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1.
Fortschr Neurol Psychiatr ; 88(2): 118-119, 2020 Feb.
Artigo em Alemão | MEDLINE | ID: mdl-32102103
2.
PLoS One ; 9(11): e112535, 2014.
Artigo em Inglês | MEDLINE | ID: mdl-25383553

RESUMO

BACKGROUND: Data on survival with mucosal melanoma and on prognostic factors of are scarce. It is still unclear if the disease course allows for mucosal melanoma to be treated as primary cutaneous melanoma or if differences in overall survival patterns require adapted therapeutic approaches. Furthermore, this investigation is the first to present 10-year survival rates for mucosal melanomas of different anatomical localizations. METHODOLOGY: 116 cases from Sep 10 1984 until Feb 15 2011 retrieved from the Comprehensive Cancer Center and of the Central Register of the German Dermatologic Society databases in Tübingen were included in our analysis. We recorded anatomical location and tumor thickness, and estimated overall survival at 2, 5 and 10 years and the mean overall survival time. Survival times were analyzed with the Kaplan-Meier method. The log-rank test was used to compare survival times by localizations and by T-stages. PRINCIPAL FINDINGS: We found a median overall survival time of 80.9 months, with an overall 2-year survival of 71.7%, 5-year survival of 55.8% and 10-year survival of 38.3%. The 10-year survival rates for patients with T1, T2, T3 or T4 stage tumors were 100.0%, 77.9%, 66.3% and 10.6% respectively. 10-year survival of patients with melanomas of the vulva was 64.5% in comparison to 22.3% of patients with non-vulva mucosal melanomas. CONCLUSION: Survival times differed significantly between patients with melanomas of the vulva compared to the rest (p = 0.0006). It also depends on T-stage at the time of diagnosis (p < 0.0001).


Assuntos
Melanoma/mortalidade , Melanoma/patologia , Mucosa/patologia , Adulto , Idoso , Idoso de 80 Anos ou mais , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Prognóstico , Análise de Sobrevida , Taxa de Sobrevida , Adulto Jovem
3.
J Comput Assist Tomogr ; 38(1): 123-30, 2014.
Artigo em Inglês | MEDLINE | ID: mdl-24378894

RESUMO

OBJECTIVE: The aim of this study was to search for chemotherapy-induced perfusion changes of diffuse large B-cell lymphoma, follicular lymphoma, and Hodgkin lymphoma at midtreatment versus baseline volume perfusion computed tomography (VPCT). METHODS: Forty-five consecutive patients with untreated diffuse large B-cell lymphoma, follicular lymphoma, and Hodgkin lymphoma received VPCT examinations of the tumor bulk at baseline and during chemotherapy (midtreatment). Blood flow (BF), blood volume (BV), and transit constant (K-trans) were determined. Treatment response was categorized according to the Cheson criteria into complete or partial remission and stable or relapsed/progressive disease. RESULTS: Midtreatment follow-up showed a reduction in BF, BV, and K-trans in all lymphoma subtypes compared with baseline. The reduction in BV was less pronounced in larger tumors. Notably, BF, BV, and K-trans decreased in the responders (complete remission/partial remission) when compared with the nonresponders (stable or relapsed/progressive disease). Less than 10% reduction in BF was shown to be the best VPCT criterion for the identification of nonresponse. CONCLUSIONS: Chemotherapy-induced perfusion changes in responders are recognizable at midtreatment VPCT.


Assuntos
Tomografia Computadorizada de Feixe Cônico/métodos , Linfoma/diagnóstico por imagem , Linfoma/tratamento farmacológico , Adulto , Idoso , Idoso de 80 Anos ou mais , Velocidade do Fluxo Sanguíneo , Volume Sanguíneo , Meios de Contraste , Feminino , Humanos , Iohexol/análogos & derivados , Masculino , Pessoa de Meia-Idade , Prognóstico , Estudos Prospectivos , Resultado do Tratamento
4.
Korean J Radiol ; 14(6): 951-9, 2013.
Artigo em Inglês | MEDLINE | ID: mdl-24265572

RESUMO

OBJECTIVE: Suspicious incidental gastrointestinal FDG uptake during positron-emission tomography/computed tomography (PET/CT) examinations can be caused by different diseases, including malignancies. However, differentiation with PET alone is difficult. The aim of this study was to investigate the potential of PET alone, contrast-enhanced CT (ceCT), and low-dose CT (ldCT) in routine PET/CT protocols for differentiation of incidental gastrointestinal lesions. MATERIALS AND METHODS: Sixty patients with incidental gastrointestinal lesions who underwent a routine PET/CT protocol with ldCT and ceCT were retrospectively analysed. The PET lesions were evaluated regarding their FDG uptake patterns and the standard uptake value. The anatomical correlates in both CT protocols were compared in regard to the correct lesion classification with the reference standard endoscopy. RESULTS: Sixty-two lesions were found in 60 patients (17 malignant, 10 premalignant, 5 benign, 13 inflammatory, 17 physiological). The differentiation of the FDG uptake patterns did not enable reliable lesion classification. The positive predictive value for pathology was 0.81 for ceCT in PET/CT and 0.70 for ldCT. Malignancies were detected in 100% of the patients by ceCT vs. 29.4% by ldCT. The false negative rate of ceCT for all pathologies was 31.1%, vs. 68.9% for ldCT. False positive results (17/62) could not be excluded sufficiently by either CT protocol. CONCLUSION: PET/ceCT protocols provide additional benefit especially in detecting gastrointestinal malignancies as a cause of suspicious incidental gastrointestinal FDG uptake. However, since follow-up endoscopy cannot be forgone due to the considerable false negative rate even with ceCT, the addition of ceCT to a routine PET/ldCT protocol cannot be recommended for this purpose.


Assuntos
Fluordesoxiglucose F18 , Gastroenteropatias/diagnóstico , Trato Gastrointestinal/metabolismo , Tomografia por Emissão de Pósitrons/métodos , Tomografia Computadorizada por Raios X/métodos , Adulto , Idoso , Idoso de 80 Anos ou mais , Meios de Contraste/farmacocinética , Feminino , Fluordesoxiglucose F18/farmacocinética , Seguimentos , Gastroenteropatias/metabolismo , Trato Gastrointestinal/diagnóstico por imagem , Humanos , Masculino , Pessoa de Meia-Idade , Reprodutibilidade dos Testes , Estudos Retrospectivos
5.
AJR Am J Roentgenol ; 200(4): W376-82, 2013 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-23521481

RESUMO

OBJECTIVE: The purpose of this article is to explore the role of MRI in monitoring musculoskeletal involvement in patients with morphea who are undergoing treatment with methotrexate and prednisolone. SUBJECTS AND METHODS: Twenty-two consecutive patients (six men and 16 women; median age, 52 years) with systemic scleroderma and deep morphea prospectively underwent whole-body MRI twice, before treatment (time 1) and during follow-up after 6-12 months (time 2). Images were evaluated for abnormal signal intensity or thickening of sub-cutaneous fatty tissue septa, muscular fasciae, intramuscular perifascial septa, muscle signal intensity, and articular or tendon sheath synovial abnormalities on STIR and gadolinium-enhanced scans. For clinical assessment, the localized scleroderma (morphea) severity index and a 0-6 pain score were applied. RESULTS: From a clinical point of view, none of our patients had progression of the disease, 12 patients were responders (defined as an improvement of localized scleroderma severity index and pain score ≥ 50%), and 10 patients had stable disease. Among responders, the number of patients with subcutaneous septal thickening (time 1, n = 9; time 2, n = 2), fascial enhancement (time 1, n = 8; time 2, n = 3), and articular synovitis (time 1, n = 5; time 2, n = 1) decreased more than in the stable disease group (subcutaneous septal thickening: time 1, n = 9; time 2, n = 8; fascial enhancement: time 1, n = 5; time 2, n = 5; articular synovitis: time 1, n = 8; time 2, n = 6). Subcutaneous thickening, fascial thickening, and fascial enhancement were scored significantly lower at follow-up MRI in responders. CONCLUSION: MRI findings were sensitive to changes in musculoskeletal manifestations in patients with deep morphea undergoing systemic treatment with methotrexate and prednisolone. Thus, MRI can be recommended as an additional tool for response monitoring.


Assuntos
Fármacos Dermatológicos/uso terapêutico , Glucocorticoides/uso terapêutico , Imageamento por Ressonância Magnética , Metotrexato/uso terapêutico , Prednisolona/uso terapêutico , Esclerodermia Localizada/tratamento farmacológico , Adulto , Meios de Contraste , Feminino , Gadolínio DTPA , Humanos , Masculino , Pessoa de Meia-Idade , Estudos Prospectivos , Esclerodermia Localizada/patologia , Estatísticas não Paramétricas , Resultado do Tratamento
6.
Artigo em Inglês | WPRIM (Pacífico Ocidental) | ID: wpr-184182

RESUMO

OBJECTIVE: Suspicious incidental gastrointestinal FDG uptake during positron-emission tomography/computed tomography (PET/CT) examinations can be caused by different diseases, including malignancies. However, differentiation with PET alone is difficult. The aim of this study was to investigate the potential of PET alone, contrast-enhanced CT (ceCT), and low-dose CT (ldCT) in routine PET/CT protocols for differentiation of incidental gastrointestinal lesions. MATERIALS AND METHODS: Sixty patients with incidental gastrointestinal lesions who underwent a routine PET/CT protocol with ldCT and ceCT were retrospectively analysed. The PET lesions were evaluated regarding their FDG uptake patterns and the standard uptake value. The anatomical correlates in both CT protocols were compared in regard to the correct lesion classification with the reference standard endoscopy. RESULTS: Sixty-two lesions were found in 60 patients (17 malignant, 10 premalignant, 5 benign, 13 inflammatory, 17 physiological). The differentiation of the FDG uptake patterns did not enable reliable lesion classification. The positive predictive value for pathology was 0.81 for ceCT in PET/CT and 0.70 for ldCT. Malignancies were detected in 100% of the patients by ceCT vs. 29.4% by ldCT. The false negative rate of ceCT for all pathologies was 31.1%, vs. 68.9% for ldCT. False positive results (17/62) could not be excluded sufficiently by either CT protocol. CONCLUSION: PET/ceCT protocols provide additional benefit especially in detecting gastrointestinal malignancies as a cause of suspicious incidental gastrointestinal FDG uptake. However, since follow-up endoscopy cannot be forgone due to the considerable false negative rate even with ceCT, the addition of ceCT to a routine PET/ldCT protocol cannot be recommended for this purpose.


Assuntos
Adulto , Idoso , Idoso de 80 Anos ou mais , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Meios de Contraste , Fluordesoxiglucose F18 , Seguimentos , Gastroenteropatias/diagnóstico , Trato Gastrointestinal/metabolismo , Tomografia por Emissão de Pósitrons/métodos , Reprodutibilidade dos Testes , Estudos Retrospectivos , Tomografia Computadorizada por Raios X/métodos
7.
Acta Radiol ; 53(9): 1020-5, 2012 Nov 01.
Artigo em Inglês | MEDLINE | ID: mdl-22969090

RESUMO

BACKGROUND: Sufficient radiopacity of stents is a prerequisite for safe interventions and minimization of the radiation dose for the patient and the interventionist. Modern nitinol stents are considered less radiopaque compared to formerly used stents. PURPOSE: To evaluate the objective detection rate (ODR) and the subjective radiopacity score (SRS) of four self-expanding nitinol stents with their markers on a phantom human pelvis. MATERIAL AND METHODS: We evaluated the ODR (as a percentage of correctly identified stents) and the SRS (on a scale from 0 = not visible to 4 = excellent visibility) for four self-expanding nitinol stents (SinusSuperflex, SMART, Luminexx, Zilver) with 8 mm diameter and 40 mm length. Stents were placed on a phantom human pelvis and images of the stents were taken in four different positions (right and left lumbosacral joint and near the right and left limbus acetabuli) using the following modes: spotfilm, pulsed fluoroscopy (4, 7.5, 15, and 30 pulses/min) and at three different digital magnification modes. Dose area products (DAPs) were assessed. RESULTS: ODR and SRS, respectively, were significantly increased for the SMART stent compared to all other tested stents (P < 0.05): SMART 93.53% and 2.43, SinusSuperflex 90.81% and 2.21, Luminexx 90.39% and 2.20, and Zilver 89.28% and 2.21. ODR was significantly reduced in position 3 where the bone overlap was more pronounced for all stents (detection rates 77.14-79.56%). An increase in magnification significantly improved the ODR and SRS for all stents (70.33-99.25% and 1.07-3.28, respectively, P < 0.05). Increased pulsing frequency did not improve the ODR of the various stents but did increase the DAP. CONCLUSION: The SMART stent had the best overall performance. In the presence of bone overlap, all self-expanding nitinol stents had poor results. Increased pulsing frequency did not improve ODR or SRS but did increase the DAP. Use of digital magnification modes had no effect on DAP increasing ODR and SRS.


Assuntos
Ligas , Angioplastia/instrumentação , Fluoroscopia , Humanos , Técnicas In Vitro , Pelve , Imagens de Fantasmas , Desenho de Prótese , Doses de Radiação , Estatísticas não Paramétricas , Stents
8.
AJR Am J Roentgenol ; 199(3): W276-82, 2012 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-22915418

RESUMO

OBJECTIVE: The purposes of this article are to provide a practical review of the spectrum of imaging findings in patients with systemic IgG4-related sclerosing disease and to address the differential diagnoses. CONCLUSION: IgG4-related sclerosing disease is a systemic disorder that can involve almost any organ. The imaging findings consist of diffuse and focal organ infiltration and encasement by inflammatory and fibrotic tissue. Awareness of the spectrum of imaging findings in IgG4-related disease should prompt further evaluation for systemic manifestations to avoid misdiagnosis.


Assuntos
Imunoglobulina G/análise , Escleroderma Sistêmico/diagnóstico , Idoso , Diagnóstico Diferencial , Feminino , Humanos , Imageamento por Ressonância Magnética , Masculino , Pessoa de Meia-Idade , Tomografia por Emissão de Pósitrons , Escleroderma Sistêmico/complicações , Escleroderma Sistêmico/imunologia , Tomografia Computadorizada por Raios X
9.
AJR Am J Roentgenol ; 198(6): 1239-46, 2012 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-22623535

RESUMO

OBJECTIVE: The purpose of this article is to measure perfusion parameters, including transit constant (K(trans)), in untreated follicular and diffuse large B cell lymphoma using volume perfusion CT, to establish their discriminating role and to search for a possible histopathologic background. SUBJECTS AND METHODS: Between January 2010 and June 2011, 46 consecutive patients with untreated histologically confirmed follicular lymphoma (n = 16) or diffuse large B cell lymphoma (n = 30) were enrolled. A 40-second volume perfusion CT of the tumor bulk using 6.9-cm z-axis coverage and a total of 26 volume measurements was performed. Blood flow (BF), blood volume (BV), and K(trans) were determined. Tumor size was recorded as the product of long- and short-axis diameters. In 13 of 46 patients, pathologic specimens of an appropriate size were available for assessment of microvessel density (MVD) and microvascular luminal diameter for comparison with volume perfusion CT measurements. RESULTS: Mean BF, BV, and K(trans) values were significantly higher in follicular lymphoma than in diffuse large B cell lymphoma, even after controlling for patient age and tumor size (p < 0.05, respectively). Although MVD was slightly, but not significantly, higher in follicular lymphoma versus diffuse large B cell lymphoma (p > 0.05), microvascular luminal diameter was significantly larger in follicular lymphoma than in diffuse large B cell lymphoma (p < 0.05). We defined cutoff values for BF, BV, and K(trans). If the cutoff points are met for all three parameters, the overall accuracy for correctly identifying diffuse large B cell lymphoma and follicular lymphoma was 90.5% and 87.5%, respectively. CONCLUSION: Volume perfusion CT allows assessment of differences in vascularity of follicular and diffuse large B cell lymphomas, reflecting vascular luminal variability and histopathologic anatomy.


Assuntos
Linfoma Folicular/diagnóstico por imagem , Linfoma Difuso de Grandes Células B/diagnóstico por imagem , Neovascularização Patológica/diagnóstico por imagem , Tomografia Computadorizada por Raios X/métodos , Adulto , Idoso , Idoso de 80 Anos ou mais , Algoritmos , Velocidade do Fluxo Sanguíneo , Volume Sanguíneo , Meios de Contraste , Feminino , Humanos , Imuno-Histoquímica , Iohexol/análogos & derivados , Análise dos Mínimos Quadrados , Linfoma Folicular/patologia , Linfoma Difuso de Grandes Células B/patologia , Masculino , Pessoa de Meia-Idade , Neovascularização Patológica/patologia
10.
AJR Am J Roentgenol ; 198(4): 946-54, 2012 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-22451565

RESUMO

OBJECTIVE: The objective of this study was to compare the performance and radiation doses of a flat-panel detector (FPD) angiography machine with an image intensifier (II) angiography machine. MATERIALS AND METHODS: Images of four nitinol stents (Sinus-SuperFlex, SMART, Luminexx, and Zilver stents) in a phantom of a human pelvis were acquired on an FPD system (Axiom Artis) and an II system (Fluorospot TOP) using the following modes: spot-film, continuous fluoroscopy (4, 7.5, 15, and 30 pulses/s), and three amplification modes. Objective stent detection rates and subjective radiopacity scores (scale: 0 [not visible] to 4 [excellent visibility]) were calculated. The radiation doses evaluated by the respective machines were compared. RESULTS: Over all modes and stents, the mean objective correct stent detection rates and mean subjective radiopacity scores were 89.49% and 1.81, respectively, for the Axiom Artis and 91.00% and 2.26 for the Fluorospot TOP. The stent detection rates over all modes for the SMART and Luminexx stents were better using the Axiom Artis machine (97.61% vs 93.55% and 98.28% vs 90.41%, respectively) and those for the Sinus-SuperFlex and Zilver stents were better using the Fluorospot TOP machine (90.83% vs 83.56% and 89.29% vs 80.50%). The subjective radiopacity scores of stent visibility were worse for the Axiom Artis than the Fluorospot TOP for all stents except the Luminexx stent (mean score, 2.34 vs 2.21, respectively). The objective stent detection rates and subjective radiopacity scores improved using the spot-film mode and with raising amplification, whereas increases in the fluoroscopy pulsing frequency did not improve stent detection rates or radiopacity scores for either machine. The radiation doses at continuous fluoroscopy were approximately 90% higher for the Axiom Artis than for the Fluorospot TOP (2.60 vs 1.41 µGy/m(2) at 30 pulses/s, respectively). CONCLUSION: The objective correct stent detection rates were similar for both machines with differences in detection for the respective stents. The subjective radiopacity scores were almost always better for the Fluorospot TOP machine. Also, the Axiom Artis machine generated approximately 90% higher radiation doses in fluoroscopy. For both machines, using a higher fluoroscopy pulsing frequency had no positive effect on objective correct stent detection rates or subjective radiopacity scores.


Assuntos
Angiografia/instrumentação , Pelve/diagnóstico por imagem , Stents , Ecrans Intensificadores para Raios X , Ligas , Fluoroscopia/instrumentação , Humanos , Imagens de Fantasmas , Desenho de Prótese , Doses de Radiação , Estatísticas não Paramétricas
11.
J Neurol ; 257(9): 1473-81, 2010 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-20383521

RESUMO

Neuromuscular disorders are rare diseases with a chronic and debilitating course. Unfortunately, data on the health-related quality of life (HRQoL) in neuromuscular diseases are limited. The objective of this multicentre cross-sectional study was to compare the HRQoL in patients with amyotrophic lateral sclerosis (ALS), facioscapulohumeral muscular dystrophy (FSHD) and myasthenia gravis (MG) and to identify the determinants of the HRQoL in these diseases. We recruited 91 consecutive outpatients with ALS (n = 37), FSHD (n = 17) or MG (n = 37) in seven specialized German health centres. The HRQoL was determined using the 36-Item Short Form Health Survey (SF-36) and the EuroQol (EQ-5D). Independent predictors of the HRQoL were identified using multiple regression analysis. The HRQoL in all domains of the SF-36, except for bodily pain, was significantly reduced. The domains related to physical health (physical functioning, physical role) were most affected. The EQ-5D-index score was most reduced in ALS (0.54) and least reduced in MG (0.89). Independent predictors of a reduced HRQoL were disease severity and depression in ALS, and disease severity, depression, older age and increased body-mass index in MG. The patterns of HRQoL-impairment in neuromuscular disorders share some common features, such as a more pronounced reduction in the HRQoL related to physical health, but there are a number of disease-specific features that should be considered in outcomes of clinical trials and treatment guidelines. In addition to the treatment of motor symptoms, greater attention should be paid to the treatment of depression, which was found to be among the independent predictors of the HRQoL in ALS and MG.


Assuntos
Esclerose Lateral Amiotrófica/diagnóstico , Nível de Saúde , Distrofia Muscular Facioescapuloumeral/diagnóstico , Miastenia Gravis/diagnóstico , Qualidade de Vida , Adulto , Idoso , Esclerose Lateral Amiotrófica/epidemiologia , Esclerose Lateral Amiotrófica/psicologia , Comorbidade , Estudos Transversais , Feminino , Alemanha/epidemiologia , Humanos , Masculino , Pessoa de Meia-Idade , Distrofia Muscular Facioescapuloumeral/epidemiologia , Distrofia Muscular Facioescapuloumeral/psicologia , Miastenia Gravis/epidemiologia , Miastenia Gravis/psicologia , Valor Preditivo dos Testes , Qualidade de Vida/psicologia
12.
J Neurol ; 257(1): 15-23, 2010 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-19629566

RESUMO

Neuromuscular disorders (NMD) are chronic devastating diseases. The aim of this multicenter cross-sectional study was to evaluate the socioeconomic impact of three NMDs in Germany. Patients (n = 107) with amyotrophic lateral sclerosis (ALS), myasthenia gravis (MG) or facioscapulohumeral muscular dystrophy (FSHD) were recruited consecutively in seven centers in Germany. The health-economic data were collected using a "bottom-up" approach consisting of comprehensive questionnaires and patient diaries. Costs were evaluated from the societal perspective in 2009 Euros (EUR). Total annual costs from the societal perspective were EUR 36,380 (95% CI 27,090-47,970) per patient in ALS, EUR 26,240 (95% CI 17,770-37,940) in FSHD and EUR 14,950 (95% CI 10,470-21,730) in MG. The main components of costs were the expenditures of health insurance and the loss of productivity of patients and their caregivers. The following independent cost-driving factors were identified: disease severity, assistance in activities of daily living (ADL), dementia and younger age in ALS, disease severity in FSHD and assistance in ADL, disease severity and assistance in ADL in MG. The socioeconomic burden of NMDs in Germany is considerable. Further studies evaluating both the health-economic and clinical effects of NMD treatment as well as disease management programs and benchmarking activities are necessary.


Assuntos
Esclerose Lateral Amiotrófica/economia , Distrofia Muscular Facioescapuloumeral/economia , Miastenia Gravis/economia , Adolescente , Adulto , Fatores Etários , Idoso , Idoso de 80 Anos ou mais , Esclerose Lateral Amiotrófica/tratamento farmacológico , Esclerose Lateral Amiotrófica/terapia , Estudos Transversais , Demência/economia , Feminino , Alemanha , Custos de Cuidados de Saúde , Gastos em Saúde , Humanos , Seguro Saúde , Masculino , Pessoa de Meia-Idade , Distrofia Muscular Facioescapuloumeral/tratamento farmacológico , Distrofia Muscular Facioescapuloumeral/terapia , Miastenia Gravis/tratamento farmacológico , Miastenia Gravis/terapia , Fatores Socioeconômicos , Adulto Jovem
13.
Neuromuscul Disord ; 19(3): 223-8, 2009 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-19208478

RESUMO

We report seven patients with immune-mediated rippling muscle disease (iRMD) and AChR-antibody positive myasthenia gravis (MG) without germline caveolin-3 gene mutations. We describe the follow-up of two patients and the clinical features of five new patients (1 female, 4 male, aged 32 to 69 years). These presented with significant generalized, exercise-induced and electrically-silent muscle rippling with myalgia, combined with generalized MG. In two of the seven patients, MG appeared before iRMD. Mediastinal imaging excluded thymic alterations in all, although two had other coincident tumours. Myalgia and rippling were aggravated by acetylcholinesterase-inhibitor treatment. Generalized MG and iRMD were successfully treated with plasma exchange, steroids and azathioprine in the two patients followed long-term. Muscle morphology of five patients showed a minimal myopathic pattern with rare lymphohistiocytic infiltration. In four patients, sarcolemmal caveolin-3, and dysferlin immunofluorescence staining was moderately reduced in a mosaic pattern, but caveolin-3 protein on Western blots was clearly reduced only in two. Notably, electron microscopy showed that caveolae were almost completely lost at the sarcolemma in the three biopsies examined but not in endothelium. Antibodies targeting high molecular weight muscle proteins, likely associated with the neuromuscular endplate and sarcolemma, were found in the iRMD patients but also in age-matched MG patients without iRMD. Since the generalized MG and iRMD improved with immunosuppressive treatments, it is likely that both are caused by autoantibodies, but the target for pathogenic antibodies in iRMD requires further study.


Assuntos
Músculo Esquelético/imunologia , Doenças Musculares/imunologia , Miastenia Gravis/imunologia , Adulto , Idoso , Autoanticorpos/análise , Autoanticorpos/sangue , Azatioprina/uso terapêutico , Cavéolas/patologia , Caveolina 3/metabolismo , Inibidores da Colinesterase/uso terapêutico , Eletromiografia , Feminino , Seguimentos , Humanos , Masculino , Pessoa de Meia-Idade , Contração Muscular/imunologia , Fibras Musculares Esqueléticas/imunologia , Fibras Musculares Esqueléticas/metabolismo , Fibras Musculares Esqueléticas/patologia , Proteínas Musculares/imunologia , Músculo Esquelético/metabolismo , Músculo Esquelético/patologia , Doenças Musculares/patologia , Doenças Musculares/terapia , Miastenia Gravis/patologia , Miastenia Gravis/terapia , Plasmaferese , Esteroides/uso terapêutico , Timo/imunologia , Timo/patologia , Timo/fisiopatologia
14.
Clin Neurol Neurosurg ; 108(2): 135-42, 2006 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-16412834

RESUMO

OBJECTIVE AND PURPOSE: Neurological involvement is a common feature of the antiphospholipid syndrome (APS). A variety of thrombotic and non-thrombotic manifestations may accompany the presence of antiphospholipid antibodies (aPL). PATIENTS AND METHODS: We retrospectively reviewed the prevalence of aPL in a cohort of over 350 unselected patients from a neurological clinic and studied the neurological manifestations of APS. RESULTS: We found that within this cohort the prevalence of aPL was about 15%. Most of the patients with aPL suffered from strokes and transient ischemic attacks (TIA). One patient died from spinal infarction. Non-thrombotic manifestations also occurred in 40% of these patients, such as multiple sclerosis, chorea, seizures or cerebral malignancies. No significant correlations of the titres or different types of aPL and the type of the neurological symptoms could be found. In comparison to age and sex matched patients of the cohort where the presence of aPL could be excluded, the occurrence of non-thrombotic manifestations was significantly more frequent and varied in the group of patients with aPL. The higher incidence of stroke in the non-APS group could be explained by the significantly higher presence of other laboratory risk factors, mainly hypercholesterinemia. CONCLUSION: This investigation indicates that aPL may play an important role in the etiology of various neurological syndromes.


Assuntos
Anticorpos Antifosfolipídeos/sangue , Síndrome Antifosfolipídica/complicações , Doenças do Sistema Nervoso/sangue , Doenças do Sistema Nervoso/etiologia , Adulto , Idoso , Idoso de 80 Anos ou mais , Estudos de Coortes , Feminino , Glicoproteínas/sangue , Humanos , Imunoglobulina G/sangue , Imunoglobulina M/sangue , Masculino , Pessoa de Meia-Idade , Estudos Retrospectivos , Fatores de Risco , beta 2-Glicoproteína I
15.
Immunobiology ; 210(10): 755-60, 2005.
Artigo em Inglês | MEDLINE | ID: mdl-16325494

RESUMO

High-sensitive C-reactive protein (hs-CRP) is a marker of inflammation which has been shown in several prospective studies to independently predict myocardial infarction, stroke and peripheral artery disease. Patients with antiphospholipid antibodies (aPL) are at increased risk of recurrent thromboembolic events, but the possibility of predicting this risk seems rather limited. Similarities were recently found between aPL and CRP in the pathology of thrombosis. The current study investigated the predictive role of hs-CRP in a cohort of patients with neurological manifestations. A follow-up investigation was done in a cohort of 55 aPL-positive patients with acute manifestations of neurological disease. hs-CRP levels were measured in all patients at enrollment and were compared to the patients' condition after a median period of 32 months. Lupus anticoagulants were detected according to the Standardization of Lupus Anticoagulants (SSC) of the ISTH. Anticardiolipin tests were performed by a beta2-glycoprotein I-dependent enzyme-linked immunsorbent assay (Pharmacia ELISA). hs-CRP was measured by latex-enhanced turbidometry (dimension RXL, Dade Behring). Cerebral infarctions and transient ischemic attacks were the most frequent cerebral events. In patients with aPL, elevated levels of hs-CRP were closely associated with an increased rate of recurrent or residual symptoms (OR, 12.5; 95% CI, 3.72-41.94) and were not related to other risk factors, except smoking (p<0.05). The rate at which a given patient's condition deteriorated was also related to the level of hs-CRP. In patients with antiphospholipid syndrome (APS), elevated levels of hs-CRP may identify a group of patients which is at high risk of recurrent or residual neurological symptoms and which may benefit from more careful follow-up and from antithrombotic therapy.


Assuntos
Síndrome Antifosfolipídica/metabolismo , Proteína C-Reativa/metabolismo , Doenças do Sistema Nervoso/etiologia , Adolescente , Adulto , Idoso , Idoso de 80 Anos ou mais , Anticorpos Anticardiolipina/imunologia , Síndrome Antifosfolipídica/complicações , Síndrome Antifosfolipídica/imunologia , Estudos de Coortes , Feminino , Humanos , Inibidor de Coagulação do Lúpus/imunologia , Masculino , Pessoa de Meia-Idade , Prognóstico , Fatores de Risco
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