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1.
J Dairy Sci ; 2024 Jun 20.
Artículo en Inglés | MEDLINE | ID: mdl-38908695

RESUMEN

Body-condition dynamics are known to affect the different steps of reproduction in cattle (cyclicity, estrus expression, fertilization, embryo development). This has led to a widespread idea that there is an ideal-target optimal body condition, but no clear profile has yet been identified. Here we investigated the relationships between body condition score (BCS) profiles and reproductive performance in dairy cows. Data were from Holstein or Normande herds in 6 French experimental farms. In the Holstein breed, we discriminated 4 BCS profiles based on combining BCS at calving (Low: around 2.6 points, or High: around 3.3 points) with BCS loss after calving (Moderate (M): ≤ 1.0 points, or Severe (S): > 1.0 points). The Low-M profile mostly included multiparous cows with higher milk yield and lower reproductive performance than cows in the 3 other profiles. Low-M cows that experienced abnormal ovarian activity had lower reproductive performance than their profile-mates. Moreover, 67% of Low-M cows kept the same profile at the following lactation. The High-S profile mostly included primiparous cows with lower milk yield and higher reproductive performance than cows in other profiles. In High-S cows, higher milk yields correlated to higher risk of failure to calf on first insemination. Moreover, 38% of High-S cows kept the same profile at the following lactation, and none changed to Low-M. The other 2 BCS profiles (Low-S and High-M) were intermediate in terms of milk yield and reproductive performance. In Normande, we discriminated 3 BCS profiles based on combining BCS at calving (Low: around 2.6 points, or High: around 3.5 points) with BCS loss after calving (Flat (F): flat with no loss, Moderate (M): around 0.5 points, or Severe (S): around 1.0 point). The Low-M and High-S profiles included cows with similar performance, even though High-S-profile cows showed better but not significantly different milk yield and reproduction performance. The High-F profile included cows that were more likely to experience abnormal ovarian activity and fail at first insemination than cows in other profiles. More than 50% of Normande cows with 2 successive lactations kept in the same BCS profile at the next lactation. Even though a low BCS at calving combined with severe BCS loss (more than 1 point) after calving was found to increase reproductive failure, there was no evidence of an optimal BCS profile for reproduction in dairy cows, and reproductive success or failure is multifactorial.

2.
Rev Neurol (Paris) ; 2024 Jun 11.
Artículo en Inglés | MEDLINE | ID: mdl-38866655

RESUMEN

BACKGROUND: The association between the pattern of cortical thickness (CT) and executive dysfunction (ED) in mild cognitive impairment (MCI) and subjective cognitive complaints (SCC) is still poorly understood. We aimed to investigate the association between CT and ED in a large French cohort (MEMENTO) of 2323 participants with MCI or SCC. METHODS: All participants with available CT and executive function data (verbal fluency and Trail Making Test [TMT]) were selected (n=1924). Linear regressions were performed to determine relationships between executive performance and the brain parenchymal fraction (BPF) and CT using FreeSurfer. RESULTS: The global executive function score was related to the BPF (sß: 0.091, P<0.001) and CT in the right supramarginal (sß: 0.060, P=0.041) and right isthmus cingulate (sß: 0.062, P=0.011) regions. Literal verbal fluency was related to the BPF (sß: 0.125, P<0.001) and CT in the left parsorbitalis region (sß: 0.045, P=0.045). Semantic verbal fluency was related to the BPF (sß: 0.101, P<0.001) and CT in the right supramarginal region (sß: 0.061, P=0.042). The time difference between the TMT parts B and A was related to the BPF (sß: 0.048, P=0.045) and CT in the right precuneus (sß: 0.073, P=0.019) and right isthmus cingulate region (sß: 0.054, P=0.032). CONCLUSIONS: In a large clinically based cohort of participants presenting with either MCI or SCC (a potential early stage of Alzheimer's disease [AD]), ED was related to the BPF and CT in the left pars orbitalis, right precuneus, right supramarginal, and right isthmus cingulate regions. This pattern of lesions adds knowledge to the conventional anatomy of ED and could contribute to the early diagnosis of AD.

3.
Encephale ; 50(2): 137-142, 2024 Apr.
Artículo en Inglés | MEDLINE | ID: mdl-37005193

RESUMEN

INTRODUCTION: Dopamine (DA) is likely to be involved in some depressive dimensions, such as anhedonia and amotivation, which account for a part of treatment-resistant forms. Monoamine oxidase inhibitors (MAOI) and direct D2 and D3 receptors agonists (D2/3r-dAG) are known to help, but we lack safety data about their combined usage. We report on safety and tolerance of the MAOI+D2r-dAG combination in a clinical series. METHOD: All patients referred to our recourse center for depression between 2013 and 2021 were screened to select those who did receive the combo. Data were extracted from clinical files. RESULTS: Sixteen patients of 60±17 years of age (8 women, 7 with age>65years, all suffered from treatment resistant depression, 7 with bipolar disorder) received the combo. There were no life-threatening adverse effects (AE). However, AE were reported by 14 patients (88%) most of which were mild and consisted of insomnia, nausea, nervousness, confusion, impulse control disorder and/or "sleep attacks". One patient presented a serious AE requiring a short hospitalization for confusion. Intolerance led to failure to introduce treatment in two patients (13%). The retrospective non-interventional design, the variety of molecules, and the modest sample size limited the scope of these results. CONCLUSION: There was no life-threatening safety issue in combining MAOI and D2/3r-dAG, especially regarding cardiovascular side effects. The systematic screening of AE might account for their frequency, but these precluded the treatment in only two patients. Comparative studies are needed to assess the efficacy of this new combination.


Asunto(s)
Trastorno Bipolar , Inhibidores de la Monoaminooxidasa , Humanos , Femenino , Anciano , Inhibidores de la Monoaminooxidasa/efectos adversos , Agonistas de Dopamina/efectos adversos , Depresión , Estudios Retrospectivos , Trastorno Bipolar/tratamiento farmacológico , Trastorno Bipolar/inducido químicamente
4.
J Asthma ; 60(6): 1162-1170, 2023 06.
Artículo en Inglés | MEDLINE | ID: mdl-36301080

RESUMEN

INTRODUCTION: Severe eosinophilic asthma (SEA) is associated with multiple exacerbations. Fractional exhaled nitric oxide (FeNO), a biomarker of airway T2 inflammation, is known to be correlated with the risk of exacerbations. While the use of FeNO is well established to predict the therapeutic response to dupilumab (anti-IL-4/IL-13), it remains uncertain for biologics targeting the IL-5 pathway. METHODS: We conducted an observational, retrospective, monocentric analysis of adults with SEA who started mepolizumab (anti-IL-5) or benralizumab (anti-IL-5R) between January 1, 2016 and December 31, 2020. RESULTS: Data were collected for 109 patients. All participants reported uncontrolled asthma with a median of 3 annual exacerbations and a median Asthma Control Test score of 12. They all had an initial blood eosinophilia >300/mm3, with a median at 610/mm3 (IQR 420-856). Patients with a baseline FeNO ≥50 ppb reported more exacerbations in the previous year than those with a FeNO <50 ppb (p = 0.02). After initiation of treatment, change in FeNO was not associated with therapeutic response. However, decrease in the annual number of exacerbations was significantly greater in patients with a baseline FeNO ≥50 ppb than in those with a baseline FeNO <50 ppb (-3.3 ± 2.7 vs -0.9 ± 2.4, respectively; p = 0.01). There was no association between baseline FeNO values and subsequent lung function, asthma control or reduction of oral corticosteroids use. CONCLUSION: In this real-world cohort, adults with SEA who had a baseline FeNO ≥50 ppb experienced a greater decrease in exacerbations after 12 months of anti-IL-5 or IL-5R biologics than those with a FeNO <50 ppb.


Asunto(s)
Asma , Productos Biológicos , Eosinofilia Pulmonar , Humanos , Adulto , Prueba de Óxido Nítrico Exhalado Fraccionado , Productos Biológicos/uso terapéutico , Estudios Retrospectivos , Óxido Nítrico/metabolismo , Eosinofilia Pulmonar/diagnóstico , Eosinofilia Pulmonar/tratamiento farmacológico
5.
Rev Neurol (Paris) ; 178(5): 472-483, 2022 May.
Artículo en Inglés | MEDLINE | ID: mdl-35491246

RESUMEN

Dementia with Lewy Bodies (DLB) is a common form of cognitive neurodegenerative disease. More than half of the patients affected are not or misdiagnosed because of the clinical similarity with Alzheimer's disease (AD), Parkinson's disease but also psychiatric diseases such as depression or psychosis. In this review, we evaluate the interest of different biomarkers in the diagnostic process: cerebrospinal fluid (CSF), brain MRI, FP-CIT SPECT, MIBG SPECT, perfusion SPECT, FDG-PET by focusing more specifically on differential diagnosis between DLB and AD. FP-CIT SPECT is of high interest to discriminate DLB and AD, but not at the prodromal stage. Brain MRI has shown differences in group study with lower grey matter concentration of the Insula in prodromal DLB, but its interest in clinical routine is not demonstrated. Among the AD biomarkers (t-Tau, phospho-Tau181, Aß42 and Aß40) used routinely, t-Tau and phospho-Tau181 have shown excellent discrimination whatever the clinical stages severity. CSF Alpha-synuclein assay in the CSF has also an interest in the discrimination between DLB and AD but not in segregation between DLB and healthy elderly subjects. CSF synuclein RT-QuIC seems to be an excellent biomarker but its application in clinical routine remains to be demonstrated, given the non-automation of the process.


Asunto(s)
Enfermedad de Alzheimer , Enfermedad por Cuerpos de Lewy , Enfermedades Neurodegenerativas , Anciano , Enfermedad de Alzheimer/diagnóstico por imagen , Péptidos beta-Amiloides/líquido cefalorraquídeo , Biomarcadores , Diagnóstico Diferencial , Humanos , Enfermedad por Cuerpos de Lewy/diagnóstico por imagen , Enfermedades Neurodegenerativas/diagnóstico , Proteínas tau
6.
BMC Pulm Med ; 21(1): 425, 2021 Dec 24.
Artículo en Inglés | MEDLINE | ID: mdl-34952578

RESUMEN

BACKGROUND: Allergic bronchopulmonary aspergillosis (ABPA) is a bronchopulmonary disease caused by a complex hypersensitivity to Aspergillus and is usually associated with underlying respiratory diseases such as asthma or cystic fibrosis. Mucus plugging can lead to segmental or lobar atelectasis, but complete lung atelectasis has been exceptionally reported in the literature, making it difficult to diagnose. The diagnosis of ABPA may however be suggested in patients without known predisposing respiratory disorder, even in the absence of other relevant radiographic findings. CASE PRESENTATION: We report five cases of total unilateral lung collapse secondary to ABPA in 70-81-year-old women. Two of them had a past history of ABPA, while total unilateral lung collapse was the first sign of the disease in the other three patients, contributing to the initial misdiagnosis. Flexible bronchoscopy was initially performed to remove mucus plugs from the obstructed airways but was inefficient in four cases. Corticosteroid and/or antifungal treatment was needed. CONCLUSION: ABPA can cause total unilateral lung collapse even in patients without known underlying chronic respiratory disease, making the diagnosis difficult. Flexible bronchoscopy should be considered when lung collapse is associated with respiratory distress but corticosteroids are the mainstay treatment for ABPA.


Asunto(s)
Aspergilosis Broncopulmonar Alérgica/diagnóstico , Atelectasia Pulmonar/etiología , Anciano , Anciano de 80 o más Años , Aspergilosis Broncopulmonar Alérgica/complicaciones , Femenino , Humanos
7.
J Dairy Sci ; 104(3): 3240-3249, 2021 Mar.
Artículo en Inglés | MEDLINE | ID: mdl-33455791

RESUMEN

Colostrum samples from 366 Charolais primiparous cows, as well as serum from their calves at 24 to 48 h of age, were collected to gain an overview of the situation regarding passive immune transfer in beef cattle, from both the phenotypic and genetic points of view. All samples were analyzed to quantify their G1 immunoglobulins by radial immunodiffusion (RID) and their IgG, IgA, and IgM using ELISA. The average concentrations obtained in colostrum were 84 mg/mL for RID-IgG1, and 158 mg/mL, 4.5 mg/mL and 10.8 mg/mL for ELISA-IgG, -IgA, and -IgM, respectively. The corresponding values in calf serum were 19.9, 30.6, 1.0, and 1.9 mg/mL. Apart from the general environmental effect (farm-year combination and laboratory conditions), the characteristics of the dams tested did not reveal any influence on colostrum immunoglobulin concentrations. Calving difficulty, as well as the birth weight and sex of calves, were found to be associated with serum concentrations in some cases. Heritability estimates were low to moderate, with the highest being for RID-IgG1 in colostrum (h2 = 0.28, standard error = 0.14) and serum (h2 = 0.36, standard error = 0.18). Phenotypic correlations among the different immunoglobulins were generally positive or null, and none of the genetic correlations were significant due to large standard errors. The phenotypic correlation between dam colostrum and calf serum values was 0.2 for RID-IgG1 and null for the 3 ELISA measurements. The correlation between RID-IgG1 and ELISA-IgG was, unexpectedly, null for colostrum and 0.4 for serum. Increased RID-IgG1 levels in calf serum were associated with improved survival, as well as better early growth and fewer health problems. These results thus showed that despite generally higher concentrations in beef than in dairy cattle, passive transfer was unsuccessful in a considerable number of calves. This should be brought to the attention of breeders to avoid negative effects on survival and subsequent performance. The heritability estimates were encouraging; however, obtaining phenotypes on a large scale constitutes a real limitation regarding these traits.


Asunto(s)
Calostro , Inmunoglobulina G , Animales , Animales Recién Nacidos , Bovinos , Ensayo de Inmunoadsorción Enzimática/veterinaria , Femenino , Inmunodifusión/veterinaria , Embarazo
8.
Eur J Neurol ; 25(5): 775-781, 2018 05.
Artículo en Inglés | MEDLINE | ID: mdl-29442416

RESUMEN

BACKGROUND AND PURPOSE: Magnetic resonance imaging is part of the diagnostic criteria for Alzheimer's disease (AD) through the evaluation of hippocampal atrophy. The objective of this study was to evaluate which sequence of T1-weighted (T1WI) and T2-weighted (T2WI) imaging allowed the best visual evaluation of hippocampal atrophy. METHODS: Visual qualitative ratings of the hippocampus of 100 patients with mild cognitive impairment (MCI) and 50 patients with AD were made independently by four operators according to the medial temporal lobe atrophy score based either on T1WI or T2WI. These two evaluations were compared in terms of interobserver reproducibility, concordance with a quantitative volumetric measure, discrimination power between AD and MCI groups, and correlation with several neuropsychological tests. RESULTS: The medial temporal lobe atrophy score evaluated on either T1WI or T2WI exhibited similar interobserver variability and accordance with quantitative volumetric evaluation. However, the visual evaluation on T2WI seemed to provide better discrimination power between AD and MCI groups for both left (T1WI, P = 0.0001; T2WI, P = 7.072 × 10-5 ) and right (T1WI, P = 0.008; T2WI, P = 0.001) hippocampus, and a higher overall correlation with neuropsychological tests. CONCLUSIONS: The present study suggests that T2WI provides a more adequate visual rating of hippocampal atrophy.


Asunto(s)
Enfermedad de Alzheimer/diagnóstico por imagen , Atrofia/diagnóstico por imagen , Disfunción Cognitiva/diagnóstico por imagen , Hipocampo/diagnóstico por imagen , Imagen por Resonancia Magnética/métodos , Anciano , Anciano de 80 o más Años , Enfermedad de Alzheimer/patología , Enfermedad de Alzheimer/psicología , Atrofia/patología , Disfunción Cognitiva/patología , Disfunción Cognitiva/psicología , Femenino , Hipocampo/patología , Humanos , Estudios Longitudinales , Masculino , Pruebas Neuropsicológicas , Reproducibilidad de los Resultados
9.
Phys Rev Lett ; 118(16): 162501, 2017 Apr 21.
Artículo en Inglés | MEDLINE | ID: mdl-28474951

RESUMEN

Prompt γ-ray spectroscopy of the neutron-rich ^{96}Kr, produced in transfer- and fusion-induced fission reactions, has been performed using the combination of the Advanced Gamma Tracking Array and the VAMOS++ spectrometer. A second excited state, assigned to J^{π}=4^{+}, is observed for the first time, and a previously reported level energy of the first 2^{+} excited state is confirmed. The measured energy ratio R_{4/2}=E(4^{+})/E(2^{+})=2.12(1) indicates that this nucleus does not show a well-developed collectivity contrary to that seen in heavier N=60 isotones. This new measurement highlights an abrupt transition of the degree of collectivity as a function of the proton number at Z=36, of similar amplitude to that observed at N=60 at higher Z values. A possible reason for this abrupt transition could be related to the insufficient proton excitations in the g_{9/2}, d_{5/2}, and s_{1/2} orbitals to generate strong quadrupole correlations or to the coexistence of competing different shapes. An unexpected continuous decrease of R_{4/2} as a function of the neutron number up to N=60 is also evidenced. This measurement establishes the Kr isotopic chain as the low-Z boundary of the island of deformation for N=60 isotones. A comparison with available theoretical predictions using different beyond mean-field approaches shows that these models fail to reproduce the abrupt transitions at N=60 and Z=36.

10.
Rev Neurol (Paris) ; 173(6): 374-380, 2017 Jun.
Artículo en Inglés | MEDLINE | ID: mdl-28501143

RESUMEN

Epilepsy is an increasingly recognized comorbidity in Alzheimer's disease (AD). First described as generalized in dementia patients, epileptic AD patients are nowadays fully described in earlier stages of the disease (with mild or subjective cognitive impairment). At such early stages, patients may present not only with generalized seizures, but also with focal seizures (commonly localized in the frontal or temporal lobe). Thus, partial or generalized epilepsy is part of the semiological spectrum of AD that should be borne in mind at all stages of disease to ensure early identification and prevent the risk of repeated seizures (such as accidents, injury, progression of cognitive impairment). This review of the available (and still growing) literature shows that there are already sufficient data to inform physicians on seizure semiology, and on the diagnostic value of electroencephalography and brain imaging. Taken together, these tools can help to rapidly identify epilepsy in AD patients. Nevertheless, epilepsy diagnosis can be challenging, and test medication is sometimes necessary. Some cerebrospinal fluid biomarkers (or their ratios) may also prove to be good predictors of seizures in AD, but further studies are needed. Epilepsy in AD patients is frequently pharmacosensitive, and a good response can be obtained with standard doses of antiepileptic drugs. For all these reasons and based on our review of the literature, it appears that, at present, the diagnosis of epilepsy in AD is not only possible at any stage of the disease, but also to be recommended to improve the patient's prognosis.


Asunto(s)
Enfermedad de Alzheimer/diagnóstico , Epilepsia/diagnóstico , Enfermedad de Alzheimer/complicaciones , Disfunción Cognitiva/etiología , Disfunción Cognitiva/patología , Progresión de la Enfermedad , Diagnóstico Precoz , Electroencefalografía/métodos , Epilepsia/etiología , Humanos , Síntomas Prodrómicos
11.
Rev Neurol (Paris) ; 176(9): 639-641, 2020 11.
Artículo en Inglés | MEDLINE | ID: mdl-32312499
13.
Rev Neurol (Paris) ; 171(4): 373-81, 2015 Apr.
Artículo en Francés | MEDLINE | ID: mdl-25847396

RESUMEN

INTRODUCTION: 'Theory of Mind' refers to the ability to attribute mental states, thoughts (cognitive component) or feelings (affective component), to others. This function has been studied in many neurodegenerative diseases; however, to our knowledge no studies investigating theory of mind in dementia with Lewy Bodies (DLB) have been published. The aim of our study was to search theory of mind deficits in patients with DLB. METHODS: Seven patients with DLB (DLB group), at the stage of mild dementia or mild cognitive impairments, and seven healthy elderly adults (control group) were included in the study. After a global cognitive assessment, we used the Faux Pas Recognition test to assess the cognitive component of theory of mind, and the Reading the Mind in the Eyes test for the assessment of affective component. RESULTS: We found a significant difference between the two groups for the Faux Pas test with an average score of 35.6 for the DLB group and 48.3 for the control group (P=0.04). Scores were particularly low in the DLB group for the last question of the test concerning empathy (42.9% versus 85%, P=0.01). There was not a significant difference between the two groups for the Reading the Mind in the Eyes test (P=0.077). DISCUSSION: This preliminary study showed early impairments of theory of mind in the DLB. The cognitive component seems more affected than the affective component in this pathology. This pattern is consistent with the pattern found in Parkinson's disease, but differs from other neurodegenerative diseases as Alzheimer's disease or frontotemporal lobe dementia. These patterns may help to differentiate DLB from these diseases. Further study is needed to confirm these results and to compare with other dementias.


Asunto(s)
Afecto , Cognición , Enfermedad por Cuerpos de Lewy/psicología , Teoría de la Mente , Adulto , Anciano , Anciano de 80 o más Años , Progresión de la Enfermedad , Empatía , Femenino , Humanos , Masculino , Persona de Mediana Edad , Pruebas Neuropsicológicas , Reconocimiento en Psicología , Conducta Social , Percepción Social
14.
Ann Rheum Dis ; 73(5): 831-7, 2014 May.
Artículo en Inglés | MEDLINE | ID: mdl-23606708

RESUMEN

BACKGROUND: The standard-of-care treatment of patients with hepatitis C virus (HCV)-mixed cryoglobulinemia (MC) vasculitis includes pegylated interferon α (PegIFN)-α plus ribavirin and/or rituximab. About 30-40% of patients are non-responders or relapsers to such combination. OBJECTIVE: To analyse the safety and efficacy of Peg-IFNα/ribavirin/protease inhibitor combination in HCV-MC vasculitis. PATIENTS AND METHODS: Open-label, prospective, cohort study including 23 patients with HCV-MC vasculitis. Peg-IFNα/ribavirin was associated to telaprevir (375 mg three times daily, for 12 weeks, (n=15)) or boceprevir (800 mg three times daily, for 44 weeks, (n=8)) for 48 weeks. RESULTS: The median age was 59 (52.5-66) years, with 48.8% women. Thirteen patients (56.5%) were complete clinical responders, and 10 (43.5%) were partial responders at week 24. The virological response (ie, HCV RNA negativation) was of 69.6% at week 24 (p=0.005). The cryoglobulin level decreased from 0.44 to 0.06 g/l (p=0.0006) and the C4 level increased from 0.09 to 0.15 g/l (p=0.045). Grades 3 and 4 adverse events (mainly anaemia, neutropenia and thrombocytopenia) were observed in 10 cases (43.5%). Twenty patients (87%) received erythropoietin, 9 (39.1%) had red cell transfusion, and 2 (8.7%) had granulocyte stimulating agents. Antiviral therapy discontinuation was required in 8 (34.7%) patients for virological non-response (n=5), virological relapse (n=2) and depression (n=1). CONCLUSIONS: Peg-IFNα/ribavirin/protease inhibitor combination seems highly effective in HCV-MC. Such therapeutic regimen should be administered cautiously considering the high rate of side effects.


Asunto(s)
Antivirales/administración & dosificación , Crioglobulinemia/tratamiento farmacológico , Hepatitis C Crónica/tratamiento farmacológico , Interferón-alfa/administración & dosificación , Polietilenglicoles/administración & dosificación , Inhibidores de Proteasas/administración & dosificación , Ribavirina/administración & dosificación , Anciano , Antivirales/efectos adversos , Estudios de Cohortes , Crioglobulinemia/virología , Quimioterapia Combinada , Femenino , Hepacivirus , Hepatitis C Crónica/complicaciones , Humanos , Interferón-alfa/efectos adversos , Masculino , Persona de Mediana Edad , Oligopéptidos/administración & dosificación , Oligopéptidos/efectos adversos , Polietilenglicoles/efectos adversos , Prolina/administración & dosificación , Prolina/efectos adversos , Prolina/análogos & derivados , Inhibidores de Proteasas/efectos adversos , Proteínas Recombinantes/administración & dosificación , Proteínas Recombinantes/efectos adversos , Ribavirina/efectos adversos , Resultado del Tratamiento , Vasculitis/tratamiento farmacológico , Vasculitis/virología
15.
Eur J Neurol ; 21(6): 901-6, 2014 Jun.
Artículo en Inglés | MEDLINE | ID: mdl-24698178

RESUMEN

BACKGROUND AND PURPOSE: Anti-ß2-glycoprotein I (anti-ß2-GPI) antibodies are part of the heterogeneous family of antiphospholipid antibodies and seem to be present in various neurological manifestations in addition to antiphospholipid syndrome (APS). Our objective was to analyse the clinical, radiological and therapeutic characteristics of neurological patients with positive anti-ß2-GPI antibodies and without the Sapporo criteria for APS. METHODS: The medical records were retrospectively reviewed of 28 consecutive patients hospitalized in the Neurology Department of Strasbourg University Hospital, France, in whom anti-ß2-GPI antibodies (immunoglobulin G and/or immunoglobulin M) were positive and other antiphospholipid antibodies negative, from November 2005 to July 2011. Clinical, radiological, biological and therapeutic data and clinical course were studied. RESULTS: Positive anti-ß2-GPI antibodies were present in 28 patients. The predominant physiopathological process was mainly inflammatory (25% with myelitis, 14.3% with optic neuritis) or vascular (14.3% with cerebral ischaemia, 7.1% with cerebral vasculitis). Brain magnetic resonance imaging was performed in 89.3% of patients: atypical lesions were observed in 44% and typical inflammatory and vascular lesions in 16% and 12%, respectively. CONCLUSION: The anti-ß2-GPI antibody seems to be involved in two types of neurological disease: vascular or inflammatory 'multiple sclerosis-like' disease. These two types of patients frequently develop an autoimmune disease (multiple sclerosis, systemic lupus erythematosus, APS). However, a large proportion of the patients had an undefined profile with aspecific cerebral lesions and required monitoring. This study raises questions about a separate entity at the border between APS and multiple sclerosis which remains to be better defined in a larger cohort.


Asunto(s)
Anticuerpos Antifosfolípidos/inmunología , Síndrome Antifosfolípido/inmunología , Esclerosis Múltiple/inmunología , beta 2 Glicoproteína I/inmunología , Adolescente , Adulto , Anciano , Anciano de 80 o más Años , Femenino , Francia , Humanos , Masculino , Persona de Mediana Edad , Neurología , Estudios Retrospectivos , Adulto Joven
16.
J Acoust Soc Am ; 135(6): 3221-30, 2014 Jun.
Artículo en Inglés | MEDLINE | ID: mdl-24907787

RESUMEN

A simple analytical model for the interaction between a plane jet issued from a flue and a transverse acoustic disturbance is developed in this paper. The model is inspired by direct flow simulation results confronted to experimental data. The interaction is expected to take place in the vicinity of the separation points of the jet. The influence of the detailed geometry of the channel end on the jet receptivity is discussed, and more specifically the chamfer geometries found in flute-like musical instruments. The simplified model explains quite well the difference between the jet response of a flue with square edges compared to a chamfered flue exit. The effect of rounded, lip-like flue exit is not well captured by the model.

17.
Rev Neurol (Paris) ; 170(6-7): 445-53, 2014.
Artículo en Francés | MEDLINE | ID: mdl-24746394

RESUMEN

INTRODUCTION: Cerebrotendinous xanthomatosis, a metabolic leukodystrophy with an autosomal recessive inheritance, is secondary to deficiency of sterol 27-hydroxylase, an enzyme involved in cholesterol catabolism. Classical symptoms include clinical or infraclinical xanthomas affecting the skin and tendons, early cataracts, neurological signs and diarrhea. Brain imaging reveals involvement of the dentate nuclei and periventricular white matter hyperintensities. The diagnosis is based on an increased cholestanol level in serum, confirmed by the presence of a mutation in the CYP27A1 gene. Treatment is based on chenodeoxycholic acid. METHOD: We report a retrospective multicentric study of 15 cases of cerebrotendinous xanthomatosis diagnosed in French adults. Clinical, molecular and MRI findings were recorded in all patients. RESULTS: The average age at diagnosis was 39years (range 27-65). Disease onset occurred in childhood in 73% of patients and in adulthood in 27%. All patients with a pediatric onset were diagnosed during adulthood (age range 28-65years). Clinical symptoms variably associated cerebellar syndrome, pyramidal syndrome, cognitive decline, epilepsy, neuropathy (sought in 10 of our patients, present in forms in 8), psychiatric disorders, cataract and xanthomas. One patient had an atypical presentation: monoparesis associated with xanthomas. Brain MRI was abnormal in all: findings consisted in T2-weighted hyperintensity of the dentate nuclei (47%), periventricular leuoencephalopathy (73%) which preferentially involved the posterior cerebral part (60%), leucoencephalopathy with a vascular pattern (7%), hyperintensity of the cortico-spinal tracts (53%), globi pallidi, corpus callosum and cerebral atrophy (33%). Serum cholestanol was elevated in 93% of patients. The most frequent mutation was 1183C>T (n=5/15). Under treatment with chenodeoxycholic acid, eight patients improved initially, followed by stabilization in five of them, and worsening in the others. Four patients died. CONCLUSION: Patients with the xanthoma-neurological disorder association should be tested for cerebrotendinous xanthomatosis. The disease often begins in childhood with a diagnostic delay but also in adulthood. Involvement of the dentate nuclei is specific but not sensitive and the supratentorial leucoencephalopathy is not specific but with an antero-posterior gradient. A vascular distribution and involvement of the corpus callosum are possible. Serum cholestanol assay is very reliable: an elevated level provides the diagnosis, which must nevertheless be confirmed by molecular biology.


Asunto(s)
Xantomatosis Cerebrotendinosa , Adulto , Edad de Inicio , Anciano , Sustitución de Aminoácidos , Encéfalo/patología , Ácido Quenodesoxicólico/uso terapéutico , Colestanotriol 26-Monooxigenasa/deficiencia , Colestanotriol 26-Monooxigenasa/genética , Femenino , Genes Recesivos , Humanos , Imagen por Resonancia Magnética , Masculino , Persona de Mediana Edad , Mutación Missense , Estudios Retrospectivos , Evaluación de Síntomas , Xantomatosis Cerebrotendinosa/tratamiento farmacológico , Xantomatosis Cerebrotendinosa/epidemiología , Xantomatosis Cerebrotendinosa/patología
18.
Mult Scler ; 19(10): 1320-2, 2013 Sep.
Artículo en Inglés | MEDLINE | ID: mdl-23413296

RESUMEN

BACKGROUND: Neuromyelitis optica (NMO) is an inflammatory disease associated with optic neuritis and myelitis. Recently, several studies showed that optical coherence tomography (OCT) could be an interesting method for the evaluation of disease severity; however, to date there are no studies with a longitudinal follow-up of visual function in NMO. The aim of this study was to assess the ability of OCT to evaluate the progression of visual dysfunction in NMO. PATIENTS AND METHODS: A group of 30 NMO patients (thus, 60 eyes), comprised of 20 women and 10 men with a mean age of 43.7 +/- 12.3 years, were prospectively evaluated clinically and by a whole neuro-ophthalmological work-up, including: visual acuity (VA), fundoscopy, visual evoked potential (VEP), visual field (VF) and optical coherence tomography (OCT). All patients were tested at baseline (after a mean disease duration of 6.1 years) and after a mean time of follow-up of 18 months (range: 12-36 months). RESULTS: Mean VA was similar at the two evaluation times (0.77 +/- 0.36 versus 0.77 +/- 0.35). The mean VF defect decreased slightly, but the difference was not significant (-5.9 +/- 1.3 dB versus -5.3 +/- 1.3 dB). In contrast, the mean retinal thickness seen on OCT decreased from 87.4 +/- 23.3 µm to 79.7 +/- 22.4 µm (p = 0.006). These modifications were only observed in eyes with a past or a recent history of optic neuritis (-15.1 µm; p < 0.001) and not in eyes without any history of optic neuritis (-2.4 µm; not significant). Also, they occurred independently of the occurrence of relapses (n = 13) and especially optic neuritis episodes; however, the number of optic neuritis episodes was low (n = 5). CONCLUSION: OCT seems to be a more sensitive test than VA or VF for monitoring ophthalmological function in NMO and it seems to be helpful for the detection of infra-clinical episodes in patients with a past history of optic neuritis. Our results suggest that this easily performed technique should be used in the follow-up of NMO, but complementary studies are warranted to confirm its interest at an individual level.


Asunto(s)
Neuromielitis Óptica/complicaciones , Tomografía de Coherencia Óptica , Trastornos de la Visión/diagnóstico , Agudeza Visual/fisiología , Adulto , Estudios de Cohortes , Potenciales Evocados Visuales/fisiología , Femenino , Estudios de Seguimiento , Humanos , Estudios Longitudinales , Masculino , Persona de Mediana Edad , Trastornos de la Visión/etiología , Campos Visuales/fisiología
19.
Eur J Neurol ; 20(2): 361-7, 2013 Feb.
Artículo en Inglés | MEDLINE | ID: mdl-22978568

RESUMEN

BACKGROUND: Neuromyelitis optica (NMO) is an inflammatory disease involving predominantly the spinal cord and optic nerves. Whether patients with NMO have a loss in white or grey matter (GM) volumes remains to be determined. METHODS: Thirty patients with NMO, 30 healthy subjects matched for age and gender, 21 patients with multiple sclerosis (MS) and 20 patients with a clinically isolated syndrome (CIS) were studied. We applied a SIENAX post-treatment software. We compared white matter (WM) and GM volumes between groups and explored correlations of changes in NMO patients with age, gender, duration, disease severity, visual acuity and T2 hyperintensities. We also performed a voxel-based morphometry (VBM) analysis to identify the regions affected by loss of volume. RESULTS: White matter volume was significantly reduced in patients with NMO (764.4 ± 58.3 cm(3) ) compared to healthy subjects (843.1 ± 49.3 cm(3) ) (P < 0.001), whereas no difference was observed for the GM. Patients with CIS also presented an elective atrophy of WM and MS an atrophy of both WM and GM. We did not find any predictive factors of brain atrophy. The decrease in WM volume in NMO was noted even in the absence of visible MRI hypersignals. The VBM analysis found a few regions of WM atrophy (corpus callosum and optic radiations, P < 0.005, uncorrected) and a few regions of GM atrophy (thalamus and prefrontal cortex, P < 0.001, uncorrected). CONCLUSION: These results suggest a significant brain involvement in NMO, especially an involvement of WM which appears not to be limited to secondary degeneration after spinal cord and optic nerve damage.


Asunto(s)
Encéfalo/patología , Fibras Nerviosas Mielínicas/patología , Neuromielitis Óptica/patología , Adulto , Atrofia/patología , Encéfalo/fisiopatología , Estudios de Casos y Controles , Enfermedades Desmielinizantes/patología , Enfermedades Desmielinizantes/fisiopatología , Femenino , Humanos , Imagen por Resonancia Magnética , Masculino , Persona de Mediana Edad , Esclerosis Múltiple/patología , Esclerosis Múltiple/fisiopatología , Fibras Nerviosas Mielínicas/fisiología , Fibras Nerviosas Amielínicas/patología , Fibras Nerviosas Amielínicas/fisiología , Neuroimagen , Neuromielitis Óptica/fisiopatología , Agudeza Visual/fisiología
20.
Neuropsychol Rehabil ; 23(2): 161-81, 2013.
Artículo en Inglés | MEDLINE | ID: mdl-22978301

RESUMEN

The multifocal nature of lesions in multiple sclerosis hints at the occurrence of autobiographical memory (AbM) impairment. However, the dearth of studies on AbM in multiple sclerosis is noticeable, notwithstanding the importance of AbM in everyday life. In the first section of this study, 25 multiple sclerosis patients and 35 controls underwent a detailed episodic AbM assessment. Results obtained by means of ANOVA suggested an AbM retrieval deficit in every patient. That pattern of performance paved the way for the second section of the study, in which we followed up 10 out of the 25 patients. Our objective was to assess the effectiveness of a cognitive facilitation programme designed to alleviate AbM retrieval deficits, based on the key role of mental visual imagery on AbM. Statistical group analyses by means of ANOVA and individual analyses using the χ(2) test showed significant differences in AbM test results, in post-facilitation relative to pre-facilitation training, in all 10 patients. Moreover, the patients' comments showed that the positive effects were transferred in their daily life functioning. We would like to suggest that the facilitation programme efficiently enhanced the process of self-centred mental visual imagery, which might have compensated for poor retrieval of personal memories by providing better access to visual details and detailed visual scenes of personal recollections.


Asunto(s)
Terapia Cognitivo-Conductual/métodos , Trastornos de la Memoria/etiología , Trastornos de la Memoria/rehabilitación , Memoria Episódica , Esclerosis Múltiple/complicaciones , Adulto , Análisis de Varianza , Señales (Psicología) , Terapia por Ejercicio/métodos , Femenino , Estudios de Seguimiento , Humanos , Masculino , Trastornos de la Memoria/psicología , Persona de Mediana Edad , Esclerosis Múltiple/psicología , Esclerosis Múltiple/rehabilitación , Pruebas Neuropsicológicas , Escalas de Valoración Psiquiátrica , Autoimagen , Vocabulario , Adulto Joven
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