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1.
Artigo em Inglês | MEDLINE | ID: mdl-36090594

RESUMO

SAPHO (synovitis, acne, pustulosis, hyperostosis, and osteitis) is a rare syndrome mainly characterized by cutaneous and osteoarticular manifestations. The most typical osteoarticular manifestations are localized to the anterior chest wall and include a usually noninfectious osteitis, hyperostosis, and synovitis of the sternoclavicular joints. However, clinical presentation of SAPHO syndrome can be quite heterogeneous. Several clinical and radiological features are shared with other well-defined pathological entities, and clinical signs and symptoms often occur at different timepoints. Mainly due to this complexity and its rarity, there are currently no validated diagnostic criteria for SAPHO syndrome. Inflammation of the soft tissues around the bones and possible nerve compression could contribute to dysphagia, hypophonia, or obstruction of the airways. Neurologic manifestations could therefore be part of this multiorgan involvement. Here, we present a case of SAPHO syndrome with atypical onset symptoms, characterized by left vocal cord paralysis, acute neck pain due to osteolytic atlantoepistrophic lesion, and an unusual cutaneous manifestation, diagnosed as mid-dermal elastolysis. The latest two, to the best of our knowledge, have been here first described in a case of SAPHO syndrome.

2.
Rheumatology (Oxford) ; 60(6): 2678-2687, 2021 06 18.
Artigo em Inglês | MEDLINE | ID: mdl-33507240

RESUMO

OBJECTIVES: To evaluate longitudinal variations in diffusion tensor imaging (DTI) metrics of different white matter (WM) tracts of newly diagnosed SLE patients, and to assess whether DTI changes relate to changes in clinical characteristics over time. METHODS: A total of 17 newly diagnosed SLE patients (19-55 years) were assessed within 24 months from diagnosis with brain MRI (1.5 T Philips Achieva) at baseline, and after at least 12 months. Fractional anisotropy, mean diffusivity (MD), radial diffusivity (RD) and axial diffusivity values were calculated in several normal-appearing WM tracts. Longitudinal variations in DTI metrics were analysed by repeated measures analysis of variance. DTI changes were separately assessed for 21 WM tracts. Associations between longitudinal alterations of DTI metrics and clinical variables (SLEDAI-2K, complement levels, glucocorticoid dosage) were evaluated using adjusted Spearman correlation analysis. RESULTS: Mean MD and RD values from the normal-appearing WM significantly increased over time (P = 0.019 and P = 0.021, respectively). A significant increase in RD (P = 0.005) and MD (P = 0.012) was found in the left posterior limb of the internal capsule; RD significantly increased in the left retro-lenticular part of the internal capsule (P = 0.013), and fractional anisotropy significantly decreased in the left corticospinal tract (P = 0.029). No significant correlation was found between the longitudinal change in DTI metrics and the change in clinical measures. CONCLUSION: Increase in diffusivity, reflecting a compromised WM tissue microstructure, starts in initial phases of the SLE disease course, even in the absence of overt neuropsychiatric (NP) symptoms. These results indicate the importance of monitoring NP involvement in SLE, even shortly after diagnosis.


Assuntos
Imagem de Tensor de Difusão , Lúpus Eritematoso Sistêmico/diagnóstico por imagem , Substância Branca/diagnóstico por imagem , Adulto , Análise de Variância , Anisotropia , Feminino , Humanos , Lúpus Eritematoso Sistêmico/complicações , Lúpus Eritematoso Sistêmico/tratamento farmacológico , Masculino , Pessoa de Meia-Idade , Estudos Prospectivos , Fatores de Tempo , Adulto Jovem
3.
Brain Sci ; 12(1)2021 Dec 31.
Artigo em Inglês | MEDLINE | ID: mdl-35053811

RESUMO

Diffusion-based magnetic resonance imaging (MRI) studies, namely diffusion-weighted imaging (DWI) and diffusion-tensor imaging (DTI), have been performed in the context of systemic lupus erythematosus (SLE), either with or without neuropsychiatric (NP) involvement, to deepen cerebral microstructure alterations. These techniques permit the measurement of the variations in random movement of water molecules in tissues, enabling their microarchitecture analysis. While DWI is recommended as part of the initial MRI assessment of SLE patients suspected for NP involvement, DTI is not routinely part of the instrumental evaluation for clinical purposes, and it has been mainly used for research. DWI and DTI studies revealed less restricted movement of water molecules inside cerebral white matter (WM), expression of a global loss of WM density, occurring in the context of SLE, prevalently, but not exclusively, in case of NP involvement. More advanced studies have combined DTI with other quantitative MRI techniques, to further characterize disease pathogenesis, while brain connectomes analysis revealed structural WM network disruption. In this narrative review, the authors provide a summary of the evidence regarding cerebral microstructure analysis by DWI and DTI studies in SLE, focusing on lessons learned and future research perspectives.

4.
BMC Neurol ; 16: 165, 2016 Sep 08.
Artigo em Inglês | MEDLINE | ID: mdl-27608765

RESUMO

BACKGROUND: Fahr's disease is rare a neurodegenerative idiopathic condition characterized by symmetric and bilateral calcifications of basal ganglia, usually associated with progressive neuropsychiatric dysfunctions and movement disorders. The term "Fahr's syndrome" is used in presence of calcifications secondary to a specific cause, but the variability of etiology, pathogenesis, and clinical picture underlying this condition have raised the question of the real existence of a syndrome. Several classifications based on the etiology, the location of brain calcifications and the clinical presentation have been proposed. Here we describe seven clinical cases of basal ganglia calcifications, in order to search for pathognomonic features and correlations between clinical picture and imaging findings. CASES PRESENTATION: The patients came to our attention for different reasons (most of them for memory/behavior disturbances); all underwent neuro-psychologic evaluation and neuro-imaging. All patients showed variable degrees of deterioration in cognitive function; anxiety and depression were frequent too, and resistant to treatment in all cases. Less frequent, but severe if present, were psychotic symptoms, with different grade of structure and emotional involvement, and always resistant to treatment. We observed only few cases of extrapyramidal disorders related to the disease itself; anyway, mild extrapyramidal syndrome occurred quite frequently after treatment with antipsychotics. CONCLUSION: Based on these findings we discourage the use of the term "Fahr's syndrome", and suggest to refer to Idiopathic or Secondary basal ganglia calcification. Unlike early onset forms (idiopathic or inherited), the clinical presentation of late onset form and Secondary basal ganglia calcification seems to be really heterogeneous. Case-control studies are necessary to determine the actual significance of basal ganglia calcification in the adult population and in the elderly, in cognitive, physical and emotional terms.


Assuntos
Doenças dos Gânglios da Base/patologia , Doenças dos Gânglios da Base/fisiopatologia , Calcinose/patologia , Calcinose/fisiopatologia , Doenças Neurodegenerativas/patologia , Doenças Neurodegenerativas/fisiopatologia , Adulto , Idoso , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Síndrome
5.
PLoS One ; 11(1): e0147910, 2016.
Artigo em Inglês | MEDLINE | ID: mdl-26824672

RESUMO

INTRODUCTION: The capability of CT perfusion (CTP) Alberta Stroke Program Early CT Score (ASPECTS) to predict outcome and identify ischemia severity in acute ischemic stroke (AIS) patients is still questioned. METHODS: 62 patients with AIS were imaged within 8 hours of symptom onset by non-contrast CT, CT angiography and CTP scans at admission and 24 hours. CTP ASPECTS was calculated on the affected hemisphere using cerebral blood flow (CBF), cerebral blood volume (CBV) and mean transit time (MTT) maps by subtracting 1 point for any abnormalities visually detected or measured within multiple cortical circular regions of interest according to previously established thresholds. MTT-CBV ASPECTS was considered as CTP ASPECTS mismatch. Hemorrhagic transformation (HT), recanalization status and reperfusion grade at 24 hours, final infarct volume at 7 days and modified Rankin scale (mRS) at 3 months after onset were recorded. RESULTS: Semi-quantitative and quantitative CTP ASPECTS were highly correlated (p<0.00001). CBF, CBV and MTT ASPECTS were higher in patients with no HT and mRS ≤ 2 and inversely associated with final infarct volume and mRS (p values: from p<0.05 to p<0.00001). CTP ASPECTS mismatch was slightly associated with radiological and clinical outcomes (p values: from p<0.05 to p<0.02) only if evaluated quantitatively. A CBV ASPECTS of 9 was the optimal semi-quantitative value for predicting outcome. CONCLUSIONS: Our findings suggest that visual inspection of CTP ASPECTS recognizes infarct and ischemic absolute values. Semi-quantitative CBV ASPECTS, but not CTP ASPECTS mismatch, represents a strong prognostic indicator, implying that core extent is the main determinant of outcome, irrespective of penumbra size.


Assuntos
Isquemia Encefálica/diagnóstico , Angiografia por Ressonância Magnética , Acidente Vascular Cerebral/diagnóstico , Idoso , Idoso de 80 Anos ou mais , Velocidade do Fluxo Sanguíneo/efeitos dos fármacos , Volume Sanguíneo/efeitos dos fármacos , Isquemia Encefálica/diagnóstico por imagem , Isquemia Encefálica/tratamento farmacológico , Isquemia Encefálica/patologia , Angiografia Cerebral , Circulação Cerebrovascular/efeitos dos fármacos , Cérebro/irrigação sanguínea , Cérebro/efeitos dos fármacos , Cérebro/patologia , Feminino , Fibrinolíticos/uso terapêutico , Humanos , Processamento de Imagem Assistida por Computador , Masculino , Pessoa de Meia-Idade , Estudos Retrospectivos , Acidente Vascular Cerebral/diagnóstico por imagem , Acidente Vascular Cerebral/tratamento farmacológico , Acidente Vascular Cerebral/patologia , Tomografia Computadorizada por Raios X , Resultado do Tratamento
6.
Artigo em Inglês | MEDLINE | ID: mdl-25679608

RESUMO

We theoretically investigate fluctuation relations in a classical incomplete measurement process where only partial information is available. The scenario we consider consists of two coupled single-electron boxes where one or both devices can undergo a nonequilibrium transformation according to a chosen protocol. The entropy production of only one of the two boxes is recorded and fluctuation relations for this quantity are put to a test, showing strong modifications whose nature depends upon the specific case study.

7.
Neuroradiology ; 55(2): 145-56, 2013 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-22987060

RESUMO

INTRODUCTION: Diffusion-weighted imaging (DWI) studies focusing on apparent diffusion coefficient (ADC) abnormalities have provided conflicting results about the nature and fate of perihematomal edema. METHODS: We investigated 35 patients with supratentorial spontaneous intracerebral hemorrhage (SICH) by using DWI scanning obtained at 48 h and 7 days after symptom onset. Regional ADC (rADC) values were measured in three manually outlined regions of interest: (1) the perihematomal hyperintense area, (2) 1 cm of normal appearing brain tissue surrounding the perilesional hyperintense rim, and (3) a mirror area, including the clot and the perihematomal region, located in the contralateral hemisphere. RESULTS: rADC mean levels were lower at 7 days than at 48 h in each ROI (p < 0.00001), showing a progressive normalization of initial vasogenic values. Perihematomal vasogenic rADC values were more frequent (p < 0.00001) at 48 h than at 7 days, whereas perihematomal cytotoxic and normal rADC levels were more represented (p < 0.02 and p < 0.001, respectively) at 7 days than at 48 h. A neurological worsening was more frequent (p < 0.02) in patients with than in those without perihematomal cytotoxic rADC values at 7 days. CONCLUSION: Our findings suggest that the transition from acute to subacute phases after SICH is characterized by a progressive resolution of perihematomal vasogenic edema associated with an increase in cytotoxic ADC values. In the subset of patients with perihematomal cytotoxic rADC levels in subacute stage after bleeding, irreversible damage development seems to be related to poor clinical outcome.


Assuntos
Hemorragia Cerebral/complicações , Hemorragia Cerebral/diagnóstico , Imagem de Difusão por Ressonância Magnética/métodos , Hematoma Subdural Crônico/diagnóstico , Hematoma Subdural Crônico/etiologia , Interpretação de Imagem Assistida por Computador/métodos , Doença Aguda , Idoso , Feminino , Humanos , Masculino , Reprodutibilidade dos Testes , Sensibilidade e Especificidade
8.
Rheumatology (Oxford) ; 49(8): 1540-9, 2010 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-20444860

RESUMO

OBJECTIVES: To perform an observational retrospective cross-sectional case-control study to evaluate prevalence, clinical patterns and outcomes of CNS involvement in a large cohort of primary SS (pSS) patients. METHODS: A total of 424 pSS patients, diagnosed according to the 2002 criteria proposed by the American-European Consensus Group, were checked for CNS involvement after exclusion of secondary causes. Demographic, clinical, seroimmunological data were compared between patients with and without CNS involvement. Neuroimaging data were also analysed. RESULTS: CNS involvement was detected in 25 (5.8%) patients (24 females and 1 male) both at disease onset (52%) and later (48%) with a mean latency after diagnosis of 7 years. Diffuse (40%), focal/multifocal (36%), multiple sclerosis (MS)-like disease (20%) and isolated optic neuritis (4%) were the most common CNS clinical pictures. Disease duration, lung involvement and decreased C(4) were associated with CNS involvement, while articular manifestations were more frequently observed in patients without neurological complications. Most cases had an acute, often recurrent course with spontaneous remission or only mild neurological impairment. CONCLUSIONS: CNS involvement represents a rare but not negligible complication of pSS, which may occur with a bimodal temporal pattern, both at onset and later, prompting attention in the differential diagnosis of apparently isolated neurological syndromes. Lung involvement emerged as the strongest risk factor for CNS involvement with a relative risk of 7.9, along with disease duration and decreased C(4).


Assuntos
Doenças do Sistema Nervoso Central/etiologia , Síndrome de Sjogren/complicações , Adulto , Idoso , Estudos de Casos e Controles , Doenças do Sistema Nervoso Central/patologia , Doenças do Sistema Nervoso Central/fisiopatologia , Estudos de Coortes , Progressão da Doença , Feminino , Humanos , Itália/epidemiologia , Imageamento por Ressonância Magnética , Masculino , Pessoa de Meia-Idade , Análise de Regressão , Estudos Retrospectivos , Síndrome de Sjogren/patologia , Síndrome de Sjogren/fisiopatologia , Fatores de Tempo
9.
Acta Neurochir Suppl ; 106: 283-7, 2010.
Artigo em Inglês | MEDLINE | ID: mdl-19812965

RESUMO

In this study, neoplastic perfusion abnormalities were investigated by computed tomography perfusion (CTP) scanning in 38 patients with solitary intra-axial brain tumors (19 with high grade gliomas, 7 with low grade gliomas and 12 with brain metastasis). Regional cerebral blood flow (rCBF), cerebral blood volume (rCBV), mean transit time (rMTT) and permeability surface flow (rPSF) levels were measured in two different regions of interest: (1) enhancing or non-enhancing tumor tissue and (2) a mirror area of apparently normal brain tissue located in the contralateral hemisphere. rCBF mean levels were greater in tumoral tissue than in the contralateral area for high-grade gliomas (p < 0.02). rCBV and rPSF mean values were higher in tumoral tissue than in the contralateral area for high-grade gliomas (p < 0.01 and p < 0.05, respectively) and metastasis (p < 0.05 and p < 0.001, respectively). rCBV mean values of tumoral tissue were greater in high-grade than in low-grade gliomas (p < 0.05). rPSF mean levels of tumoral tissue were higher in metastasis than in low-grade gliomas (p < 0.02). These findings indicate that multi-parametric CTP mapping may contribute to differential diagnosis of solitary intra-axial brain tumors.


Assuntos
Neoplasias Encefálicas/diagnóstico por imagem , Circulação Cerebrovascular/fisiologia , Glioma/diagnóstico por imagem , Tomografia Computadorizada por Raios X/métodos , Idoso , Velocidade do Fluxo Sanguíneo/fisiologia , Neoplasias Encefálicas/classificação , Feminino , Lateralidade Funcional , Glioma/classificação , Humanos , Masculino , Pessoa de Meia-Idade , Perfusão/métodos , Estatísticas não Paramétricas
10.
J Radiol Case Rep ; 3(3): 17-20, 2009.
Artigo em Inglês | MEDLINE | ID: mdl-22470648

RESUMO

This report describes a 60-year-old woman with intensive back pain due to metastatic vertebral body collapse, who underwent percutaneous vertebroplasty. Subsequently, the patient developed metastatic lesion extrusion into the spinal canal because of pressure of the cement, with compression of the left anterolateral spinal cord. During percutaneous vertebroplasty procedure in patient with malignant tumors, the complication rate increases owing to the risk of leakage of cement resulting from the vertebral body destruction, but as also seen in our case, for the extrusion of the neoplastic tissue and increase of the pressure in the vertebral body due to the introduction of the cement.

11.
Blood Coagul Fibrinolysis ; 19(7): 721-3, 2008 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-18832917

RESUMO

Thrombosis of the cerebral veins or sinuses is a rare cerebrovascular disorder, which seldom represents a complication of acute promyelocytic leukemia. To the best of our knowledge, it never occurred during treatment with all-trans retinoic acid. We report a case of a 35-year-old woman affected by acute promyelocytic leukemia, who developed massive thrombosis of the cerebral sinuses and veins when she was in complete morphological and molecular remission after all-trans retinoic acid and idarubicin treatment. Anticoagulant therapy contributed to progressive dissolution of the thrombosis as documented by magnetic resonance imaging with complete disappearance of neurological signs without sequelae.


Assuntos
Hemorragia Cerebral/induzido quimicamente , Leucemia Promielocítica Aguda/tratamento farmacológico , Trombose dos Seios Intracranianos/induzido quimicamente , Trombose/induzido quimicamente , Tretinoína/efeitos adversos , Adulto , Protocolos de Quimioterapia Combinada Antineoplásica/efeitos adversos , Protocolos de Quimioterapia Combinada Antineoplásica/uso terapêutico , Feminino , Humanos , Idarubicina/administração & dosagem , Trombose/prevenção & controle , Tretinoína/administração & dosagem
12.
Neuroradiology ; 50(8): 729-40, 2008 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-18504564

RESUMO

INTRODUCTION: We sought to quantify perfusion changes associated to acute spontaneous intracerebral hemorrhage (SICH) by means of computed tomography perfusion (CTP) imaging. MATERIALS AND METHODS: We studied 89 patients with supratentorial SICH at admission CT by using CTP scanning obtained within 24 h after symptom onset. Regional cerebral blood flow (rCBF), cerebral blood volume (rCBV) and mean transit time (rMTT) levels were measured in four different regions of interest manually outlined on CT scan: (1) hemorrhagic core; (2) perihematomal low-density area; (3) 1 cm rim of normal-appearing brain tissue surrounding the perilesional area; and (4) a mirrored area, including the clot and the perihematomal region, located in the non-lesioned contralateral hemisphere. RESULTS: rCBF, rCBV, and rMTT mean levels showed a centrifugal distribution with a gradual increase from the core to the periphery (p < 0.0001). Perfusion absolute values were indicative of ischemia in hemorrhagic core, oligemia in perihematomal area, and hyperemia in normal-appearing and contralateral areas. Perihematomal rCBF and rCBV mean levels were higher in small (< or = 20 ml) than in large (>20 ml) hematomas (p < 0.01 and p < 0.02, respectively). CONCLUSION: Multi-parametric CTP mapping of acute SICH indicates that perfusion values show a progressive improvement from the core to the periphery. In the first 24 h, perihemorrhagic region was hypoperfused with CTP values which were not suggestive of ischemic penumbra destined to survive but more likely indicative of edema formation. These findings also argue for a potential influence of early amounts of bleeding on perihematomal hemodynamic abnormalities.


Assuntos
Hemorragia Cerebral/diagnóstico por imagem , Hemorragia Cerebral/fisiopatologia , Circulação Cerebrovascular/fisiologia , Tomografia Computadorizada por Raios X/métodos , Idoso , Velocidade do Fluxo Sanguíneo/fisiologia , Volume Sanguíneo , Edema Encefálico/diagnóstico por imagem , Edema Encefálico/etiologia , Edema Encefálico/fisiopatologia , Hemorragia Cerebral/complicações , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Estudos Prospectivos , Reprodutibilidade dos Testes
13.
Auris Nasus Larynx ; 34(2): 229-32, 2007 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-17064866

RESUMO

Merkel cells carcinoma (MCC) is an uncommon skin lesion, considered a malignancy of the neuroendocrine system, which is found mainly in elderly people. Its incidence is highly correlated with sun exposure or immunodeficiency syndromes. MCC is often an aggressive tumour with high tendency for local recurrence, lymph node involvement and distant metastasis. To our best knowledge 20 cases originated from the auricle have been described, 2 of them arising from external ear canal. The authors report a case of the ear canal characterized by two others synchronous tumours and the occurrence of a malignant high grade lymphoma, in which contribute of the pathologist was essential for a critical review. MCC diagnosis is not always easy for its pathological and clinical features and it should always be considered in presence of lymphoma. A multidisciplinary approach is basic.


Assuntos
Carcinoma de Célula de Merkel/diagnóstico , Neoplasias da Orelha/diagnóstico , Orelha Externa , Idoso , Protocolos de Quimioterapia Combinada Antineoplásica/uso terapêutico , Biomarcadores Tumorais/análise , Biópsia por Agulha Fina , Carcinoma de Célula de Merkel/tratamento farmacológico , Carcinoma de Célula de Merkel/patologia , Neoplasias da Orelha/tratamento farmacológico , Neoplasias da Orelha/patologia , Orelha Externa/patologia , Evolução Fatal , Neoplasias de Cabeça e Pescoço/diagnóstico , Neoplasias de Cabeça e Pescoço/terapia , Humanos , Queratinas/análise , Linfoma Folicular/diagnóstico , Linfoma Folicular/terapia , Imageamento por Ressonância Magnética , Masculino , Segunda Neoplasia Primária/diagnóstico , Segunda Neoplasia Primária/tratamento farmacológico , Segunda Neoplasia Primária/patologia , Glândula Parótida/patologia , Neoplasias Cutâneas/diagnóstico , Neoplasias Cutâneas/terapia
14.
Rheumatol Int ; 26(12): 1113-9, 2006 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-16794844

RESUMO

The aim of this study was to evaluate neurological involvement in a series of 110 North Italian patients with Behçet disease (BD), a multisystemic vasculitis of unknown origin, followed up for a period of 5 years. During this time, 27 (24.5%) patients with neuro-BD were identified. Twenty out of 27 showed at least one acute attack in their clinical course. In 14 of them, a neurological evaluation was carried out during the attack. The other 13 patients were evaluated during a remission phase. The onset of neuro-BD was usually characterized by an acute attack with motor symptoms (66.6%) and behavioural/cognitive changes (47.6%), while headache was more frequent in the remission phase (76.9%). On magnetic resonance imaging, large brain-stem/diencephalon lesions were usually seen during the attack. In the remission phase, they were often located in the white-matter. Aspecific cerebrospinal fluid abnormalities were usually seen during the attacks. Cerebrospinal fluid analysis together with radiological and clinical features seems to be useful for the differential diagnosis in these patients.


Assuntos
Síndrome de Behçet/diagnóstico , Doenças do Sistema Nervoso Central/diagnóstico , Adolescente , Adulto , Síndrome de Behçet/complicações , Síndrome de Behçet/patologia , Encéfalo/patologia , Doenças do Sistema Nervoso Central/complicações , Doenças do Sistema Nervoso Central/patologia , Líquido Cefalorraquidiano/química , Criança , Pré-Escolar , Diagnóstico Diferencial , Feminino , Humanos , Itália , Imageamento por Ressonância Magnética , Masculino , Medula Espinal/patologia
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