODS: Eight men and eight women with FD (mean age, 38.8 years +/- 13.9 [standard deviation]) with absent or mild neurologic deficit and 16 healthy control subjects (eight men and eight women; mean age, 42.7 years +/- 15.3) gave informed consent to participate in the study, which was approved by the local ethical committee. Patients and control subjects underwent MR imaging, 1H MR spectroscopy of the frontal cortex and subcortical white matter, and functional MR imaging during repetitive flexion-extension of the last four fingers of the right hand. Extent of cerebral white matter damage was rated on fluid-attenuated inversion recovery MR images by using a visual score. Areas of activation were identified by using statistical parametric mapping software and the adoption of a height threshold of P < .001 (uncorrected) and an extent threshold of P < .05 (corrected). RESULTS: Men and women with FD showed a similar distribution of cerebral white matter changes, lacunar and cortical infarcts, small hemorrhages, and vertebrobasilar dolichoectasia. No significant (P > .05) difference was observed between patients with FD and control subjects for concentration of N-acetylaspartate, creatine, and choline. During the motor task, patients showed recruitment of additional cortical areas in comparison with control subjects. Increased activation of the contralateral sensorimotor area correlated (P = .002) with extent of white matter damage. CONCLUSION: Subcortical ischemic changes in men and women with FD are similar and are associated with increased recruitment of the sensorimotor network during a simple motor task, which might limit the functional effect of the white matter small-vessel disease.


Assuntos
Encéfalo/patologia , Doença de Fabry/patologia , Imageamento por Ressonância Magnética/métodos , Espectroscopia de Ressonância Magnética/métodos , Adulto , Encéfalo/metabolismo , Estudos de Casos e Controles , Doença de Fabry/metabolismo , Feminino , Humanos , Modelos Lineares , Masculino , Estudos Prospectivos , Estatísticas não Paramétricas
7.
Neuroimage ; 22(2): 698-705, 2004 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-15193598

RESUMO

Analysis of the apparent diffusion coefficient (ADC) maps derived from diffusion-weighted MR imaging is emerging as a reproducible, sensitive, and quantitative tool to evaluate brain damage in diseases of the white and gray matter. To explore the potentials of ADC maps analysis in degenerative ataxias, we examined 28 patients and 26 age-matched controls with T1, T2, and diffusion (b values 0-1000 along the three main body axes)-weighted MR images. Twenty-four patients had inherited genetically proven diseases including spinocerebellar ataxia type 1 (SCA1) (n = 9), spinocerebellar ataxia type 2 (SCA2) (n = 8), and Friedreich's ataxia (FA) (n = 7), whereas four patients had sporadic adult onset pure cerebellar ataxia (three idiopathic, one gluten intolerance). Area and linear measurements of the CNS structures contained in the posterior cranial fossa (PCF) preliminary enabled classification of the patients in the three morphological categories reflecting the gross pathology findings, namely olivopontocerebellar atrophy (OPCA) (n = 10: six SCA2 and four SCA1), spinal atrophy (SA) (n = 7: all FA), and cortical cerebellar atrophy (CCA) (n = 4: three idiopathic and one gluten intolerance). Seven patients with SCA1 (n = 5) or SCA2 (n = 2) had morphologic changes reminiscent of OPCA, but their values were still in the lower normal range and were classified as undefined. Mean diffusivity (D) maps of the entire brain were generated and D was measured with regions of interest (ROI) in the medulla, pons, middle cerebellar peduncles, and the peridentate white matter. Moreover, after exclusion of the skull with manual segmentation and of the CSF with application of a threshold value, histograms were obtained for D of the brainstem and cerebellum and for D of the cerebral hemispheres. As compared to controls, a (P < 0.001) increase of D was observed in the medulla, middle cerebellar peduncles, and peridentate white matter in OPCA and undefined patients groups who had also significantly increased values of the 25th and 50th percentiles in the brainstem and cerebellum D histogram. In CCA (P = 0.01), an increase of the 25th and 50th percentile of the D value was observed in the brainstem and cerebellum histograms. The SA group showed (P < 0.001) an increased D in the medulla only. A correlation between clinical severity as assessed with the Inherited Ataxias Clinical Rating Scale (IACRS) and the 50th percentile of the D value in the brainstem and cerebellum histogram (r = 0.69) was observed in patients with SCA1 or SCA2. Diffusion MR imaging reveals variable patterns of increase of D in the brainstem, cerebellum, and cerebral hemispheres in degenerative ataxias that match the known distribution of the neuropathological changes.


Assuntos
Mapeamento Encefálico/métodos , Tronco Encefálico/patologia , Cerebelo/patologia , Degeneração Neural/patologia , Ataxias Espinocerebelares/patologia , Adulto , Progressão da Doença , Feminino , Humanos , Imageamento por Ressonância Magnética/métodos , Masculino , Pessoa de Meia-Idade
8.
Magn Reson Imaging ; 20(8): 619-22, 2002 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-12467871

RESUMO

Single voxel proton MR spectroscopy ((1)H-MRS) of the vermis was obtained in two patients with cerebellitis. In the acute phase (1)H-MRS revealed low N-acetyl-aspartate (NAA)/creatine (Cr) and NAA/choline (Cho) and normal Cho/Cr ratios. Decrease of the concentration of NAA was confirmed by quantitative analysis in one patient. The NAA/Cr and NAA/Cho ratios and NAA concentration were increased in (1)H-MRS examinations obtained 10 and 24 months after the acute episode. (1)H-MRS demonstrates reversible metabolite changes in cerebellitis.


Assuntos
Ácido Aspártico/análogos & derivados , Doenças Cerebelares/diagnóstico , Doenças Cerebelares/metabolismo , Espectroscopia de Ressonância Magnética , Reação de Fase Aguda/metabolismo , Adulto , Ácido Aspártico/metabolismo , Doenças Cerebelares/patologia , Colina/metabolismo , Creatina/metabolismo , Humanos , Masculino , Prótons
9.
J Magn Reson Imaging ; 16(5): 532-7, 2002 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-12412029

RESUMO

PURPOSE: To evaluate the feasibility of single voxel 1H-MRS of the CNS structures contained in the posterior cranial fossa and to determine the distribution of the normal metabolite ratios, concentrations, and T2 relaxation times in the midbrain, pons, medulla, dentate nucleus and cerebellar vermis. MATERIALS AND METHODS: A total of 147 single voxel 1H-MR spectra with a point-resolved proton spectroscopy sequence (PRESS) sequence and echo time (TE) of 136 or 272 msec were obtained in the midbrain, pons, medulla, dentate, and vermis of 31 healthy volunteers. In seven additional patients; the concentrations and T2 relaxation times of metabolites were obtained in the same locations (except the medulla) with an external phantom calibration method and a four TE PRESS technique. RESULTS: Ten (27%) of 36 spectra acquired in the medulla were of poor quality. A similar ranking of the N-acetyl aspartate (NAA)/creatine (Cr) ratio and choline(Cho)/Cr ratios in the five locations for the two TEs was observed, with the highest values in the pons (mean NAA/Cr = 4.16 +/- 0.6 and Cho/Cr =2.66 +/- 0.6 at TE 272) and the lowest values in the dentate and vermis (mean NAA/Cr = 1.66 +/- 0.2 and Cho/Cr = 1.20 +/- 0.2 at TE 272). The analysis of variance showed significant regional differences of the NAA and Cr concentrations, which had the highest values in the dentate. Non-significant regional differences were observed for the concentration of Cho and for the T2 of the metabolites. CONCLUSION: With the exception of the medulla, single voxel 1H-MRS enables an in vivo biochemical analysis of the CNS structures contained in the posterior cranial fossa. Regional differences in the metabolite ratios and concentrations must be considered when employing 1H-MRS for evaluation of diseases of the brainstem and cerebellum.


Assuntos
Ácido Aspártico/análogos & derivados , Tronco Encefálico/química , Cerebelo/química , Espectroscopia de Ressonância Magnética , Adulto , Idoso , Ácido Aspártico/análise , Colina/análise , Creatina/análise , Feminino , Humanos , Espectroscopia de Ressonância Magnética/métodos , Masculino , Bulbo/química , Mesencéfalo/química , Pessoa de Meia-Idade , Ponte/química , Valores de Referência , Reprodutibilidade dos Testes
10.
AJNR Am J Neuroradiol ; 23(10): 1803-6, 2002.
Artigo em Inglês | MEDLINE | ID: mdl-12427642

RESUMO

Two patients with acute thiamine deficiency were examined with thalamic single-voxel proton MR spectroscopy. T2-weighted images exhibited increased signal intensity. N-acetylaspartate (NAA)/creatine (Cr) ratios were low without detectable lactate. Owing to substantially decreased choline (Cho) T2, the Cho/Cr ratio was not decreased. After thiamine therapy, the NAA/Cr ratio increased, paralleling clinical improvement and reduction in the areas of signal-intensity changes.


Assuntos
Espectroscopia de Ressonância Magnética , Prótons , Encefalopatia de Wernicke/diagnóstico , Humanos , Masculino , Pessoa de Meia-Idade , Deficiência de Tiamina/complicações , Deficiência de Tiamina/diagnóstico , Encefalopatia de Wernicke/etiologia
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