Objective: To determine whether people with a Sardinian genetic background who live in the megacities of South America have a higher frequency of hypomania than residents of Sardinia. Methods: A community survey of Sardinian immigrants was carried out in four Brazilian metropoles (n=218) and Buenos Aires (n=306). The results were compared with those of a study involving a similar methodology (Mood Disorder Questionnaire [MDQ] as a screening tool) conducted in seven Italian regions, including a sub-sample from Sardinia. Results: There was a higher prevalence of lifetime hypomania among Sardinians living in the Brazilian metropoles than among those living in Sardinia. This result was also consistent with Sardinian immigrants in Buenos Aires. After stratification by sex and age, the lifetime prevalence of MDQ scores ≥ 8 among Sardinians in South-American megacities and Sardinia was 8.6% vs. 2.9%, respectively (p < 0.0001). Conclusions: The higher frequency of hypomania in migrant populations appears to favor an evolutionary view in which mood disorders may be a maladaptive aspect of a genetic background with adaptive characteristics.

RESUMO Caatinga sensu stricto é a vegetação típica do semiárido brasileiro, adaptada à forte sazonalidade das chuvas e às altas temperaturas. Possui alta riqueza de espécies e alto nível de diversidade beta. O objetivo do estudo foi documentar a flora e fitossociologia das plantas lenhosas e herbáceas de uma área de caatinga. Para isso, amostramos um hectare na Reserva Particular do Patrimônio Natural Fazenda Almas (Estado da Paraíba). A área apresentou diversidade florística de 114 espécies (34 lenhosas/80 herbáceas), semelhante a outros estudos realizados nessa vegetação. O componente lenhoso apresentou densidade absoluta de 4822 ind.ha-1 e área basal de 38,851 m2.ha-1. Para plantas herbáceas foi registrada uma variação na densidade, percentual de cobertura do solo e riqueza de espécies entre as estações seca e chuvosa. Apesar da alta biodiversidade, a caatinga continua passando por extensos processos de degradação ambiental. O presente trabalho documentou a estrutura de um trecho conservado de caatinga que pode ser uma referência para futuros projetos de restauração.

ABSTRACT The caatinga sensu stricto is a typical vegetation type of the Brazilian semiarid region and is adapted to strong seasonality in rainfall and high temperatures. It has a high species richness and a high level of beta diversity. The objective of this study was to document the flora and structure of woody and herbaceous plant assemblages of a site with caatinga vegetation. We sampled one hectare in the Reserva Particular do Patrimônio Natural Fazenda Almas (Paraíba State, Brazil). Our study recorded 114 species (34 woody/80 herbaceous), which is similar to other studies conducted in this vegetation type. The woody component presented density of 4822 ind.ha-1 and a basal area of 38,851 m2.ha-1. For the herbaceous plants, we recorded variation in density, percentage of soil cover, and species richness between the dry and rainy seasons. Despite the high biodiversity, the caatinga still undergoes extensive environmental degradation processes. The present study documented the structure of a conserved tract of caatinga that can be a reference for future restoration projects.

RESUMO Manifestações motoras e não motoras são comuns e incapacitantes nas paraparesias espásticas hereditárias (PEH). Toxina botulínica do tipo A (TB-A) é considerada eficaz no tratamento da espasticidade e pode melhorar a marcha nesses pacientes. Pouco se sabe sobre os efeitos da TB-A sobre sintomas não-motores. Objetivo avaliar a eficácia da TB-A sobre manifestações motoras e não-motoras nas PEH. Método trinta e três pacientes adultos com PEH foram avaliados antes e depois das aplicações de TB-A. Resultados A média de idade foi 41,7 ± 13,6 anos e havia 18 mulheres. A maioria dos pacientes portava a forma pura e o genótipo mais comum foi SPG4. Houve diminuição da espasticidade dos músculos adutores da coxa sem melhora da marcha. A pontuação da fadiga reduziu após as injeções. Conclusão As aplicações de TB-A não melhoraram a marcha nos pacientes mas a redução da fadiga foi significativa após o tratamento.

ABSTRACT Motor and non-motor manifestations are common and disabling features of hereditary spastic paraplegia (HSP). Botulinum toxin type A (Btx-A) is considered effective for spasticity and may improve gait in these patients. Little is known about the effects of Btx-A on non-motor symptoms in HSP patients. Objective To assess the efficacy of Btx-A on motor and non-motor manifestations in HSP patients. Methods Thirty-three adult patients with a clinical and molecular diagnosis of HSP were evaluated before and after Btx-A injections. Results Mean age was 41.7 ± 13.6 years and there were 18 women. Most patients had a pure phenotype and SPG4 was the most frequent genotype. The Btx-A injections resulted in a decrease in spasticity at the adductor muscles, and no other motor measure was significantly modified. In contrast, fatigue scores were significantly reduced after Btx-A injections. Conclusion Btx-A injections resulted in no significant functional motor improvement for HSP, but fatigue improved after treatment.

Abstract Background: Previous studies discussed the repeatability and variability in plantar pressure measurement, but a few considered different age groups. Here we determine within and between-days repeatability and variability of plantar pressure measurement during gait in participants from different age groups. Method: Plantar pressure was recorded in children, young adults and older adults walking at preferred speed in four non-consecutive days within one week. Data from 10 steps from each foot in each day were analyzed considering the different regions of the foot. Mean and peak plantar pressure and data variability were compared between the steps, foot regions and days. Results: To describe mean and peak pressure during gait in children and adults a single measurement can be enough, but elderly will requires more attention especially concerning peak values. Variability in mean pressure did not differ between age groups, but peak pressure variability differed across foot regions and age groups. Conclusion: One single observation can be used to describe plantar pressure during gait in children and adults. When the interest concerns older people, it might be pertinent to consider more than one day of assessment, especially when looking at peak pressure.

Resumen Para determinar la densidad de ensilado, los métodos más usados son los directos mientras métodos indirectos, que se puedan usar a nivel de finca, están aún siendo desarrollados y mejorados. El objetivo de este estudio fue determinar la correlación entre las densidades de ensilado de maíz determinadas con un método directo, y las determinadas con un método indirecto. Las mediciones se hicieron en 14 silos de maíz de fincas lecheras en 5 municipios del estado de Paraná, Brasil. El método directo consistió en el uso de un cilindro metálico con un filo cortante de dientes serrados unido a una motosierra para extraer una muestra del ensilado; la densidad se determinó con base en el volumen del cilindro y el promedio del peso de las muestras extraídas en 5 puntos. El método indirecto consistió en el uso de un penetrómetro digital para medir la resistencia a la penetración en 2 puntos adyacentes a los sitios donde se tomaron las muestras del método directo (10 lecturas por silo). Los datos se sometieron a un análisis de regresión polinomial que mostró una relación cuadrática positiva entre la resistencia a la penetración (medida en MPa) y la densidad del ensilaje con base en los valores de la masa del ensilado (kg/m3) tanto para la materia natural como la materia seca (R2 = 0.57 y R2 = 0.80, respectivamente). Se concluye que el penetrómetro fue un método indirecto razonablemente confiable y sencillo para determinar la densidad de la materia seca en ensilado de maíz. Para verificar estos resultados se requieren pruebas adicionales con este equipo en otros silos.

Abstract Several methodologies have been tested to evaluate silage density, with direct methods most popular, whereas indirect methods that can be used under field conditions are still in development and improvement stages. This study aimed to establish relationships between estimates of maize silage density determined using a direct and an indirect method, in an endeavor to provide an alternative to direct measurement for use in the field. Measurements were performed on maize silage in 14 silos. The direct method involved the use of a metal cylinder with a saw-tooth cutting edge attached to a chainsaw to extract a core of silage. Density of the silage was determined taking into consideration the cylinder volume and dry matter weight of silage removed at 5 points on the silage face. With the indirect method, a digital penetrometer was used to estimate silage density by measuring the penetration resistance at 2 points adjacent to the spots where the silage cores were taken, i.e. 10 readings per silo. Values of penetration resistance (measured in MPa) were correlated with the values of silage mass (kg/m3) obtained by direct measurement through polynomial regression analysis. A positive quadratic relationship was observed between penetration resistance and silage density for both natural matter and dry matter (R2 = 0.57 and R2 = 0.80, respectively), showing that the penetrometer was a reasonably reliable and simple indirect method to determine the density of dry matter in maize silage. Further testing of the machine on other silos is needed to verify these results.

Objective: To ascertain lifetime prevalence of positivity to a screening questionnaire for bipolar disorders (BD) in Sardinian immigrants to Argentina and residents of Sardinia and assess whether such positivity affects quality of life (QoL) in either group. Our hypothesis is that screen positivity for BD may be more frequent in immigrants. Methods: Observational study. Subjects were randomly selected from the membership lists of associations of Sardinian immigrants in Argentina. A study carried out in Sardinia using the same methodology was used for comparison. The Mood Disorder Questionnaire was used to screen for mania/hypomania and the Short-Form Health Survey-12 to measure QoL. Results: A higher prevalence of manic/hypomanic episodes was found in Sardinian immigrants to Argentina (p < 0.0001; odds ratio = 3.0, 95% confidence interval 1.87-4.77). Positivity at screening was associated with a lower QoL both in Sardinian immigrants to Argentina and in residents of Sardinia. Conclusions: To the best of our knowledge, this is the first study to show a higher lifetime prevalence of manic/hypomanic episodes in a general-population sample of individuals who migrated to a foreign country. Our results are in agreement with the hypothesis that hyperactive/novelty-seeking features may represent an adaptive substrate in certain conditions of social change.

A new approach based on multicomponent/domino combined reactions for the synthesis of γ-nitroesters promoted by a mixed aluminium-magnesium oxides derived from hydrotalcite-like material was developed. Different γ-nitroesters were synthesized in 15-95% yield using Meldrum's acid, aromatic aldehydes, nitromethane and different alcohols as reagents and solvents. The γ-aminobutyric acid derivatives, Phenibut and Baclofen, were prepared in 63 and 61% overall yield, respectively, through a two steps synthetic strategy. A mechanistic pathway was proposed based on the gas chromatography mass spectrometry (GC-MS) and electrospray ionization mass spectrometry (ESI-MS) experiments.

RESUMO Os autores apresentam uma revisão histórica sobre a ataxia spinocerebelar tipo 3/doença de Machado-Joseph (SCA3/DMJ), que representa a forma de SCA mais comum em nosso país, considerando a alta frequência de casos oriundos de famílias da cidade de Itajaí, no litoral de Santa Catarina, cidade com ascendência portuguesa-açoriana.

ABSTRACT The authors present a historical review of spinocerebellar ataxia type 3/Machado-Joseph disease (SCA3/MJD), the most common form of spinocerebellar ataxia in Brazil, and consider the high frequency of cases in families from Itajaí, a city on the coast of the state of Santa Catarina with a large population of Portuguese/Azorean descent.

RESUMO A exacerbação do impulso sexual (EIS) tem sido descrita em diversas doenças neurológicas. O objetivo deste estudo foi apresentar uma série de casos de EIS em pacientes com distúrbios do movimento. Métodos Quinze pacientes com diferentes formas de distúrbios do movimento (Doença de Parkinson, doença de Huntington, síndrome de Tourette, ataxia espinocerebellar tipo 3), foram avaliados na Unidade de Distúrbios de Movimento-Universidade Federal do Paraná. Resultados Entre os pacientes com doença de Parkinson houve sete casos com diferentes formas de EIS devido ao uso de agonista dopaminérgico, abuso de levodopa ou estimulação cerebral profunda (DBS). No grupo com distúrbios hipercinéticos, dois pacientes com doença de Huntington, dois com síndrome de Tourette, e quatro com ataxia espinocerebelar tipo 3 apresentaram EIS. Conclusões EIS nesses pacientes decorreu de diferentes etiologias, relacionadas com o tratamento dopaminérgico ou DBS na doença de Parkinson, parte do quadro clinico na doença de Huntington e síndrome de Tourette, e provavelmente relacionado com aspectos culturais em pacientes com ataxia espinocerebelar tipo 3.

ABSTRACT Increased of sexual arousal (ISA) has been described in different neurological diseases. The purpose of this study was present a case series of ISA in patients with movement disorders. Method Fifteen patients with different forms of movement disorders (Parkinson’s disease, Huntington’s disease, Tourette´s syndrome, spinocerebellar ataxia type 3), were evaluated in the Movement Disorders Unit of the Federal University of Paraná. Results Among Parkinson’s disease patients there were seven cases with different forms of ISA due to dopaminergic agonist use, levodopa abuse, and deep brain stimulation (DBS). In the group with hyperkinetic disorders, two patients with Huntington’s disease, two with Tourette’s syndrome, and four with spinocerebellar ataxia type 3 presented with ISA. Conclusions ISA in this group of patients had different etiologies, predominantly related to dopaminergic treatment or DBS in Parkinson’s disease, part of the background clinical picture in Huntington’s disease and Tourette’s syndrome, and probably associated with cultural aspects in patients with spinocerebellar ataxia type 3.

RESUMOOs autores apresentam uma série de casos incluindo oito pacientes com ataxia cerebellar de início muito tardio (média de 75,5 anos de idade) apresentando ataxia de marcha, associada à atrofia cerebelar.Método: 26 pacientes adultos com diagnóstico de ataxia cerebelar de início tardio idiopática foram analisados ambulatorialmente e acompanhados regularmente ao longo de 20 anos. Destes, oito pacientes idosos foram diagnosticados como provável ataxia cerebelar início muito tardio. Os pacientes foram submetidos a um exame neurológico completo, avaliação cognitive e oftalmológica assim como ressonância magnética do cérebro e eletroneuromiografia tambem foram realizados.Resultados: 62,5% dos pacientes eram do sexo masculino, com idade média de 81,9 anos, com média de idade de início aos 75,5 anos. Ataxia cerebelar predominante de marcha foi observada em todos os pacientes, bem como, a atrofia cerebelar na ressonância magnética cerebral. Comprometimento cognitivo leve e perda visual, devido à degeneração macular, foram observados em 50% dos casos. Coréia foi encontrada em 3 pacientes.Conclusão: Acreditamos que esta condição é semelhante à descrita por Marie-Foix-Alajouanine apresentando disartria leve, associada a ataxia de marcha, disfunção cognitiva e coréia.

ABSTRACTThe authors present a Brazilian case series of eight patients with idiopathic very-late onset (mean 75.5 years old) cerebellar ataxia, featuring predominantly gait ataxia, associated with cerebellar atrophy.Method: 26 adult patients with a diagnosis of idiopathic late onset cerebellar ataxia were analyzed in a Brazilian ataxia outpatient clinic and followed regularly over 20 years. Among them, 8 elderly patients were diagnosed as probable very late onset cerebellar ataxia. These patients were evaluated with neurological, ophthalmologic and Mini-Mental Status examinations, brain MRI, and EMG.Results: 62.5% of patients were males, mean age was 81.9 years-old, and mean age of onset was 75.5 years. Gait cerebellar ataxia was observed in all patients, as well as, cerebellar atrophy on brain MRI. Mild cognitive impairment and visual loss, due to macular degeneration, were observed in 50% of cases. Chorea was concomitantly found in 3 patients.Conclusion: We believe that this condition is similar the one described by Marie-Foix-Alajouanine presenting with mild dysarthria, associated with gait ataxia, and some patients had cognitive dysfunction and chorea.

A superexpressão das proteínas glicoproteína-P (Gp-P), proteína associada à resistência à múltiplas drogas 1 (MRP1) e p53 mutante e a enzima glutationa-S-transferase pi (GSTpi) está relacionada com resistência à quimioterapia em neoplasias humanas e caninas. Este estudo avaliou a expressão, por meio da imuno-histoquímica desses marcadores em espécimes de TVT caninos sem histórico de quimioterapia prévia (TVT1, n=9) e em TVT caninos que apresentaram resposta clínica insatisfatória ao sulfato de vincristina (TVT2, n=5). A porcentagem de espécimes positivos para Gp-P, MRP1, GSTpi e p53 foram, respectivamente 88,8%, 0%, 44,5% e 22,2% no grupo TVT1 e 80%, 0%, 80% e 0% no grupo TVT2. No TVT1, um espécime apresentou expressão positiva para três marcadores e quatro para dois marcadores. No TVT2, três espécimes expressaram a Gp-P e GSTpi. Em conclusão, os TVTs caninos estudados expressaram os quatro marcadores avaliados, no entanto apenas a Gp-P e GSTpi foram significativamente expressas, principalmente no citoplasmas e no citoplasma e no núcleo, respectivamente, tanto antes da quimioterapia quanto após à exposição ao sulfato de vincristina. Estudos futuros são necessários para demonstrar a função desses dois marcadores em conferir resistência à multiplas drogas (RMD) ou predizer a resposta a quimioterapia no TVT canino.

The overexpression of proteins P-glycoprotein (P-gp), multidrug resistance-associated protein (MRP1), mutant p53, and the enzyme glutathione-S-transferase (GSTpi) are related to resistance to chemotherapy in neoplasms. This study evaluated the expression of these markers by immunohistochemistry in two groups of canine TVT, without history of prior chemotherapy (TVT1, n=9) and in TVTs presented unsatisfactory clinical response to vincristine sulfate (TVT2, n=5). The percentage of specimens positively stained for P-gp, MRP1, GSTpi and p53 were, respectively 88.8%, 0%, 44.5% and 22.2% in TVT1 and 80%, 0%, 80% and 0% in TVT2. In TVT1, one specimen presented positive expression for three markers and four specimens for two markers. In TVT2, three specimens expressed P-gp and GSTpi. In conclusion, the canine TVTs studied expressed the four markers evaluated, but just P-gp and GSTpi were significantly expressed, mainly at cytoplasm and cytoplasm and nuclei, respectively, either before chemotherapy as after vincristine sulfate exposure. Future studies are needed to demonstrate the function of these two markers in conferring multidrug resistance (MDR) or predict the response to chemotherapy in canine TVT.

O professor Jean-Martin Charcot é considerado o professor mais importante da Neurologia e também o chefe da Escola de Neurologia de Salpêtrière. Em um quadro célebre pintado por André Brouillet e apresentado no Salão de 1887, sob o título "Uma Lição Clínica na Salpêtrière", o professor Charcot apresenta um caso de histeria a um grande público composto de médicos e intelectuais de renome. Cópias desse quadro também estão disponíveis para venda na loja do Museu da Escola de Medicina de Paris e são frequentemente utilizadas em palestras ministradas por neurologistas em todo o mundo. No entanto, nessas reproduções, Gilles de la Tourette e Charles Féré estão em posições invertidas. Esta nota histórica alerta sobre esse pequeno engano em algumas das reproduções da famosa pintura de Brouillet, a fim de que mais confusão seja evitada.

Professor Jean-Martin Charcot is considered the most important professor of Neurology and also the head of the Salpêtrière School of Neurology. In a famous picture painted by André Brouillet and presented at the Salon of 1887, under the title "A clinical lesson at the Salpêtrière", Professor Charcot presents a case of hysteria to a large audience of physicians and renowned intellectuals. Copies of this guided picture are also available for sale at the shop of the Museum of the School of Medicine of Paris and are frequently used in lectures by neurologists worldwide. However, in these reproductions, Gilles de la Tourette's and Charles Féré's positions are inverted. This historical note sheds some light on this little mistake in some of the reproductions of Brouillet's famous painting, so that further confusion can be avoided.

Os autores apresentaram uma revisão histórica sobre o professor MacDonald Critchley, no seu 15º ano de falecimento.

The authors presented a historical review on Professor MacDonald Critchley, 15 years after his death.

Potamotrygon signata é uma espécie de raia de água doce endêmica da bacia do rio Parnaíba, Brasil. Após a sua descrição original, apenas citações em listas sistemáticas foram registradas na literatura e a biologia da espécie permanece desconhecida, incluindo os hábitos alimentares. Este estudo teve como objetivo caracterizar a dieta de P. signata com base na análise do conteúdo estomacal, e fornecer informações preliminares sobre a variabilidade intraespecífica da dieta entre os sexos e segundo os estádios de maturidade. O conteúdo estomacal de 56 espécimes de P. signata foi analisado. A identificação taxonômica dos itens alimentares revelou a presença de 13 ordens de presas, incluindo insetos, moluscos, crustáceos e peixes teleósteos. O Índice de Importância Relativa (IRI%) indicou P. signata como uma espécie insetívora, com predominância de larvas de Diptera (60.64%) e ninfas de Ephemeroptera (34.68%). Diferenças na dieta entre os sexos foram observadas, bem como entre os indivíduos maduros e imaturos. A IRI% das fêmeas mostrou uma ocorrência similar de Diptera e Ephemeroptera (47.12% e 47.86%, respectivamente), enquanto para os machos, Ephemeroptera foi o principal item (79.56%). Indivíduos imaturos mostraram um predomínio de Diptera (76.20%), enquanto indivíduos maduros apresentaram uma ocorrência similar de Diptera (46.95%) e Ephemeroptera (47.23%). As diferenças sexuais e ontogenéticas observadas na dieta podem estar relacionadas a diferentes necessidades nutricionais de machos e fêmeas, e à variação de aspectos morfológicos do aparelho bucal e dentição de machos e fêmeas e de indivíduos imaturos e maduros. A dieta essencialmente insetívora desta espécie possivelmente é uma vantagem adaptativa para esta espécie no ambiente semiárido da Caatinga, onde a disponibilidade de peixes como presa pode sofrer grandes variações sazonais.

Potamotrygon signata is an endemic freshwater stingray species in the Parnaíba River basin, Brazil. After its original description, only citations in systematic lists were recorded in the literature and the biology of the species remains unknown, including the feeding habits. The aim of this study is to characterize the overall diet of P. signata based on analysing stomach contents and to provide preliminary information on intraspecific diet variability between sexes and maturity stages. The stomach contents of 56 specimens of P. signata were analyzed. The taxonomic identification of food items showed the presence of 13 prey orders, including insects, mollusks, crustaceans and teleost fish. The Index of Relative Importance (IRI%) indicated P. signata as an insectivorous species, with a dominance of Diptera larvae (60.64%) and Ephemeroptera nymphs (34.68%). Differences in diet were observed between sexes, as well as between mature and immature individuals. The IRI% of females showed a similar occurrence of Diptera and Ephemeroptera (47.12% and 47.86%, respectively), whereas for males, Ephemeroptera was the main item (79.56%). Immature individuals showed a dominance of Diptera (76.20%) while mature individuals showed a similar occurrence of Diptera (46.95%) and Ephemeroptera (47.23%). The observed sexual and ontogenetic differences in diet may be related to distinct nutritional requirements of males and females, and to the variation of morphological aspects of the oral apparatus and dentition of males and females and of immature individuals and adults. The essentially insectivorous diet of the species is possibly adaptively advantageous in the Caatinga semi-arid environment, where the availability of fish as prey may be subject to strong seasonal variation.

Estudos neurossonológicos, especialmente o Doppler transcraniano (DTC) e o duplex transcraniano codificado a cores (DTCC), apresentam elevados níveis de especificidade e sensibilidade quando utilizados como exames complementares no diagnóstico de morte encefálica (ME). Um grupo de peritos do Departamento Científico de Doppler transcraniano da Academia Brasileira de Neurologia criou uma força-tarefa de forma a determinar os critérios neurossonológicos para os seguintes aspectos no diagnóstico de ME no Brasil: metodologia do DTC; metodologia do DTCC; treinamento e habilidades em Neurossonologia; diagnóstico de parada circulatória encefálica e documentação do exame para a ME. Os resultados deste encontro foram apresentados neste artigo.

Neurosonological studies, specifically transcranial Doppler (TCD) and transcranial color-coded duplex (TCCD), have high level of specificity and sensitivity and they are used as complementary tests for the diagnosis of brain death (BD). A group of experts, from the Neurosonology Department of the Brazilian Academy of Neurology, created a task force to determine the criteria for the following aspects of diagnosing BD in Brazil: the reliability of TCD methodology; the reliability of TCCD methodology; neurosonology training and skills; the diagnosis of encephalic circulatory arrest; and exam documentation for BD. The results of this meeting are presented in the current paper.