RESUMEN
BACKGROUND: Respiratory complications resulting from motor neurons degeneration are the primary cause of death in amyotrophic lateral sclerosis (ALS). Predicting the need for non-invasive ventilation (NIV) in ALS is important for advance care planning and clinical trial design. The aim of this study was to assess the potential of quantitative MRI at the brainstem and spinal cord levels to predict the need for NIV during the first six months after diagnosis. METHODS: Forty-one ALS patients underwent MRI and spirometry shortly after diagnosis. The need for NIV was monitored according to French health guidelines for 6 months. The performance of four regression models based on: clinical variables, brainstem structures volumes, cervical spinal measurements, and combined variables were compared to predict the need for NIV within this period. RESULTS: Both the clinical model (R2 = 0.28, AUC = 0.85, AICc = 42.67, BIC = 49.8) and the brainstem structures' volumes model (R2 = 0.30, AUC = 0.85, AICc = 40.13, BIC = 46.99) demonstrated good predictive performance. In addition, cervical spinal cord measurements model similar performance (R2 = 0.338, AUC = 0.87, AICc = 37.99, BIC = 44.49). Notably, the combined model incorporating predictors from all three models yielded the best performance (R2 = 0.60, AUC = 0.959, AICc = 36.38, BIC = 44.8). These findings are supported by observed positive correlations between brainstem volumes, cervical (C4/C7) cross-sectional area, and spirometry-measured lung volumes. CONCLUSIONS: Our study shows that brainstem volumes and spinal cord area are promising measures to predict respiratory intervention needs in ALS.
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Esclerosis Amiotrófica Lateral , Ventilación no Invasiva , Humanos , Esclerosis Amiotrófica Lateral/diagnóstico por imagen , Esclerosis Amiotrófica Lateral/terapia , Esclerosis Amiotrófica Lateral/complicaciones , Ventilación no Invasiva/métodos , Progresión de la Enfermedad , Imagen por Resonancia Magnética/métodos , Tronco Encefálico/diagnóstico por imagenRESUMEN
BACKGROUND: Motor capacity is crucial in amyotrophic lateral sclerosis (ALS) clinical trial design and patient care. However, few studies have explored the potential of multimodal MRI to predict motor capacity in ALS. This study aims to evaluate the predictive value of cervical spinal cord MRI parameters for motor capacity in ALS compared to clinical prognostic factors. METHODS: Spinal multimodal MRI was performed shortly after diagnosis in 41 ALS patients and 12 healthy participants as part of a prospective multicenter cohort study, the PULSE study (NCT00002013-A00969-36). Motor capacity was assessed using ALSFRS-R scores. Multiple stepwise linear regression models were constructed to predict motor capacity at 3 and 6 months from diagnosis, based on clinical variables, structural MRI measurements, including spinal cord cross-sectional area (CSA), anterior-posterior, and left-to-right cross-section diameters at vertebral levels from C1 to T4, and diffusion parameters in the lateral corticospinal tracts (LCSTs) and dorsal columns. RESULTS: Structural MRI measurements were significantly correlated with the ALSFRS-R score and its sub-scores. And as early as 3 months from diagnosis, structural MRI measurements fit the best multiple linear regression model to predict the total ALSFRS-R (R2 = 0.70, p value = 0.0001) and arm sub-score (R2 = 0.69, p value = 0.0002), and combined with DTI metric in the LCST and clinical factors fit the best multiple linear regression model to predict leg sub-score (R2 = 0.73, p value = 0.0002). CONCLUSIONS: Spinal multimodal MRI could be promising as a tool to enhance prognostic accuracy and serve as a motor function proxy in ALS.
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Esclerosis Amiotrófica Lateral , Humanos , Esclerosis Amiotrófica Lateral/diagnóstico , Estudios de Cohortes , Estudios Prospectivos , Imagen por Resonancia Magnética/métodos , Tractos PiramidalesRESUMEN
The segmentation from MRI of macroscopically ill-defined and highly variable structures, such as the hippocampus (Hc) and the amygdala (Am), requires the use of specific constraints. Here, we describe and evaluate a fast fully automatic hybrid segmentation that uses knowledge derived from probabilistic atlases and anatomical landmarks, adapted from a semi-automatic method. The algorithm was designed at the outset for application on images from healthy subjects and patients with hippocampal sclerosis. Probabilistic atlases were built from 16 healthy subjects, registered using SPM5. Local mismatch in the atlas registration step was automatically detected and corrected. Quantitative evaluation with respect to manual segmentations was performed on the 16 young subjects, with a leave-one-out strategy, a mixed cohort of 8 controls and 15 patients with epilepsy with variable degrees of hippocampal sclerosis, and 8 healthy subjects acquired on a 3 T scanner. Seven performance indices were computed, among which error on volumes RV and Dice overlap K. The method proved to be fast, robust and accurate. For Hc, results with the new method were: 16 young subjects {RV=5%, K=87%}; mixed cohort {RV=8%, K=84%}; 3 T cohort {RV=9%, K=85%}. Results were better than with atlas-based (thresholded probability map) or semi-automatic segmentations. Atlas mismatch detection and correction proved efficient for the most sclerotic Hc. For Am, results were: 16 young controls {RV=7%, K=85%}; mixed cohort {RV=19%, K=78%}; 3 T cohort {RV=10%, K=77%}. Results were better than with the semi-automatic segmentation, and were also better than atlas-based segmentations for the 16 young subjects.
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Amígdala del Cerebelo/anatomía & histología , Inteligencia Artificial , Hipocampo/anatomía & histología , Interpretación de Imagen Asistida por Computador/métodos , Imagenología Tridimensional/métodos , Imagen por Resonancia Magnética/métodos , Reconocimiento de Normas Patrones Automatizadas/métodos , Adulto , Algoritmos , Femenino , Humanos , Aumento de la Imagen/métodos , Masculino , Reproducibilidad de los Resultados , Sensibilidad y EspecificidadRESUMEN
OBJECTIVES: Since physical activity (PA) has demonstrated benefits for cardiovascular health, it is possible to hypothesize that higher or increasing PA slows the progression of white matter hyperintensities (WMH). We investigated the association between PA and the progression of WMH in non-demented older adults with memory complaints. DESIGN: We included 152 participants (mean age 74.7±3.8 years; 63.8% women) in the analyses, in whom information on self-reported PA and MRI was available at both baseline and 3-year follow-up. From the PA questionnaire, the baseline metabolic equivalent of task (MET-minute/week) and changes in MET-minute/week over three years were separately calculated for overall, leisure-time, and non-leisure time PA. WMH volume at baseline and 3-year follow-up was obtained by using an automated segmentation algorithm. RESULTS: Mixed-effect linear regression models showed that none of the baseline PA variables was associated with progression of WMH over time. People who had decreased their PA levels over three years tended to show greater progression of WMH compared with those who had maintained PA levels of ≥1200 MET-min/week (roughly equivalent to ≥300 minutes of brisk walking) in the unadjusted model (ß±SE=4.85±2.42, p=0.045); however, this association was no longer significant after adjustment for confounders (ß±SE =3.63±2.18, p=0.096). CONCLUSIONS: We did not find any significant association between PA and WMH in non-demented older adults with memory complaints. However, decrease over time in PA levels tended to be associated with progression of WMH. A larger longitudinal study with data on PA assessed using objective measures would provide important information in this field.
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Actividades Cotidianas , Cognición/fisiología , Ejercicio Físico , Trastornos de la Memoria/fisiopatología , Caminata , Sustancia Blanca/fisiología , Anciano , Progresión de la Enfermedad , Femenino , Humanos , Estudios Longitudinales , Imagen por Resonancia Magnética , MasculinoRESUMEN
OBJECTIVES: The association between circulating biomarkers of red blood cells (RBC) omega-3 polyunsaturated fatty acids (PUFAs) and cerebral white matter hyperintensities (WMH) on the brain MRI remains yet unclear. We investigated the cross-sectional and prospective associations of RBC omega-3 PUFAs with WMH in dementia-free older adults with subjective memory complaints. DESIGN: Participants were 234 older adults with assessments for both PUFA and MRI near to baseline; among them, 79 also had an MRI assessment at 3-year follow-up. The measurement of WMH volume was obtained by an automated segmentation algorithm. We related individual or combinational baseline RBC omega-3 PUFAs levels with baseline WMH volumes and WMH evolution over 3 years. We carried out multiple (cross-sectional) and mixed-effect (prospective analysis, with random effects at participant's level) linear regressions with adjustment for age, sex, time interval between date of blood draw for measurement of fatty acids and date of brain MRI, the status of APOE e4 carrier, body mass index, and vascular risk factors. Associations were considered significant at p ≤ 0.006 to take into account multiplicity (8 comparisons). RESULTS: None of the eight RBC omega-3 PUFAs tested was significantly associated with WMH at both cross-sectional and prospective analyses. CONCLUSIONS: We did not find any association between omega-3 PUFAs and WMH in non-demented older adults with memory complaints. A longer longitudinal study with data on omega-3 PUFAs and WMH would contribute important information to this field.
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Eritrocitos/metabolismo , Ácidos Grasos Omega-3/uso terapéutico , Sustancia Blanca/irrigación sanguínea , Anciano , Estudios Transversales , Ácidos Grasos Omega-3/administración & dosificación , Ácidos Grasos Omega-3/farmacología , Femenino , Humanos , Estudios Longitudinales , MasculinoRESUMEN
The major etiologies of primary aldosteronism are aldosterone-producing adrenal adenoma, requiring a surgical treatment, and bilateral hyperplasia, usually managed with medical therapy. We only report a retrospective study on 22 patients with primary aldosteronism diagnosed by clinical and usual biochemical tests. All the patients were explored by computed tomography scan (CT) and iodomethyl-norcholesterol scintigraphy. The purpose of this study was to compare the capacity of the two methods to differentiate adrenal adenoma and hyperplasia. The CT scan was more sensitive (sensitivity: 88%) compare to scintigraphy (sensitivity: 64%) for the diagnosis of adrenal adenoma. However, the scintigraphy was a useful tool to detect asymmetric macronodular adrenal hyperplasia. Therefore, the catheterisation of adrenal venous would be only necessary for a few cases. We conclude from this study and the literature review that CT scan and iodomethyl-norcholesterol scintigraphy are complementary and both useful to increase diagnostic reliability of primary aldosteronism.
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19-Yodocolesterol/análogos & derivados , Glándulas Suprarrenales/patología , Hiperaldosteronismo/etiología , Radioisótopos de Yodo , Tomografía Computarizada por Rayos X , Adenoma/diagnóstico , Adenoma/diagnóstico por imagen , Neoplasias de las Glándulas Suprarrenales/diagnóstico , Neoplasias de las Glándulas Suprarrenales/diagnóstico por imagen , Glándulas Suprarrenales/diagnóstico por imagen , Adulto , Anciano , Diagnóstico Diferencial , Femenino , Humanos , Hiperaldosteronismo/diagnóstico por imagen , Hiperplasia , Masculino , Persona de Mediana Edad , Cintigrafía , Estudios RetrospectivosRESUMEN
The authors report a case of breast metastasis from a "tall cell" variant of papillary thyroid carcinoma in a 59-year-old woman. This metastasis was discovered two months after the diagnosis of an inextirpable thyroid tumor. Tall cell variant of papillary thyroid carcinoma is a sub-type of papillary carcinoma with an aggressive course and frequent metastases (in patients over 50 years of age). No mammary metastasis of this tumor type has been reported so far.
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Neoplasias de la Mama/secundario , Carcinoma Papilar/secundario , Neoplasias de la Tiroides/patología , Femenino , Humanos , Persona de Mediana Edad , PronósticoRESUMEN
The authors distinguish among the hormonal and metabolic disturbances found in Anorexia nervosa those, numerous, which are the results of emaciation only, and others which could be signs of central neuro-chemical dysregulation. The disturbances of the gonadotropic function and of the control of the "hunger-fattening" phenomena are probably of the latter type; however the nature of those dysfunctions and of their corresponding neuro-mediators remain so far hypothetical.
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Anorexia Nerviosa/fisiopatología , Enfermedades del Sistema Endocrino/etiología , Enfermedades Metabólicas/etiología , Amenorrea/etiología , Anorexia Nerviosa/metabolismo , Peso Corporal , Femenino , Gonadotropinas Hipofisarias/metabolismo , Hormonas/metabolismo , Humanos , Hipogonadismo/etiología , Enfermedades Hipotalámicas/etiología , Hipotálamo/fisiopatología , Islotes Pancreáticos/fisiología , Neurotransmisores/fisiologíaRESUMEN
Plasma estradiol, estriol, progesterone, H.C.S. and cortisol were measured (RIA methods) every two days, in 20 diabetic pregnancies (classes B, C or D of White), between the 33th week and the delivery, and were compared with normal pregnancies. Estriol was always normal, and progesterone values were at the upper limit of the normal range; estradiol, H.C.S. and cortisol were normal or elevated, with mean values significantly higher than controls. E2 and/or progesterone dramatically decreased in 5 cases before birth, just as the urinary measurements. In this study, the insulin requirements were not correlated with these plasma hormone values. Besides pathogenic hypothesis )glycemic control of H.C.S. and cortisol--abnormalities of metabolic pathways of estrogens ?), this work informs the inferred hormonal deficiency in diabetic placenta. Also, it is unable to determine the practical usefulness of these plasma measurements in the management of diabetic pregnancy.
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Hormonas/sangre , Embarazo en Diabéticas/sangre , Adulto , Estradiol/sangre , Estriol/sangre , Femenino , Humanos , Hidrocortisona/sangre , Lactógeno Placentario/sangre , Embarazo , Tercer Trimestre del Embarazo , Progesterona/sangreRESUMEN
Observations of two patients illustrating the problem of Shulman syndroma. The first had presented an oedema of the four limbs, without injury of the trunk, but with myalgias, histological fasciitis, eosinophilia and hypergammaglobulinemia. In spite of corticotherapy, evolution toward a generalized morphea. The second has presented the same oedema of the four limbs, with myalgias, but also Raynaud syndroma, histological fasciitis and eosinophilia. Evolution toward acrosclerosis in spite of corticotherapy. For the authors, Shulman's syndroma is apprehended as a particular clinical form of scleroderma.
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Eosinofilia/patología , Fascitis/patología , Esclerodermia Sistémica/patología , Anciano , Edema/etiología , Femenino , Humanos , Esclerosis , Piel/patología , SíndromeRESUMEN
OBJECTIVE: The Multidomain Alzheimer Preventive Trial (MAPT study) was designed to assess the efficacy of isolated supplementation with omega-3 fatty acid, an isolated multidomain intervention (consisting of nutritional counseling, physical exercise, cognitive stimulation) or a combination of the two interventions on the change of cognitive functions in frail subjects aged 70 years and older for a period of 3 years. Ancillary neuroimaging studies were additionally implemented to evaluate the impact of interventions on cerebral metabolism (FDG PET scans) and atrophy rate (MRIs), as well as brain amyloïd deposit (AV45 PET scans). DESIGN PATIENTS: 1680 subjects (mean age: 75.3 years; female: 64.8 %), enrolled by 13 memory clinics, were randomized into one of the following four groups: omega-3 supplementation alone, multidomain intervention alone, omega-3 plus multidomain intervention, or placebo. Participants underwent cognitive, functional and biological assessments at M6, M12, M24 and M36 visits. The primary endpoint is a change of memory function at 3 years, as assessed by the Free and Cued Selective Reminding test. All participants will be followed for 2 additional years after the 3-years intervention (MAPT PLUS extension study). INTERVENTIONS: 1/Omega-3 supplementation: two soft capsules daily as a single dose, containing a total of 400 mg docosahexaenoic acid (DHA), i.e., 800 mg docosahexaenoic acid per day, for 3 years. 2/ Multidomain intervention: collective training sessions conducted in small groups (6-8 participants) in twelve 120-minute sessions over the first 2 months (two sessions a week for the first month, and one session a week the second month) then a 60-minute session per month in the following three areas: nutrition, physical activity, and cognition until the end of the 3 years. In addition to the collective sessions, individualized preventive outpatient visits exploring possible risk factors for cognitive decline are performed at baseline, M12 and M24. BASELINE POPULATION: For cognition, the mean MMSE at baseline was 28.1 (± 1.6). About 58% and 42% of participants had a CDR score equal to 0 and 0.5, respectively. Regarding mobility status, 200 (11.9%) had a 4-m gait speed lower or equal to 0.8 m/s. According to the Fried criteria, 673 (42.1%) participants were considered pre frail, and 51 (3.2%) frail. The red blood cell DHA content was 26.1 ± 8.1 µg/g. Five hundred and three participants underwent baseline MRI. AV45 PET scans were performed in 271 individuals and preliminary results showed that 38.0% had a cortical SUVR > 1.17, which gave an indication of significant brain amyloïd deposit. DISCUSSION: The MAPT trial is presently the first largest and longest multidomain preventive trial relevant to cognitive decline in older adults with subjective memory complaints. The multidomain intervention designed for the MAPT trial is likely to be easily implemented within the general population.
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Glucosa/metabolismo , Hepatitis Viral Humana/metabolismo , Enfermedad Aguda , Adolescente , Adulto , Convalecencia , Femenino , Prueba de Tolerancia a la Glucosa , Hormona del Crecimiento/sangre , Hepatitis Viral Humana/complicaciones , Humanos , Hidrocortisona/sangre , Hiperinsulinismo/complicaciones , Insulina/sangre , Resistencia a la Insulina , Lípidos/sangre , Masculino , Persona de Mediana EdadAsunto(s)
Glándulas Suprarrenales/fisiopatología , Colesterol/análogos & derivados , Síndrome de Cushing/diagnóstico , Glándulas Suprarrenales/patología , Hormona Adrenocorticotrópica/fisiología , Síndrome de Cushing/fisiopatología , Humanos , Hiperplasia/fisiopatología , Radioisótopos de Yodo , CintigrafíaAsunto(s)
Metabolismo de los Hidratos de Carbono , Hepatopatías/metabolismo , Péptido C/metabolismo , Prueba de Tolerancia a la Glucosa , Hepatitis Viral Humana/metabolismo , Humanos , Hipoglucemia/etiología , Insulina/sangre , Resistencia a la Insulina , Cirrosis Hepática/metabolismo , Hepatopatías/complicacionesRESUMEN
For further evaluation of B-cell secretion in diabetic keto-acidosis (KA) and in non-ketotic hyperosmolar coma (NKHC), basal and post-i.v. tolbutamide blood CPR and IRI values were measured in 34 patients (22 KA and 12 NKHC). FFA, cortisol and HGH measurements were also performed. IRI was low in both KA and NKHC (0.07 +/- 0.01 and 0.082 +/- 0.01 nmol/l) as opposed to CPR which was significantly higher in NKHC (1.14 +/- 0.1 nmol/l) than in KA (0.21 +/- 0.03 nmol/l). After tolbutamide injection, CPR and IRI levels did not change in any of the KA cases, whereas they significantly increased in half of the NKHC cases. Cortisol and FFA values were similarly increased in both situations, as opposed to HGH which was significantly higher (6.1 +/- 1.2 ng/ml) in KA than in NKHC (1.9 +/- 0.2 ng/ml). These results suggest that B-cell function is less deficient in NKHC than in KA. Residual insulin amounts reaching the liver via the portal vein could partly account for the absence of ketosis in NKHC.
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Péptido C/sangre , Coma Diabético/sangre , Cetoacidosis Diabética/sangre , Coma Hiperglucémico Hiperosmolar no Cetósico/sangre , Insulina/sangre , Péptidos/sangre , Adulto , Anciano , Ácidos Grasos no Esterificados/sangre , Femenino , Hormona del Crecimiento/sangre , Humanos , Hidrocortisona/sangre , Masculino , Persona de Mediana Edad , TolbutamidaRESUMEN
Report of a case of metastatic adrenal cortical carcinoma with Cushing's syndrome treated with OP'DDD, 15 g per day. This therapy was followed by the regression of the adrenal tumour and of its metastases since three years.
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Neoplasias de la Corteza Suprarrenal/tratamiento farmacológico , Carcinoma/tratamiento farmacológico , Mitotano/uso terapéutico , Neoplasias de la Corteza Suprarrenal/complicaciones , Carcinoma/complicaciones , Síndrome de Cushing/complicaciones , Femenino , Humanos , Neoplasias Hepáticas/secundario , Persona de Mediana Edad , Metástasis de la NeoplasiaRESUMEN
On the basis of 7 cases representing the essential types of aetiology of Cushing's syndrome, it is possible to define the value, limitations and role of adrenal scintigraphy using 131I-19-iodocholesterol in the diagnostic investigation of hypercortisolism. The essential value of the investigation is morphological rather than functional drome. Thus adrenal scintigraphy has the same role in diagnostic investigation as various radiological procedures such as pneumoperitoneum and angiography. The latter, however, are technically difficult and fraught with risk, in contrast to the isotopic examination. The principle limitations of the method are not dosimetric but lie in the relative difficulty of interpretation of the documents obtained.
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Síndrome de Cushing/diagnóstico , Neoplasias de las Glándulas Suprarrenales/diagnóstico , Colesterol/análogos & derivados , Síndrome de Cushing/etiología , Humanos , Hiperplasia/diagnóstico , Radioisótopos de Yodo , Lesiones Precancerosas/diagnóstico , Cintigrafía/métodosRESUMEN
Thirty-six male patients with idiopathic haemochromatosis were subjected to measurements of basal plasma values of testosterone, LH and FSH and to an LRH test. Nineteen were also subjected to basal plasma determinations of T3, T4, cortisol, TSH and prolactin and to a TRH test. In 11 cases GH values were measured before, during and after an arginine infusion. Seventeen patients were found to hae low levels of testosterone, LH and FSH, and no gonadotrophin responses to LRH. Seventeen others had normal levels of these three hormones, with normal responses to LRH. The two remaining patients had normal testosterone values but very increased gonadotrophin values: a fact which remains unexplained. Basal levels of prolactin, GH, T3, T4, and TSH were normal: cortisol levels were either normal or increased in cases of poorly controlled diabetes. Prolactin responses to TRH were always normal. TSH responses to TRH were impaired in 2 cases, and GH responses to arginine in 3 cases. Considering that other factors may be involved in the few impairments found in TSH and GH stimulations, it is concluded that the only indisputable pituitary insufficiency in about half of the cases of idiopathic haemochromatosis is gonadotrophic.