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1.
BMC Oral Health ; 23(1): 381, 2023 06 12.
Artigo em Inglês | MEDLINE | ID: mdl-37308874

RESUMO

BACKGROUND: The difficulties and challenges faced by people with Parkinson's disease (PD) in performing daily orofacial function are not systematically investigated. In this study, specific orofacial non-motor and motor symptoms and functions were systematically examined in PD patients in comparison to a matched control group. METHODS: The clinical case-controlled study was conducted from May 2021 to October 2022 and included persons with PD and age- and gender-matched persons without PD. The participants with PD were outpatients diagnosed with PD at the Department of Neurology at Bispebjerg University Hospital in Copenhagen, Denmark. The participants underwent a systematic clinical and relevant self-assessment of the orofacial function and temporomandibular disorders (TMD). The primary outcomes were objective and subjective assessments of the general orofacial function, mastication, swallowing, xerostomia and drooling. The secondary outcomes were the prevalence of TMD and orofacial pain. The difference in outcome measures between the two groups was analysed using chi-square and Mann-Whitney U test. RESULTS: The study included 20 persons with PD and 20 age- and gender-matched persons without PD. Both objectively and subjectively, persons with PD had poorer orofacial function than the control group. Persons with PD had also a significantly more severe limitation of jaw mobility and jaw function. The objective masticatory function was also significantly reduced for persons with PD compared to the control group, and 60% of persons with PD found it difficult to eat foods with certain consistencies while 0% of the control group reported that problem. Persons with PD could swallow less water per second and the average swallowing event was significantly longer for PD persons. Even though PD persons reported more xerostomia (58% for persons with PD and 20% for control persons), they also reported significantly more drooling than the control group. Additionally, orofacial pain was more prevalent in PD persons. CONCLUSIONS: Persons with PD have a compromised orofacial function. Furthermore, the study indicates a link between PD and orofacial pain. In order to screen and treat persons with PD accordingly, healthcare professionals should be aware of and address these limitations and symptoms. TRIAL REGISTRATION: The trial was approved by the Regional Committee on Research Health Ethics of the Capital Region (H-20,047,464), the Danish Data Protection Agency (514 - 0510/20-3000), and registered at ClinicalTrials.gov (NCT05356845).


Assuntos
Doença de Parkinson , Sialorreia , Transtornos da Articulação Temporomandibular , Humanos , Conscientização , Dor Facial
2.
J Mov Disord ; 16(1): 98-100, 2023 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-36353802
3.
J Endocr Soc ; 6(9): bvac111, 2022 Sep 01.
Artigo em Inglês | MEDLINE | ID: mdl-35935071

RESUMO

Context and objective: Obesity and inactivity are risk factors for developing impaired glucose tolerance characterized by insulin resistance and reduced beta-cell function. The stimulatory effect of glucagon-like peptide 1 (GLP-1) on insulin secretion is also impaired in obese, inactive individuals. The aim of this study was to investigate whether endurance training influences beta-cell sensitivity to GLP-1. Participants and intervention: Twenty-four female participants, age 46 ±â€…2 years, body mass index 32.4 ±â€…0.9 kg/m2, and maximal oxygen consumption 24.7 ±â€…0.8 mL/kg/min participated in a 10-week exercise training study. Methods: Beta-cell sensitivity to GLP-1 was assessed in a subset of participants (n = 6) during a 120-minute hyperglycemic glucose clamp (8.5 mM) including a 1-hour GLP-1 (7-36 amide) infusion (0.4 pmol/kg/min). Changes in glucose tolerance, body composition, and cardiorespiratory fitness were assessed by oral glucose tolerance tests (OGTTs), dual-energy X-ray absorptiometry scans, magnetic resonance scans, and maximal oxygen consumption (VO2max) tests, respectively. Results: The c-peptide response to infusion of GLP-1 increased 28 ±â€…3% (P < 0.05) toward the end of the hyperglycemic clamp. The insulin response remained unchanged. Training improved glucose tolerance and reduced GLP-1, insulin, and glucagon levels during the OGTTs. Training increased VO2max (from 24.7 ±â€…0.8 to 27.0 ±â€…0.7 mL/kg/min; P < 0.05) and reduced visceral fat volume (from 4176 ±â€…265 to 3888 ±â€…266 cm3; P < 0.01). Conclusion: Along with improved glycemic control, endurance training improved beta-cell sensitivity to GLP-1 in overweight women. The study was deemed not to constitute a clinical trial and was not registered as such.

4.
J Neurosci Methods ; 378: 109659, 2022 08 01.
Artigo em Inglês | MEDLINE | ID: mdl-35772608

RESUMO

BACKGROUND: Neuromodulation is a rapidly expanding therapeutic option considered within neuropsychiatry, pain and rehabilitation therapy. Combining electrostimulation with feedback from fMRI can provide information about the mechanisms underlying the therapeutic effects, but so far, such studies have been hampered by the lack of technology to conduct safe and accurate experiments. Here we present a system for fMRI compatible electrical stimulation, and the first proof-of-concept neuroimaging data with deep brain stimulation (DBS) in pigs obtained with the device. NEW METHOD: The system consists of two modules, placed in the control and scanner room, connected by optical fiber. The system also connects to the MRI scanner to timely initiate the stimulation sequence at start of scan. We evaluated the system in four pigs with DBS in the subthalamic nucleus (STN) while we acquired BOLD responses in the STN and neocortex. RESULTS: We found that the system delivered robust electrical stimuli to the implanted electrode in sync with the preprogrammed fMRI sequence. All pigs displayed a DBS-STN induced neocortical BOLD response, but none in the STN. COMPARISONS WITH EXISTING METHOD: The system solves three major problems related to electric stimuli and fMRI examinations, namely preventing distortion of the fMRI signal, enabling communication that synchronize the experimental conditions, and surmounting the safety hazards caused by interference with the MRI scanner. CONCLUSIONS: The fMRI compatible electrical stimulator circumvents previous problems related to electroceuticals and fMRI. The system allows flexible modifications for fMRI designs and stimulation parameters, and can be customized to electroceutical applications beyond DBS.


Assuntos
Estimulação Encefálica Profunda , Núcleo Subtalâmico , Animais , Estimulação Elétrica , Imageamento por Ressonância Magnética/métodos , Neuroimagem/métodos , Núcleo Subtalâmico/diagnóstico por imagem , Núcleo Subtalâmico/fisiologia , Suínos
5.
Aesthet Surg J ; 42(11): 1279-1289, 2022 10 13.
Artigo em Inglês | MEDLINE | ID: mdl-35704475

RESUMO

BACKGROUND: The main challenge with fat grafting is loss of some of the graft to postsurgery resorption. Previous studies suggest that adipose-derived stromal cells (ASCs) can improve the volume retention of fat grafts but there is a lack of randomized trials to support the use of ASCs in clinical practice. OBJECTIVES: This trial aimed to investigate whether ASCs improve fat graft volume retention in patients undergoing breast augmentation with lipofilling. METHODS: This was a double-blind, randomized controlled trial of breast augmentation with ASC-enriched fat grafting. Healthy women aged 30 to 45 years were enrolled. First, the participants underwent liposuction to obtain fat for culture expansion of ASCs. Then, the participants were randomly assigned to undergo a 300- to 350-mL breast augmentation with ASC-enriched fat grafting (10 × 106 ASCs/mL fat graft) to 1 of their breasts and placebo-enriched fat grafting of identical volume to the contralateral breast. The primary outcome was fat graft volume retention after a 1-year follow-up measured with MRI. The trial is registered at www.clinicaltrialsregister.eu (EudraCT-2014-000510-59). RESULTS: Ten participants were included in the trial; all completed the treatment and follow-up. No serious adverse events occurred. Fat graft volume retention after 1 year was 54.0% (95% CI, 30.4%-77.6%) in the breasts treated with ASC-enriched fat grafting (n = 10) and 55.9% (95% CI, 28.9%-82.9%) in the contralateral breasts treated with placebo-enriched fat grafting (n = 10) (P = 0.566). CONCLUSIONS: The findings of this trial do not support that ASC-enriched fat grafting is superior to standard fat grafting for breast augmentation.


Assuntos
Lipectomia , Mamoplastia , Transplante de Células-Tronco Mesenquimais , Tecido Adiposo/transplante , Feminino , Humanos , Células Estromais/transplante
6.
Sci Rep ; 11(1): 14467, 2021 07 14.
Artigo em Inglês | MEDLINE | ID: mdl-34262077

RESUMO

As part of normal ageing, conductance arteries lose their cushion function, left ventricle (LV) filling and also left atrial emptying are impaired. The relation between conductance artery stiffness and LV diastolic function is normally explained by arterial hypertension and LV hypertrophy as needed intermediaries. We examined whether age-related aortic stiffening may influence LV diastolic function in normal healthy subjects. Aortic distensibility and pulse wave velocity (PWV) were related to LV emptying and filling parameters and left atrial emptying parameters as determined by magnetic resonance imaging in 36 healthy young (< 35 years) and 16 healthy middle-aged and elderly (> 35 years) with normal arterial blood pressure and myocardial mass. In the overall cohort, total aorta PWV correlated to a decrease in LV peak-emptying volume (r = 0.43), LV peak-filling (r = 0.47), passive atrial emptying volume (r = 0.66), and an increase in active atrial emptying volume (r = 0.47) (all p < 0.001). PWV was correlated to passive atrial emptying volume even if only the > 35-year-old were considered (r = 0.53; p < 0.001). Total peripheral resistance demonstrated similar correlations as PWV, but in a regression analysis only the total aorta PWV was related to left atrial (LA) passive emptying volume. Via impaired ventriculo-arterial coupling, the increased aortic PWV seen with normal ageing hence affects atrio-ventricular coupling, before increased aortic PWV is associated with significantly increased arterial blood pressure or LV hypertrophic remodelling. Our findings reinforce the existence of atrio-ventriculo-arterial coupling and suggest aortic distensibility should be considered an early therapeutic target to avoid diastolic dysfunction of the LV.


Assuntos
Hipertensão/fisiopatologia , Rigidez Vascular , Remodelação Ventricular/fisiologia , Adulto , Fatores Etários , Idoso , Estudos de Casos e Controles , Feminino , Humanos , Hipertensão/diagnóstico por imagem , Imageamento por Ressonância Magnética , Masculino , Pessoa de Meia-Idade , Análise de Regressão , Função Ventricular Esquerda
7.
Sci Rep ; 11(1): 14750, 2021 07 20.
Artigo em Inglês | MEDLINE | ID: mdl-34285301

RESUMO

Menopause is associated with a redistribution of adipose tissue towards central adiposity, known to cause insulin resistance. In this cross-sectional study of 33 women between 45 and 60 years, we assessed adipose tissue inflammation and morphology in subcutaneous adipose tissue (SAT) and visceral adipose tissue (VAT) across menopause and related this to menopausal differences in adipose tissue distribution and insulin resistance. We collected paired SAT and VAT biopsies from all women and combined this with anthropometric measurements and estimated whole-body insulin sensitivity. We found that menopause was associated with changes in adipose tissue phenotype related to metabolic dysfunction. In SAT, postmenopausal women showed adipocyte hypertrophy, increased inflammation, hypoxia and fibrosis. The postmenopausal changes in SAT was associated with increased visceral fat accumulation. In VAT, menopause was associated with adipocyte hypertrophy, immune cell infiltration and fibrosis. The postmenopausal changes in VAT phenotype was associated with decreased insulin sensitivity. Based on these findings we suggest, that menopause is associated with changes in adipose tissue phenotype related to metabolic dysfunction in both SAT and VAT. Whereas increased SAT inflammation in the context of menopause is associated with VAT accumulation, VAT morphology is related to insulin resistance.


Assuntos
Gordura Intra-Abdominal/patologia , Gordura Subcutânea Abdominal/patologia , Envelhecimento , Distribuição da Gordura Corporal , Receptor alfa de Estrogênio/genética , Receptor alfa de Estrogênio/metabolismo , Feminino , Humanos , Inflamação/patologia , Insulina/metabolismo , Resistência à Insulina , Gordura Intra-Abdominal/metabolismo , Menopausa , Síndrome Metabólica/patologia , Pessoa de Meia-Idade , Receptores Ativados por Proliferador de Peroxissomo/genética , Receptores Ativados por Proliferador de Peroxissomo/metabolismo , Fenótipo , Gordura Subcutânea Abdominal/metabolismo
8.
Am J Ophthalmol Case Rep ; 22: 101092, 2021 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-34095606

RESUMO

PURPOSE: Rhino-orbital-cerebral mucormycosis (ROCM) is a rare opportunistic infection with a high mortality despite relevant treatment. OBSERVATIONS: A 3-year-old girl under treatment for acute lymphoblastic leukemia developed periorbital swelling, ophthalmoplegia and a necrotic palatal lesion during a period of neutropenia. Imaging revealed sinusitis, pre- and postseptal cellulitis. The disease later progressed to cerebral involvement and orbital apex syndrome with complete ophthalmoplegia, ptosis and loss of vision. The patient was treated with systemic antifungal therapy, hyperbaric oxygen and extensive surgery. This included orbital exenteration, skull base resection, cerebral debridement with placement of an Ommaya reservoir for intrathecal administrations of amphotericin B (AmB) and in addition endoscopic sinus surgery with local AmB installation. Chemotherapy was safely continued after resolution of the ROCM and the patient remains in complete remission after 5 years. CONCLUSION AND IMPORTANCE: Patients with ROCM can be cured with aggressive multimodality treatment, including surgical intervention, even if in myelosuppression.

9.
Neuro Oncol ; 23(12): 2107-2116, 2021 12 01.
Artigo em Inglês | MEDLINE | ID: mdl-33864083

RESUMO

BACKGROUND: Central nervous system (CNS) tumors cause the highest death rates among childhood cancers, and survivors frequently have severe late effects. Magnetic resonance imaging (MRI) is the imaging modality of choice, but its specificity can be challenged by treatment-induced signal changes. In adults, O-(2-[18F]fluoroethyl)-l-tyrosine ([18F]FET) PET can assist in interpreting MRI findings. We assessed the clinical impact and diagnostic accuracy of adding [18F]FET PET to MRI in children with CNS tumors. METHODS: A total of 169 [18F]FET PET scans were performed in 97 prospectively and consecutively included patients with known or suspected childhood CNS tumors. Scans were performed at primary diagnosis, before or after treatment, or at relapse. RESULTS: Adding [18F]FET PET to MRI impacted clinical management in 8% [95% confidence interval (CI): 4%-13%] of all scans (n = 151) and in 33% [CI: 17%-53%] of scans deemed clinically indicated due to difficult decision making on MRI alone (n = 30). Using pathology or follow-up as reference standard, the addition of [18F]FET PET increased specificity (1.00 [0.82-1.00] vs 0.48 [0.30-0.70], P = .0001) and accuracy (0.91 [CI: 0.87-0.96] vs 0.81 [CI: 0.75-0.89], P = .04) in 83 treated lesions and accuracy in 58 untreated lesions (0.96 [CI: 0.91-1.00] vs 0.90 [CI: 0.82-0.92], P < .001). Further, in a subset of patients (n = 15) [18F]FET uptake correlated positively with genomic proliferation index. CONCLUSIONS: The addition of [18F]FET PET to MRI helped discriminate tumor from non-tumor lesions in the largest consecutive cohort of pediatric CNS tumor patients presented to date.


Assuntos
Neoplasias Encefálicas , Glioma , Adulto , Criança , Humanos , Imageamento por Ressonância Magnética , Tomografia por Emissão de Pósitrons , Compostos Radiofarmacêuticos , Tirosina
10.
J Cereb Blood Flow Metab ; 41(8): 1954-1963, 2021 08.
Artigo em Inglês | MEDLINE | ID: mdl-33461410

RESUMO

Patients with Parkinson's disease (PD) often suffer from non-motor symptoms, which may be caused by serotonergic dysfunction. Apart from alleviating the motor symptoms, Deep Brain Stimulation (DBS) in the subthalamic nucleus (STN) may also influence non-motor symptoms. The aim of this study is to investigate how turning DBS off affects the serotonergic system. We here exploit a novel functional PET neuroimaging methodology to evaluate the preservation of serotonergic neurons and capacity to release serotonin. We measured cerebral 5-HT1BR binding in 13 DBS-STN treated PD patients, at baseline and after turning DBS off. Ten age-matched volunteers served as controls. Clinical measures of motor symptoms were assessed under the two conditions and correlated to the PET measures of the static and dynamic integrity of the serotonergic system. PD patients exhibited a significant loss of frontal and parietal 5-HT1BR, and the loss was significantly correlated to motor symptom severity. We saw a corresponding release of serotonin, but only in brain regions with preserved 5-HT1BR, suggesting the presence of a presynaptic serotonergic deficit. Our study demonstrates that DBS-STN dynamically regulates the serotonin system in PD, and that preservation of serotonergic functions may be predictive of DBS-STN effects.


Assuntos
Estimulação Encefálica Profunda , Doença de Parkinson/terapia , Tomografia por Emissão de Pósitrons , Núcleo Subtalâmico/fisiopatologia , Idoso , Estudos de Casos e Controles , Feminino , Lobo Frontal/diagnóstico por imagem , Lobo Frontal/fisiologia , Humanos , Masculino , Pessoa de Meia-Idade , Doença de Parkinson/diagnóstico por imagem , Receptor 5-HT1B de Serotonina/metabolismo , Neurônios Serotoninérgicos/metabolismo , Serotonina/metabolismo
11.
Neurology ; 95(9): e1211-e1221, 2020 09 01.
Artigo em Inglês | MEDLINE | ID: mdl-32611642

RESUMO

OBJECTIVE: We followed up patients with facioscapulohumeral muscular dystrophy (FSHD) with sequential examinations over 2 years to investigate whether inflammatory lesions always precede fat replacement, if inflammation can be resolved without muscle degeneration, and if inflammatory lesions in muscle are always followed by fat replacement. METHODS: In this longitudinal study of 10 sequential MRI assessments over 2.5 years, we included 10 patients with FSHD. We used MRI with short TI inversion recovery to identify regions of interest (ROIs) with hyperintensities indicating muscle inflammation. Muscle T2 relaxation time mapping was used as a quantitative marker of muscle inflammation. Dixon sequences quantified muscle fat replacement. Ten healthy controls were examined with a magnetic resonance scan once for determination of normal values of T2 relaxation time. RESULTS: We identified 68 ROIs with T2 elevation in the patients with FSHD. New ROIs with T2 elevation arising during the study had muscle fat content of 6.4% to 33.0% (n = 8) and 47.0% to 78.0% lesions that resolved (n = 6). ROIs with T2 elevation had a higher increase in muscle fat content from visits 1 to 10 (7.9 ± 7.9%) compared to ROIs with normal muscle T2 relaxation times (1.7 ± 2.6%; p < 0.0001). Severe T2 elevations were always followed by an accelerated replacement of muscle by fat. CONCLUSIONS: Our results suggest that muscle inflammation starts in mildly affected muscles in FSHD, is related to a faster muscle degradation, and continues until the muscles are completely fat replaced. CLINICALTRIALSGOV IDENTIFIER: NCT02159612.


Assuntos
Tecido Adiposo/diagnóstico por imagem , Inflamação/diagnóstico por imagem , Músculo Esquelético/diagnóstico por imagem , Distrofia Muscular Facioescapuloumeral/diagnóstico por imagem , Adulto , Progressão da Doença , Feminino , Humanos , Perna (Membro) , Estudos Longitudinais , Imageamento por Ressonância Magnética , Masculino , Pessoa de Meia-Idade , Força Muscular , Dinamômetro de Força Muscular , Distrofia Muscular Facioescapuloumeral/fisiopatologia , Coxa da Perna , Teste de Caminhada
12.
Sci Rep ; 10(1): 7246, 2020 04 29.
Artigo em Inglês | MEDLINE | ID: mdl-32350361

RESUMO

Muscle inflammation is an important component of disease pathophysiology in several muscular dystrophies. Hyperintensities on MRI sequences with short TI inversion recovery (STIR) reflect edema, or inflammation (STIR+). Conventionally, STIR evaluation has been done by visual inspection. In this study, we developed a quantitative STIR method, and tested its ability to identify STIR+ lesions in healthy controls and patients with Facioscapulohumeral muscular dystrophy and compared the results with visual STIR evaluation and quantitative T2 relaxation time mapping. The method was based on pixel-by-pixel histograms of the distribution of signal intensities from muscles. Signal intensities from healthy control muscles were averaged and used to define an upper reference limit. Muscles with >2.5% pixels above the limit were defined as being STIR+. The new method showed agreement with T2 relaxation time mapping in 95% of muscles. The visual STIR method only showed agreement with the quantitative STIR method and T2 relaxation time mapping in 88 and 84%, respectively. STIR sequences are available on most MR scanners and the post-processing used in the new quantitative method can be performed using free software. We therefore believe that the new method can play an important role in identifying STIR+ lesions in patients with neuromuscular diseases.


Assuntos
Edema/diagnóstico por imagem , Imageamento por Ressonância Magnética/métodos , Músculo Esquelético/diagnóstico por imagem , Adulto , Estudos de Casos e Controles , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Músculo Esquelético/patologia , Distrofia Muscular Facioescapuloumeral/diagnóstico por imagem , Distrofia Muscular Facioescapuloumeral/patologia
13.
Acta Otolaryngol ; 139(11): 1024-1029, 2019 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-31498002

RESUMO

Background/objectives: The intraorbital contents are thought to be affected by oedema in the days following a blowout fracture. We posit that this oedema can be detected by Magnetic resonance imaging (MRI) as changes in muscle volume, in muscle cross-sectional area, and in the MRI parameter 'mean grey value' (MGV) of the orbital fat and extraocular muscles (EOMs). Materials and methods: Patients with a blowout fracture underwent an MRI scan within 72 h after the trauma and again after 10-14 days. Measurements of EOMS and fat tissue on the fractured orbit were compared to the unfractured orbit. Results: Eighteen patients were included. Measurements showed significantly larger volume, cross-sectional area and MGV of the EOM closest to the fracture compared to the same muscle in the unfractured orbit. This significance disappeared for some parameters on the second scan. The volume of herniated orbital contents was significantly smaller on the second scan than on the first. Conclusions and significance: Based on the first longitudinal MRI study on patients with blowout fractures, our results indicate post-traumatic oedema in the intraorbital soft tissue which subsides between scans. A watchful waiting period is recommended in the initial post-traumatic days in patients without muscle entrapment.


Assuntos
Edema/diagnóstico por imagem , Músculos Oculomotores/diagnóstico por imagem , Fraturas Orbitárias/diagnóstico por imagem , Adolescente , Adulto , Idoso , Edema/etiologia , Humanos , Imageamento por Ressonância Magnética , Masculino , Pessoa de Meia-Idade , Fraturas Orbitárias/complicações , Adulto Jovem
14.
Sci Rep ; 9(1): 4680, 2019 03 18.
Artigo em Inglês | MEDLINE | ID: mdl-30886222

RESUMO

Spinobulbar muscular atrophy (SBMA) is caused by a trinucleotide repeat expansion in the androgen receptor gene on the X chromosome. There is a toxic effect of the mutant receptor on muscle and neurons resulting in muscle weakness and atrophy. The weakness can be explained by wasting due to loss of muscle cells, but it is unknown whether weakness also relates to poor muscle contractility of the remaining musculature. In this study, we investigated the muscle contractility in SBMA. We used stationary dynamometry and quantitative MRI to assess muscle strength and absolute and fat-free, cross-sectional areas. Specific muscle force (strength per cross-sectional area) and contractility (strength per fat-free cross-sectional area) were compared with healthy controls and their relation to walking distance and disease severity was investigated. Specific force was reduced by 14-49% in SBMA patients compared to healthy controls. Contractility was reduced by 22-39% in elbow flexion, knee extension, ankle dorsi- and plantarflexion in SBMA patients. The contractility decreased with increasing muscle fat content in muscles with affected contractility in SBMA. The decreased muscle contractility in SBMA may relate to motor neuron degeneration and changed fibre type distribution and muscle architecture.


Assuntos
Atrofia Bulboespinal Ligada ao X/fisiopatologia , Neurônios Motores/fisiologia , Contração Muscular/fisiologia , Força Muscular/fisiologia , Músculos/fisiologia , Adulto , Idoso , Idoso de 80 Anos ou mais , Atrofia Bulboespinal Ligada ao X/genética , Cromossomos Humanos X/genética , Estudos Transversais , Feminino , Humanos , Imageamento por Ressonância Magnética , Masculino , Pessoa de Meia-Idade , Dinamômetro de Força Muscular , Debilidade Muscular/genética , Degeneração Neural , Receptores Androgênicos/genética , Expansão das Repetições de Trinucleotídeos/genética
15.
Diagnostics (Basel) ; 9(1)2019 Mar 01.
Artigo em Inglês | MEDLINE | ID: mdl-30823678

RESUMO

We describe the clinical course of a 24-year old male with Crohn's disease in immunosuppressive therapy admitted with a 6-week history of fever, weight loss, night sweat, and general malaise. The patient received extensive workup for a fever of unknown origin and received empiric antibiotics. Workup with Fluorine-18 fluoro-2-deoxy-d-glucose ([18F]FDG) positron-emission tomography (PET/CT), and magnetic resonance imaging (MRI) with intravenous contrast showed multifocal ostitis of the columna and os sacrum, as well as abscesses in m. iliopsoas and m. iliacus and affection of the retroperitoneum, liver, and spleen. Initially, malignancy was suspected, but a subsequent liver biopsy showed necrotizing granulomatous inflammation and a later polymerase chain reaction (PCR) showed Bartonella henselae. The patient had relevant exposure from housecats. He was treated with Doxycycline and Rifampicin for 12 weeks resulting in complete recovery. This case is, to our knowledge, a rare example of disseminated infection with Bartonella henselae visualized on both [18F]FDG-PET/CT and MRI.

16.
J Neurol ; 266(5): 1127-1135, 2019 May.
Artigo em Inglês | MEDLINE | ID: mdl-30778707

RESUMO

OBJECTIVE: Unlike most muscular dystrophies that progress symmetrically at a constant rate, facioscapulohumeral muscular dystrophy (FSHD) is characterized by stepwise, asymmetric progression of muscle wasting, and weakness. Muscle tissue is progressively replaced by fat; however, its relation to preceding inflammation is unclear. In this longitudinal study of FSHD, we assessed muscle inflammation and fat replacement and their relation quantitatively. We also investigated whether fat replacement in muscle varies along its length. METHODS: Forty-five patients with FSHD were evaluated twice, 14 months apart. Using MRI sequences with short TI inversion recovery (STIR), we quantified the degree of STIR hyperintensity in muscles (≥ 2 SD above control intensity). STIR hyperintensities (STIR+) suggest edema or inflammation. We used Dixon MRI to quantify fat content. RESULTS: Of 370 thigh muscles, 83 were STIR+ at baseline and 103 at follow-up. The highest frequency of STIR+ was seen in muscles with inter-mediate fat content (40-60% fat). The progression of fat replacement was higher in STIR+ muscles (5.0 ± 4.0%) vs. STIR- muscles [2.3 ± 3.3% (P < 0.0001)]. In addition, muscles with severe STIR+ at baseline had a higher fat replacement progression than muscles with milder STIR+ (R = 0.39, P = 0.001). The fat content was higher in the distal part vs. proximal part of most muscles (P < 0.05). However, the progression of the fat replacement was uniform along the length of all the muscles. CONCLUSION: Muscles with STIR+, indicating inflammation, have a faster progression of fat replacement than STIR- muscles, and the fat replacement progression correlated with the severity of STIR+.


Assuntos
Gorduras/metabolismo , Imageamento por Ressonância Magnética , Músculo Esquelético/metabolismo , Músculo Esquelético/patologia , Distrofia Muscular Facioescapuloumeral/diagnóstico por imagem , Miosite/diagnóstico por imagem , Progressão da Doença , Feminino , Humanos , Processamento de Imagem Assistida por Computador , Estudos Longitudinais , Masculino , Pessoa de Meia-Idade , Distrofia Muscular Facioescapuloumeral/complicações , Miosite/complicações , Estudos Retrospectivos
17.
Neurology ; 92(6): e548-e559, 2019 02 05.
Artigo em Inglês | MEDLINE | ID: mdl-30610091

RESUMO

OBJECTIVE: To investigate the phenotypic features, with emphasis on muscle, in 40 patients with spinobulbar muscular atrophy (SBMA) using quantitative MRI, stationary dynamometry, questionnaires, and functional tests. METHODS: Patients with genetically confirmed SBMA were included. MRI was used to describe muscle involvement and quantify muscle fat fractions of arm, back, and leg muscles. Muscle strength was assessed with a stationary dynamometer. All patients were evaluated with the SBMA functional rating scale and the 6-minute walk test among others. MRI and muscle strength results were compared with healthy controls. RESULTS: Forty patients with SBMA were included. The muscle fat content was significantly higher in patients with SBMA than in controls: paraspinal fat fraction was 45% vs 33% in controls, thigh fat fraction 36% vs 14%, calf fat fraction 37% vs 15%, upper arm fat fraction 20% vs 8%, and forearm fat fraction was 20% vs 9%. Muscle strength in patients was reduced to approximately half of that in controls in all muscles. Muscle fat content correlated with muscle strength, SBMA functional rating scale score, and 6-minute walk test distance. CONCLUSIONS: Our results show that there is a diffuse muscle involvement pattern in SBMA. Leg muscles are more vulnerable than arm muscles, especially the posterior flexor muscles. The muscle fat content correlates with muscle function and disease severity.


Assuntos
Tecido Adiposo/diagnóstico por imagem , Atrofia Bulboespinal Ligada ao X/diagnóstico por imagem , Força Muscular , Músculo Esquelético/diagnóstico por imagem , Adulto , Idoso , Braço , Músculos do Dorso/diagnóstico por imagem , Músculos do Dorso/fisiopatologia , Atrofia Bulboespinal Ligada ao X/fisiopatologia , Estudos de Casos e Controles , Antebraço , Humanos , Perna (Membro) , Imageamento por Ressonância Magnética , Masculino , Pessoa de Meia-Idade , Músculo Esquelético/fisiopatologia , Fenótipo , Coxa da Perna , Teste de Caminhada
18.
J Nucl Med ; 60(8): 1053-1058, 2019 08.
Artigo em Inglês | MEDLINE | ID: mdl-30683767

RESUMO

Complete resection is the treatment of choice for most pediatric brain tumors, but early postoperative MRI for detection of residual tumor may be misleading because of MRI signal changes caused by the operation. PET imaging with amino acid tracers in adults increases the diagnostic accuracy for brain tumors, but the literature in pediatric neurooncology is limited. A hybrid PET/MRI system is highly beneficial in children, reducing the number of scanning procedures, and this is to our knowledge the first larger study using PET/MRI in pediatric neurooncology. We evaluated if additional postoperative 18F-fluoro-ethyl-tyrosine (18F-FET) PET in children and adolescents would improve diagnostic accuracy for the detection of residual tumor as compared with MRI alone and would assist clinical management. Methods: Twenty-two patients (7 male; mean age, 9.5 y; range, 0-19 y) were included prospectively and consecutively in the study and had 27 early postoperative 18F-FET PET exams performed preferentially in a hybrid PET/MRI system (NCT03402425). Results: Using follow-up (93%) or reoperation (7%) as the reference standard, PET combined with MRI discriminated tumor from treatment effects with a lesion-based sensitivity/specificity/accuracy (95% confidence intervals) of 0.73 (0.50-1.00)/1.00 (0.74-1.00)/0.87 (0.73-1.00) compared with MRI alone: 0.80 (0.57-1.00)/0.75 (0.53-0.94)/0.77 (0.65-0.90); that is, the specificity for PET/MRI was 1.00 as compared with 0.75 for MRI alone (P = 0.13). In 11 of 27 cases (41%), results from the 18F-FET PET scans added relevant clinical information, including one scan that directly influenced clinical management because an additional residual tumor site was identified. 18F-FET uptake in reactive changes was frequent (52%), but correct interpretation was possible in all cases. Conclusion: The high specificity for detecting residual tumor suggests that supplementary 18F-FET PET is relevant in cases where reoperation for residual tumor is considered.


Assuntos
Neoplasias Encefálicas/diagnóstico por imagem , Imageamento por Ressonância Magnética , Tomografia por Emissão de Pósitrons , Neoplasias da Medula Espinal/diagnóstico por imagem , Adolescente , Astrocitoma/diagnóstico por imagem , Encéfalo/diagnóstico por imagem , Neoplasias Encefálicas/cirurgia , Criança , Pré-Escolar , Feminino , Fluordesoxiglucose F18 , Seguimentos , Glioma/diagnóstico por imagem , Humanos , Lactente , Recém-Nascido , Masculino , Imagem Multimodal , Neoplasia Residual/diagnóstico por imagem , Pediatria , Período Pós-Operatório , Estudos Prospectivos , Reoperação , Reprodutibilidade dos Testes , Tumor Rabdoide/diagnóstico por imagem , Sensibilidade e Especificidade , Neoplasias da Medula Espinal/cirurgia , Teratoma/diagnóstico por imagem , Tomografia Computadorizada por Raios X , Adulto Jovem
19.
J Clin Endocrinol Metab ; 103(9): 3394-3404, 2018 09 01.
Artigo em Inglês | MEDLINE | ID: mdl-29889238

RESUMO

Context: Menopause is associated with an increased incidence of insulin resistance and diabetes. Objective: The aim of this study was to explore the lipid deposition in liver and skeletal muscle and investigate the association with insulin sensitivity in postmenopausal and premenopausal women. Design and Setting: Single-center cross-sectional study of 55 healthy women between 45 and 60 years of age. We measured lipid deposition in the liver with magnetic resonance spectroscopy, intramuscular and intra-abdominal lipid deposition with MRI, body composition with a dual-energy X-ray absorptiometry scan, and insulin sensitivity with the composite Matsuda Index. Outcome Measures: We studied the association between fat distribution, ectopic lipid deposition, and insulin sensitivity in pre- and postmenopausal women. Results: Postmenopausal women had an increased lipid deposition in the liver [0.68% (0.44 to 0.99) vs 0.49% (0.38 to 0.64), P = 0.01] and skeletal muscle [3% (2 to 4) vs 2% (1 to 3), P = 0.001] and had a 28% lower Matsuda insulin sensitivity index during an oral glucose tolerance test (6.31 ± 3.48 vs 8.78 ± 4.67, P = 0.05) compared with premenopausal women. Total fat mass and leg fat mass were stronger predictors of ectopic lipid deposition, and visceral fat mass was a stronger predictor of both ectopic lipid deposition and insulin resistance in postmenopausal women compared with premenopausal women. Conclusions: For a given subcutaneous and visceral fat depot size, postmenopausal women show increased ectopic lipid deposition and insulin resistance compared with premenopausal women. It is suggested that lipid deposition in liver and skeletal muscle may represent important mechanistic links between the changes in fat depots and the increased incidence of insulin resistance seen after menopause.


Assuntos
Resistência à Insulina/fisiologia , Metabolismo dos Lipídeos/fisiologia , Lipidoses/metabolismo , Pós-Menopausa/metabolismo , Absorciometria de Fóton , Composição Corporal , Estudos Transversais , Feminino , Teste de Tolerância a Glucose , Humanos , Gordura Intra-Abdominal/patologia , Fígado/metabolismo , Imageamento por Ressonância Magnética , Pessoa de Meia-Idade , Músculo Esquelético/metabolismo , Pré-Menopausa
20.
Ultrasound Med Biol ; 44(8): 1751-1761, 2018 08.
Artigo em Inglês | MEDLINE | ID: mdl-29804906

RESUMO

Magnetic resonance phase contrast angiography (MRA) is the gold standard for blood flow evaluation. Spectral Doppler ultrasound (SDU) is the first clinical choice, although the method is angle dependent. Vector flow imaging (VFI) is an angle-independent ultrasound method. The aim of the study was to compare VFI- and SDU-estimated peak systolic velocities (PSV) of the common carotid artery (CCA) with PSV obtained by MRA. Furthermore, intra- and inter-observer agreement was determined. MRA estimates were significantly different from SDU estimates (left CCA: p < 0.001, right CCA: p < 0.001), but not from VFI estimates (left CCA: p = 0.28, right CCA: p = 0.18). VFI measured lower PSV in both CCAs compared with SDU (p < 0.001) with improved precision (VFI: left: 24%, right: 18%; SDU: left 38%, right: 23%). Intra- and inter-observer agreement was almost perfect for VFI and SDU (inter-observer correlation coefficient: VFI 0.88, SDU 0.91; intra-observer correlation coefficient: VFI 0.96, SDU 0.97). VFI is more accurate than SDU in evaluating PSV compared with MRA.


Assuntos
Artéria Carótida Primitiva/fisiologia , Angiografia por Ressonância Magnética/métodos , Ultrassonografia Doppler/métodos , Adulto , Velocidade do Fluxo Sanguíneo , Artéria Carótida Primitiva/diagnóstico por imagem , Feminino , Hemodinâmica , Humanos , Masculino , Pessoa de Meia-Idade , Valores de Referência
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