Doença De Pelizaeus Merzbacher: Dismielinização Versus Desmielinização

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First, Do No Harm: The Risks Of Overtreating Children With Epilepsy

Background: Although overtreatment with antiepileptic drugs contributes to the morbidity associated with epilepsy, many children still are overtreated. Objective: To evaluate if the withdrawal of at least one antiepileptic drug (AED) in children with refractory epilepsy using polytherapy enable a better seizure control. Method: This was a prospective study. Children with refractory epilepsy using at least two AEDs were included. Once the patient, or guardian, agreed to participate in the study, one or more AED were slowly tapered off. The remaining AEDs dosages could be adjusted as needed, but a new AED could not be introduced. Results: Fifteen patients were evaluated, three girls; ages ranging from 3 to 18 (mean=8.7 years). After at least one AED withdrawal, two (13.5%) patients became seizure free, seizures improved >50% in 5 (33.5%) patients, did not change in 5 (33.5%), and seizure frequency became worse in 3 (20%). Adverse events improved in 12 patients (80%). Conclusion: The withdrawal of at least one AED is a valuable option in the treatment of selected children with refractory epilepsy.65114Camfield, C.S., Camfield, P., Gordon, K., Smith, B., Dooley, J., Outcome of childhood epilepsy: A population-based study with a simple scoring system for those treated with medication (1993) J Pediatr, 122, pp. 861-868Silva, M., MacArdle, B., MaGowan, M., Randomised comparative monotherapy trial of phenobarbitone, phenytoin, carbamazepine, or sodium valproate for newly diagnosed childhood epilepsy (1996) Lancet, 347, pp. 709-713Baulac, M., Rational conversion from antiepileptic polytherapy to monotherapy (2003) Epileptic Disord, 5, pp. 125-132Holmes, G.L., Overtreatment in children with epilepsy (2002) Epilepsy Res, 52, pp. 35-42Fischbacher, E., Effect of reduction of anticonvulsants on wellbeing (1982) Br Med J (Clin Res Ed), 285, pp. 423-424Schmidt, D., Reduction of two-drug therapy in intractable epilepsy (1983) Epilepsia, 24, pp. 368-376Alvarez, N., Discontinuance of antiepileptic medications in patients with developmental disability and diagnosis of epilepsy (1989) Am J Ment Retard, 93, pp. 593-595Guerrini, R., Belmonte, A., Genton, P., Antiepileptic drug-induced worsening of seizures in children (1998) Epilepsia, 39 (SUPPL. 3), pp. S2-S10TS, K., Holmes, G.L., EEG and clinical predictors of medically intractable childhood epilepsy (1999) Clin Neurophysiol, 110, pp. 1245-1251Huttenlocker, P.R., Hapke, R.J., A follow-up study of intractable seizures in childhood (1990) Ann Neurol, 28, pp. 699-705Ferngren, H., Akerstrom, I., Rane, A., Mono or polypharmacotherapy in institutionalized epileptic children with severe mental retardation? A team approach for optimizing antiepileptic therapy (1991) Acta Paediatr Scand, 80, pp. 458-465Dooley, J., Gordon, K., Camfield, C., Smith, E., Discontinuation of anticonvulsant therapy in children free of seizures for 1 year: A prospective study (1996) Neurology, 46, pp. 969-974Shinnar, S., Berg, A.T., Moshé, S.L., Discontinuing antiepileptic drugs in children with epilepsy:a prospective study (1994) Ann Neurol, 35, pp. 534-545Shorvon, S.D., Reynolds, E.H., Unnecessary polypharmacy for epilepsy (1977) Br Med J, 1, pp. 1635-1637Vickery, B.G., Hay, R., Engel, J., Outcome assessment for epilepsy surgery: The impact of measuring health-related quality of life (1995) Ann Neurol, 37, pp. 158-16

IL-1β, TNF-α, TGF-β, and bFGF expression in bone biopsies before and after parathyroidectomy

IL-1β, TNF-α, TGF-β, and b FGF expression in bone biopsies before and after parathyroidectomy.BackgroundThere is growing evidence pointing to an involvement of cytokines and growth factors in renal osteodystrophy. In this study, the expression of interleukin-lβ (IL-1β), tumor necrosis factor-α (TNF-α), transforming growth factor-β (TGF-β), and basic fibroblast growth factor (bFGF) in bone biopsies taken from uremic patients before and 1 year after parathyroidectomy (PTX) was evaluated. Biochemical features and histomorphometric outcome were also studied.MethodsIliac bone biopsies were taken before and 1 year after PTX in nine uremic patients with severe hyperparathyroidism (HPT). Immunohistochemical techniques were used to identify the expression of IL-1β, TNF-α, TGF-β, and bFGF in these bone samples.ResultsAt the time of the second bone biopsy, the mean serum total alkaline phosphatase activity was normal, whereas mean serum intact parathyroid hormone (iPTH) level was slightly above the upper limit of normal values. Histomorphometric analysis showed a decrease in resorption parameters and static bone formation parameters after PTX. Dynamically, mineral apposition rate (MAR) and mineralization surface (MS/BS) decreased significantly. There was a marked local expression of IL-1β, TNF-α, TGF-β, and bFGF in bone biopsies before PTX, particularly in fibrous tissue and resorption areas. One year after PTX, IL-1β decreased from 23.6 ± 7.5% to 9.9 ± 3.1%, TNF-α from 4.5 ± 1.5% to 0.7 ± 0.8%, TGF-β from 49.6 ± 9.8% to 15.2 ± 4.6%, and bFGF from 50.9 ± 12.7% to 12.9 ± 7.9% (P < 0.001). A significant correlation was documented between cytokines and growth factors expression in bone with iPTH levels before and after PTX (P < 0.05).ConclusionsBased on these results, we suggest that IL-1β, TNF-α, TGF-β, and bFGF are involved in bone remodeling regulation, acting as local effectors, possibly under the control of PTH

Megalencephalic Leukoencephalopathy With Subcortical Cysts (mlc) – A Case With Clinical And Magnetic Resonance Imaging (mri) Dissociation [leucoencefalopatia Megalencefálica Com Cistos Subcorticais (mlc) – Um Caso Com Dissociação Clínica E De Resonância Magnética]

[No abstract available]732171172Nunes, R.H., Pacheco, F.T., Rocha, A.J., Magnetic resonance imaging of anterior temporal lobe cysts in children: Discriminating special imaging features in a particular group of diseases (2014) Neuroradiology, 56, pp. 569-577. , http://dx.doi.org/10.1007/s00234-014-1356-9Olivier, M., Lenard, H.G., Aksu, F., Gärtner, J., A new leukoencephlapathy with bilateral anterior temporal lobe cysts (1998) Neuropediatrics, 29 (5), pp. 225-228. , http://dx.doi.org/10.1055/s-2007-973566Knaap, M.S., Boor, I., Estévez, R., Megalencephalic leukoencephalopathy with subcortical cysts: Chronic white matter oedema due to a defect in brain ion and water homoeostasis (2012) Lancet Neurol, 11 (11), pp. 973-985. , http://dx.doi.org/10.1016/S1474-4422(12)70192-8Sinhal, B.S., Gorospe, J.R., Naidu, S., Megalencephalic leukoencephalopathy with subcotical cysts (2003) J Child Neurol, 18 (9), pp. 645-652. , http://dx.doi.org/10.1177/0883073803018009120

Clinical And Neuropsychological Correlation In Patients With Rolandic Epilepsy [correlação Entre Achados Clínicos E Neuropsicológicos Em Pacientes Com Epilepsia Rolândica]

Objectives: To evaluate the presence of neurological soft signs (NSS) and to correlate them with the Wechsler Intelligence Scale for Children (WISC III) in patients with rolandic epilepsy (RE). Methods: Forty children and adolescents aged between 9 and 15 years were studied. They were divided into two groups: G1 - patients with RE (n=20) - and G2 - healthy controls without epilepsy (n=20). They were assessed with the Quick Neurological Screening Test (QNST II) - clinical trial to search for NSS -, and the WISC III - neuropsychological test. Results: No statistical difference between groups was found in WISC III and QNST II. However, children with poorer motor skills had worse performance in the QNST II and also in the execution intelligence quotient - IQ (p=0.001) and in total IQ (p=0.004), thus showing a positive correlation between them. Conclusions: The QNST II is a good screening tool for the neurologist to detect abnormalities in fine motor skills.709691693Berg, A.T., Berkovic, S.F., Brodie, M.J., Revised terminology and concepts for organization of seizures and epilepsies: Report of the ILAE Commission on Classification and Terminology, 2005-2009 (2010) Epilepsia, 51, pp. 676-685Lerman, P., Kivity, S., Benign focal epilepsy of childhood. A follow-up study of 100 recovered patients (1975) Arch Neurol, 32, pp. 261-264Bouma, P.A., Bovenkerk, A.C., Westendorp, R.G., Brouwer, O.F., The course of benign partial epilepsy of childhood with centrotemporal spikes: A meta-analysis (1997) Neurology, 48 (2), pp. 430-437Hommet, C., Billard, C., Motte, J., Cognitive function in adolescents and young adults in complete remission from benign childhood epilepsy with centro-temporal spikes (2001) Epileptic Disord, 3, pp. 207-216D'Alessandro, P., Piccirilli, M., Tiacci, C., Neuropsychological features of benign partial epilepsy in children (1990) Ital J Neurol Sci, 11, pp. 265-269Binnie, C.D., Marston, D., Cognitive correlates of interictal discharges (1992) Epilepsia, 33 (SUPPL. 6), pp. S11-S17Fonseca, L.C., Tedrus, G.M., Pacheco, E.M., Epileptiform EEG discharges in benign childhood epilepsy with centrotemporal spikes: Reactivity and transitory cognitive impairment (2007) Epilepsy Behav, 11, pp. 65-70Baglietto, M.G., Battaglia, F.M., Nobili, N., Neuropsychological disorders related to interictal epileptic discharges during sleep in benign epilepsy of childhood with centrotemporal or rolandic spikes (2001) Dev Med Child Neurol, 43, pp. 407-412Hermann, B.P., Jones, J., Sheth, R., Seidenberg, M., Cognitive and magnetic resonance volumetric abnormalities in new-onset pediatric epilepsy (2007) Semin Pediatri Neurol, 14, pp. 173-180Panayiotopoulos, C.P., Benign childhood partial epilepsies: Benign childhood seizures susceptibility syndromes (1993) J Neurol Neurosurg Psychiatry, 56, pp. 2-5Lindgren, A., Kihlgren, M., Melin, L., Croona, C., Development of cognitive functions in children with rolandic epilepsy (2004) Epilepsy Behav, 5, pp. 903-910Weglage, J., Demsky, A., Pietsh, M., Kurlemann, G., Neuropsychological, intellectual, and behavioral findings in patients with centrotemporal spikes with and without seizure (1997) Dev Med Child Neurol, 39, pp. 645-651Deonna, T., Rolandic epilepsy: Neuropsychology of the active epilepsy phase (2000) Epileptic Disord, 2 (SUPPL. 1), pp. S59-S61Deonna, T., Zesiger, P., Davidoff, V., Benign partial epilepsy of childhood: A longitudinal neuropsychological and EEG study of cognitive function (2000) Dev Med Child Neurol, 42, pp. 595-60

Observational tests for \Lambda(t)CDM cosmology

We investigate the observational viability of a class of cosmological models in which the vacuum energy density decays linearly with the Hubble parameter, resulting in a production of cold dark matter particles at late times. Similarly to the flat \Lambda CDM case, there is only one free parameter to be adjusted by the data in this class of \Lambda(t)CDM scenarios, namely, the matter density parameter. To perform our analysis we use three of the most recent SNe Ia compilation sets (Union2, SDSS and Constitution) along with the current measurements of distance to the BAO peaks at z = 0.2 and z = 0.35 and the position of the first acoustic peak of the CMB power spectrum. We show that in terms of $\chi^2$ statistics both models provide good fits to the data and similar results. A quantitative analysis discussing the differences in parameter estimation due to SNe light-curve fitting methods (SALT2 and MLCS2k2) is studied using the current SDSS and Constitution SNe Ia compilations. A matter power spectrum analysis using the 2dFGRS is also performed, providing a very good concordance with the constraints from the SDSS and Constitution MLCS2k2 data.Comment: Revised version, to appear in JCA

The LOFAR Two Meter Sky Survey: Deep Fields, I -- Direction-dependent calibration and imaging

The Low Frequency Array (LOFAR) is an ideal instrument to conduct deep extragalactic surveys. It has a large field of view and is sensitive to large-scale and compact emission. It is, however, very challenging to synthesize thermal noise limited maps at full resolution, mainly because of the complexity of the low-frequency sky and the direction dependent effects (phased array beams and ionosphere). In this first paper of a series, we present a new calibration and imaging pipeline that aims at producing high fidelity, high dynamic range images with LOFAR High Band Antenna data, while being computationally efficient and robust against the absorption of unmodeled radio emission. We apply this calibration and imaging strategy to synthesize deep images of the Boötes and Lockman Hole fields at ∼150 MHz, totaling ∼80 and ∼100 h of integration, respectively, and reaching unprecedented noise levels at these low frequencies of .30 and .23 µJy beam−1 in the inner ∼3 deg2 . This approach is also being used to reduce the LOTSS-wide data for the second data release