Sound Absorption and Dispersion in Dilute Polyatomic Gases: A Generalized Kinetic Approach

A generalized kinetic model equation which takes into account the frequency depence of the thermal conductivity is used to analyze the problem of sound propagation in dilute polyatomic gases. By comparing the theoretical results with some available experimental data we infer that our model equation provides a precise transition between low and high-frequency limits.Comment: 16 pages, 2 figure

X-linked adrenoleukodystrophy in heterozygous female patients: women are not just carriers

X-linked adrenoleukodystrophy (X-ALD) is a recessive X-linked disorder associated with marked phenotypic variability. Female carriers are commonly thought to be normal or only mildly affected, but their disease still needs to be better described and systematized. Objectives: To review and systematize the clinical features of heterozygous women followed in a Neurogenetics Clinic. Methods: We reviewed the clinical, biochemical, and neuroradiological data of all women known to have X-ADL. Results: The nine women identified were classified into three groups: with severe and aggressive diseases; with slowly progressive, spastic paraplegia; and with mildly decreased vibratory sensation, brisk reflexes, and no complaints. Many of these women did not have a known family history of X-ALD. Conclusions: Heterozygous women with X-ADL have a wide spectrum of clinical manifestations, ranging from mild to severe phenotypes

Paraparesia espástica como manifestação inicial da ataxia espinocerebelar do tipo 7

Conselho Nacional de Pesquisa (CNPq)(FAEPA) Fundação de Apoio ao Ensino, Pesquisa e Assistência do Hospital das Clínicas da Faculdade de Medicina de Ribeirão Pret

Equilibrium and stability properties of detonation waves in the hydrodynamic limit of a kinetic model

A shock wave structure problem, like the one which can be formulated for the planar detonation wave, is analyzed here for a binary mixture of ideal gases undergoing the symmetric reaction A1+A1=A2+A2 . The problem is studied at the hydrodynamic Euler limit of a kinetic model of the reactive Boltzmann equation. The chemical rate law is deduced in this frame with a second-order reaction rate, in a hemical regime such that the gas flow is not far away from the chemical equilibrium. The caloric and the thermal equations of state for the specific internal energy and temperature are employed to close the system of balance laws. With respect to other approaches known in the kinetic literature for detonation problems with a reversible reaction, this paper aims to improve some aspects of the wave solution. Within the mathematical analysis of the detonation model, the equation of the equilibrium Hugoniot curve of the final states is explicitly derived for the first time and used to define the correct location of the equilibrium Chapman–Jouguet point in the Hugoniot diagram. The parametric space is widened to investigate the response of the detonation solution to the activation energy of the chemical reaction. Finally, the mathematical formulation of the linear stability problem is given for the wave detonation structure via a normal-mode approach, when bidimensional disturbances perturb the steady solution. The stability equations with their boundary conditions and the radiation condition of the considered model are explicitly derived for small transversal deviations of the shock wave location. The paper shows how a second-order chemical kinetics description, derived at the microscopic level, and an analytic deduction of the equilibrium Hugoniot curve, lead to an accurate picture of the steady detonation with reversible reaction, as well as to a proper bidimensional linear stability analysis.Brazilian Research Council (CNPq), by Italian Research Council GNFM-INdAM, and by the Research Centre of Mathematics of the University of Minho with the Portuguese Funds of FCT, project PEstOE/MAT/UI0013/2014

Balance and muscle power of children with Charcot-Marie-Tooth

ABSTRACT | Background: In certain diseases, functional constraints establish a greater relationship with muscle power than muscle strength. However, in hereditary peripheral polyneuropathies, no such relationship was found in the literature. Objective: In children with Charcot-Marie-Tooth (CMT), to identify the impact of muscle strength and range of movement on the static/dynamic balance and standing long jump based on quantitative and functional variables. Method: The study analyzed 19 participants aged between 6 and 16 years, of both genders and with clinical diagnoses of CMT of different subtypes. Anthropometric data, muscle strength of the lower limbs (hand-held dynamometer), ankle and knee range of movement, balance (Pediatric Balance Scale) and standing long jump distance were obtained by standardized procedures. For the statistical analysis, Pearson and Spearman correlation coefficients were used. Results: There was a strong positive correlation between balance and the muscle strength of the right plantar flexors (r=0.61) and dorsiflexors (r=0.59) and a moderate correlation between balance and the muscle strength of inversion (r=0.41) and eversion of the right foot (r=0.44). For the long jump and range of movement, there was a weak positive correlation with right and left plantar flexion (r=0.20 and r=0.12, respectively) and left popliteal angle (r=0.25), and a poor negative correlation with left dorsiflexion (r=-0.15). Conclusions: The data on the patients analyzed suggests that the maintenance of distal muscle strength favors performance during balance tasks, while limitations in the range of movement of the legs seem not to be enough to influence the performance of the horizontal long jump. Keywords: Charcot-Marie-Tooth disease; strength; balance; range of movement; assessment; physical therapy. HOW TO CITE THIS ARTICLE Silva TR, Testa A, Baptista CRJA, Marques Jr W, Mattiello-Sverzut AC. Balance and muscle power of children with CharcotMarie-Tooth. Braz J Phys Ther. http://d

Clinical and genetic analysis of 29 Brazilian patients with Huntington’s disease-like phenotype

Huntington’s disease (HD) is a neurodegenerative disorder characterized by chorea, behavioral disturbances and dementia, caused by a pathological expansion of the CAG trinucleotide in the HTT gene. Several patients have been recognized with the typical HD phenotype without the expected mutation. The objective of this study was to assess the occurrence of diseases such as Huntington’s disease-like 2 (HDL2), spinocerebellar ataxia (SCA) 1, SCA2, SCA3, SCA7, dentatorubral-pallidoluysian atrophy (DRPLA) and choreaacanthocytosis (ChAc) among 29 Brazilian patients with a HD-like phenotype. In the group analyzed, we found 3 patients with HDL2 and 2 patients with ChAc. The diagnosis was not reached in 79.3% of the patients. HDL2 was the main cause of the HD-like phenotype in the group analyzed, and is attributable to the African ancestry of this population. However, the etiology of the disease remains undetermined in the majority of the HD negative patients with HD-like phenotype. Key words: Huntington’s disease, Huntington’s disease-like, chorea-acanthocytosis, Huntington’s disease-like 2

Definição e diagnóstico de neuropatia de fibras finas: consenso do Departamento Científico de Neuropatias Periféricas da Academia Brasileira de Neurologia

The aim of this study was to describe the results of a Brazilian Consensus on Small Fiber Neuropathy (SFN). Fifteen neurologists (members of the Brazilian Academy of Neurology) reviewed a preliminary draft. Eleven panelists got together in the city of Fortaleza to discuss and finish the text for the manuscript submission. Small fiber neuropathy can be defined as a subtype of neuropathy characterized by selective involvement of unmyelinated or thinly myelinated sensory fibers. Its clinical picture includes both negative and positive manifestations: sensory (pain/ dysesthesias/pruritus) or combined sensory and autonomic complaints, associated with an almost entirely normal neurological examination. Standard electromyography is normal. A growing list of medical conditions is associated with SFN. The classification of SFN may also serve as a useful terminology to uncover minor discrepancies in the normal values from different neurophysiology laboratories. Several techniques may disclose sensory and/or autonomic impairment. Further studies are necessary to refine these techniques and develop specific therapies.O objetivo deste estudo é descrever os resultados de um Consenso Brasileiro sobre Neuropatia de Fibras Finas (NFF). Quinze neurologistas (membros da Academia Brasileira de Neurologia) revisaram uma versão preliminar do artigo. Onze panelistas se reuniram na cidade de Fortaleza para discutir e terminar o texto para a submissão do manuscrito. NFF pode ser definida como um subtipo de neuropatia caracterizada pelo envolvimento seletivo de fibras sensitivas amielínicas ou pouco mielinizadas. Seu quadro clínico inclui manifestações negativas e positivas: sensitivas (dor/disestesias/prurido) ou queixas sensitivas e autonômicas combinadas, associadas a exame neurológico quase totalmente normal. A eletromiografia convencional é normal. Uma lista crescente de condições médicas causa NFF. NFF também pode servir como uma terminologia útil para referenciar pequenas discrepâncias nos valores normais de diferentes laboratórios de neurofisiologia. Diferentes técnicas podem evidenciar anormalidades sensitivas e/ou autonômicas. São necessários mais estudos para refiná-las e para o desenvolvimento de terapias específicas.Univ Fed Ceara, Fac Med, Hosp Univ Walter Cantidio, Fortaleza, CE, BrazilUniv Sao Paulo, Fac Med Ribeirao Preto, Dept Neurociencias & Hosp Clin, Ribeirao Preto, SP, BrazilUniv Fed Santa Catarina, Florianopolis, SC, BrazilUniv Fed Rio de Janeiro, Dept Neurol, Rio De Janeiro, RJ, BrazilUniv Fed Cariri, Barbalha, CE, BrazilUniv Fed Fluminense, Fac Med, Dept Neurol, Niteroi, RJ, BrazilUniv Estadual Campinas, Dept Neurol, Campinas, SP, BrazilUniv Regiao Joinville, Joinville, SC, BrazilUniv Fed Sao Paulo, Dept Neurol, Sao Paulo, SP, BrazilUniv Fed Uberlandia, Fac Med, Uberlandia, MG, BrazilHosp Moinhos De Vento, Porto Alegre, RS, BrazilUniv Fed Sao Paulo, Dept Neurol, Sao Paulo, SP, BrazilWeb of Scienc