Cervical nerve root synovial sarcoma in a child with chromosomal (X;18) translocation. Case report and review of the literature.

We report on an 11-year-old female with a history of cervicobrachialgia and progressive weakness of the right arm. Cervical spine MRI showed an enhancing heterogeneous intradural mass occupying the right C6-C7 foramen. She underwent a right C6-C7 foraminotomy with a complete macroscopic removal of the lesion. Pathological examination revealed a synovial sarcoma. Treatment was completed by chemotherapy and proton radiotherapy, and the girl remained free of symptoms for 3 years. After appearance of new symptoms, a local recurrence was confirmed, and despite aggressive treatment with salvage chemotherapy and radiotherapy, the disease progressed beyond medical control, and the child died, 6 years after diagnosis. Early recognition of this rare entity compared to its more benign differential diagnosis is crucial, as an aggressive management is needed.

Peripheral Nerve Neuropathy is Associated with a Glial Reaction in the Gracile Nucleus Associated with a regulation of the GABA transporter GAT-1

Allodynia (pain in response to normally non painful stimulation) and paresthesia (erroneous sensory experience) are two debilitating symptoms of neuropathic pain. These stem, at least partly, from profound changes in the non-nociceptive sensory pathway that comprises large myelinated neuronal afferents terminating in the gracile and cuneate nuclei. Further than neuronal changes, well admitted evidence indicates that glial cells (especially in the spinal cord) are key actors in neuropathic pain, in particular the possible alteration in astrocytic capacity to reuptake neurotransmitters (glutamate and GABA). Yet, the possibility of such a changed astrocytic scavenging capacity remains unexplored in the dorsal column pathway. The present study was therefore undertaken to assess whether peripheral nerve injury (spared nerve injury model, SNI) could trigger a glial reaction, and especially changes in glutamate and GABA transporters, in the gracile nucleus. SNI surgery was performed on male Sprague-Dawley rats. Seven days after surgery, rats were used for immunofluorescence (fixation and brain slicing), western-blot (fresh brain freezing and protein extraction) or GABA reuptake on synaptosomes. We found that SNI results in a profound glial reaction in the ipsilateral gracile nucleus. This reaction was characterized by an enhanced immunolabelling for microglial marker Iba1 as well as astrocytic protein GFAP (further confirmed by western-blot, p <0.05, n = 7). These changes were not observed in sham animals. Immunofluorescence and western-blot analysis shows that the GABA transporter GAT-1 is upregulated in the ipsilateral gracile nucleus (p <0.001; n = 7), with no detectable change in GAT-3 or glutamate transporters EAAT-1 and EAAT-2. Double immunoflurescence shows that GAT-1 and GFAP colocalize within the same cells. Furthermore, the upregulation of GFAP and GAT-1 were shown to occur all along the rostrocaudal axis of the gracile nucleus. Finally, synaptosomes from ipsilateral gracile nucleus show an increased capacity to reuptake GABA. Together, the data presented herein show that glial cells in the gracile nucleus react to neuropathic lesion, in particular through an upregulation of the GABA transporter GAT-1. Hence, this study points to role of an increased GABA transport in the dorsal column nuclei in neuropathic pain, calling attention to GAT-1 as a putative future pharmacological target to treat allodynia and paresthesia.

Patterns of neurovascular compression in patients with classic trigeminal neuralgia: A high-resolution MRI-based study.

PURPOSE: To describe the anatomical characteristics and patterns of neurovascular compression in patients suffering classic trigeminal neuralgia (CTN), using high-resolution magnetic resonance imaging (MRI). MATERIALS AND METHODS: The analysis of the anatomy of the trigeminal nerve, brain stem and the vascular structures related to this nerve was made in 100 consecutive patients treated with a Gamma Knife radiosurgery for CTN between December 1999 and September 2004. MRI studies (T1, T1 enhanced and T2-SPIR) with axial, coronal and sagital simultaneous visualization were dynamically assessed using the software GammaPlan?. Three-dimensional reconstructions were also developed in some representative cases. RESULTS: In 93 patients (93%), there were one or several vascular structures in contact, either, with the trigeminal nerve, or close to its origin in the pons. The superior cerebellar artery was involved in 71 cases (76%). Other vessels identified were the antero-inferior cerebellar artery, the basilar artery, the vertebral artery, and some venous structures. Vascular compression was found anywhere along the trigeminal nerve. The mean distance between the nerve compression and the origin of the nerve in the brainstem was 3.76±2.9mm (range 0-9.8mm). In 39 patients (42%), the vascular compression was located proximally and in 42 (45%) the compression was located distally. Nerve dislocation or distortion by the vessel was observed in 30 cases (32%). CONCLUSIONS: The findings of this study are similar to those reported in surgical and autopsy series. This non-invasive MRI-based approach could be useful for diagnostic and therapeutic decisions in CTN, and it could help to understand its pathogenesis.

Biblioteca de l’Escola Tècnica Superior d’Arquitectura de Barcelona: Informe d’actuacions 2012

Informe d'actuacions dutes a terme durant l'any 2012: Instal·lacions; Innovació Tecnológica; Col•leccions; Bibliotècnica; Serveis; Habilitats informacionals; Coneixement UPC; Accés a la cultura; Professionals; Organització i gestió.

Global transcriptional programs in peripheral nerve endoneurium and DRG are resistant to the onset of type 1 diabetic neuropathy in Ins2 mice.

While the morphological and electrophysiological changes underlying diabetic peripheral neuropathy (DPN) are relatively well described, the involved molecular mechanisms remain poorly understood. In this study, we investigated whether phenotypic changes associated with early DPN are correlated with transcriptional alterations in the neuronal (dorsal root ganglia [DRG]) or the glial (endoneurium) compartments of the peripheral nerve. We used Ins2(Akita/+) mice to study transcriptional changes underlying the onset of DPN in type 1 diabetes mellitus (DM). Weight, blood glucose and motor nerve conduction velocity (MNCV) were measured in Ins2(Akita/+) and control mice during the first three months of life in order to determine the onset of DPN. Based on this phenotypic characterization, we performed gene expression profiling using sciatic nerve endoneurium and DRG isolated from pre-symptomatic and early symptomatic Ins2(Akita/+) mice and sex-matched littermate controls. Our phenotypic analysis of Ins2(Akita/+) mice revealed that DPN, as measured by reduced MNCV, is detectable in affected animals already one week after the onset of hyperglycemia. Surprisingly, the onset of DPN was not associated with any major persistent changes in gene expression profiles in either sciatic nerve endoneurium or DRG. Our data thus demonstrated that the transcriptional programs in both endoneurial and neuronal compartments of the peripheral nerve are relatively resistant to the onset of hyperglycemia and hypoinsulinemia suggesting that either minor transcriptional alterations or changes on the proteomic level are responsible for the functional deficits associated with the onset of DPN in type 1 DM.

Caracterização fenológica e produtiva da variedade de uva Superior Seedless cultivada no Vale do São Francisco

A variedade Superior Seedless, devido não somente às excelentes características morfológicas, mas também ao agradável sabor de seus frutos, tem se destacado nos últimos anos como a principal uva sem sementes cultivada no Vale do São Francisco.Este trabalho teve por objetivo caracterizar o comportamento fenológico e produtivo da variedade de uva Superior Seedless cultivada no Vale do São Francisco. As avaliações foram realizadas em uma área comercial, durante o segundo semestre de 1999 e primeiro semestre de 2000. A variedade Superior Seedless apresentou ciclo médio de 94 dias, sendo que, quando a poda foi realizada no primeiro semestre, ocorreu uma antecipação da colheita em 14 dias. A produtividade foi muito baixa (5,3 t.ha-1), mas as características qualitativas como diâmetro e comprimento de baga, sólidos solúveis totais e acidez total titulável, atendem aos padrões exigidos pelo comercio internacional.

Análisis bibliométrico sobre el Aprendizaje Basado en Problemas (ABP) en la educación superior a partir de la base de datos ERIC

[spa]El estudio bibliométrico descriptivo que sigue presenta tendencias de investigación sobre el aprendizaje basado en problemas entre 1974 y 2009. Se sirve de la base de datos ERIC y trabaja con una muestra de 1007 documentos. Se analizan los registros a tenor de cuatro variables: el año de publicación o realización, la titulación, el área de conocimiento y la tipología de investigación. En cuanto a la producción científica, pueden diferenciarse tres fases: la primera, de 1974 a 1989, supone el inicio de las publicaciones y muestra escasa relevancia estadística; la segunda, de crecimiento, durante la década de los 90 del pasado siglo; y la última, de maduración, desde el año 2000 hasta el año 2009. La distribución de los registros por sectores de conocimiento destaca Ciencias de la Salud, Ciencias Sociales y Enseñanzas Técnicas. Las titulaciones en las que más ha proliferado el ABP son Medicina, Económicas, Empresariales, Pedagogía, Formación del Profesorado y el conjunto de las ingenierías.

Increased axonal regrowth of lesioned rat sciatic nerve by veratrylguanidine methane sulfonate.

Neurotrophic factors appear as essential factors for normal development and repair of the nervous tissue. Veratrylguanidine methane sulfonate, has been shown to induce important neurite outgrowth of cultured dorsal root ganglia isolated from newborn rats. Its action was similar to that of NGF and was found to be additive to that of NGF. In order to see if this compound was able to stimulate axonal growth in adult animals, we examined the effect of this substance on the regeneration of the lesioned sciatic nerve. Using histochemical, immunohistochemical and ultrastructural studies, it is shown that a single intraperitoneal injection of veratrylguanidine methane sulfonate significantly increases the axonal growth during repair of the adult rat sciatic nerve. The efficiency of this substance is explained by its good targeting and long life time in the sciatic nerve.

Spatial distribution of motor units recruited during electrical stimulation of the quadriceps muscle versus the femoral nerve.

INTRODUCTION: In this study we investigated differences in the spatial recruitment of motor units (MUs) in the quadriceps when electrical stimulation is applied over the quadriceps belly versus the femoral nerve. METHODS: M-waves and mechanical twitches were evoked using over-the-quadriceps and femoral nerve stimulation of gradually increasing intensity from 22 young, healthy subjects. Spatial recruitment was investigated using recruitment curves of M-waves recorded from the vastus medialis (VM) and vastus lateralis (VL) and of twitches recorded from the quadriceps. RESULTS: At maximal stimulation intensity (Imax), no differences were found between nerve and over-the-quadriceps stimulation. At submaximal intensities, VL M-wave amplitude was higher for over-the-quadriceps stimulation at 40% Imax, and peak twitch force was greater for nerve stimulation at 60% and 80% Imax. CONCLUSIONS: For the VM, MU spatial recruitment during nerve and over-the-quadriceps stimulation of increasing intensity occurred in a similar manner, whereas significant differences were observed for the VL. Muscle Nerve, 2013.

KIAA1985, a Protein Mutant in Charcot-Marie-Tooth Neuropathy, Links Peripheral Nerve Myelination to Endosomal Recycling Pathways

Charcot-Marie-Tooth neuropathy (CMT) represents a heterogenous group of inherited disorders of the peripheral nervous system. One form of autosomal recessive demyelinating CMT (CMT4C, 5q32) is caused by mutations in the gene encoding KIAA1985, a protein of so far unknown function. Here we show that KIAA1985 is exclusively expressed in Schwann cells. KIAA1985 is tethered to cellular membranes through an N-terminal myristic acid anchor and localizes to the perinuclear recycling compartment. A search for proteins that interact with KIAA1985 identified the small GTPase Rab11, a key regulator of recycling endosome functions. CMT4C-related missense mutations disrupt the KIAA1985/Rab11 interaction. Protein binding studies indicate that KIAA1985 functions as a Rab11 effector, as it interacts only with active forms of Rab11 (WT and Q70L) and does not interact with the GDP locked mutant (S25N). Consistent with a function of Rab11 in Schwann cell myelination, myelin formation was strongly impaired when dorsal root ganglion neurons were co-cultured with Schwann cells infected with Rab11 S25N. Our data indicate that the KIAA1985/Rab11 interaction is relevant for peripheral nerve pathophysiology and place endosomal recycling on the list of cellular mechanisms involved in Schwann cell myelination.

Características físicas e físico-químicas de um tipo de bacuri (Platonia insignis Mart.) com rendimento industrial superior

Efetuou-se a caracterização física do fruto e físico-química da polpa do bacuri CPATU 207-3, oriundo de uma planta-matriz do Banco de Germoplasma de Bacurizeiro da Embrapa Amazônia Oriental. Para a caracterização física foi utilizada uma amostra de 50 frutos, os quais foram individualmente analisados quanto aos seguintes aspectos: cor do epicarpo, formato, peso, comprimento, diâmetro, espessura da casca, volume da cavidade interna, número de sementes e de segmentos partenocárpicos, rendimentos porcentuais (p/p) de casca, polpa, sementes e do conjunto representado pela coluna placentária e óvulos abortados. A caracterização físico-química foi efetuada em três amostras de polpa congelada a 18ºC negativos, provenientes de frutos em completo estádio de maturação, coletados nos meses de janeiro, fevereiro e março de 2001, tendo sido consideradas as seguintes variáveis: teores de umidade, de sólidos totais e de sólidos solúveis totais (ºBrix), pH, acidez total e a relação sólidos solúveis totais/acidez total titulável. O bacuri CPATU 207-3 apresentou formato ovalado e com ápice ligeiramente pontiagudo, epicarpo de coloração amarelada, fruto de tamanho considerado médio e peso médio de 265,8 g. As principais características diferenciais desse tipo de bacuri são: a espessura da casca (0,75cm), o rendimento porcentual de polpa (27,7%) e o número de segmentos partenocárpicos (2,8 unidades/fruto). A polpa do bacuri CPATU 207-3 apresentou boas características físico-químicas, com 84,35% de umidade, 15,65% de sólidos totais, teor de sólidos solúveis totais de 14,53ºBrix, pH 3,34, acidez total titulável de 1,24% e relação ºBrix/acidez total titulável igual a 11,4. As características físicas e físico-químicas desse tipo de bacuri permitem sua utilização tanto para consumo na forma de fruta fresca como na forma industrializada. Nesse último caso, é particularmente indicado para fabricação de compota, em decorrência do elevado número de segmentos partenocárpicos que apresenta.

Anelamento e reguladores de crescimento: efeitos sobre as medidas biométricas e qualidade de cachos da videira 'Superior Seedless'

Com o objetivo de se obter maior tamanho de baga, peso médio de cacho e produtividade, os cachos da variedade Superior Seedless foram pulverizados com ácido giberélico (1 + 20 mg.L-1), bioestimulante Crop Set® nas doses de 0,1 e 0,2% e com ou sem anelamento no caule. Esses tratamentos foram aplicados de forma isolada ou combinados entre si. O trabalho foi conduzido durante o período 2001-2002 (dois ciclos de produção), no Campo Experimental de Bebedouro da Embrapa Semi-Árido em Petrolina-PE. O delineamento experimental foi em blocos ao acaso, com 12 tratamentos e três repetições, sendo duas plantas por parcela. Não foram observadas diferenças significativas entre os tratamentos nos dois ciclos de produção. Entretanto, quando o ácido giberélico foi associado ao Crop Set® 0,1% e anelamento, observou-se uma tendência de aumento no peso de cachos, bem como no peso e tamanho de bagas na safra de 2001. Os cachos tratados com ácido giberélico apresentaram engaços mais grossos e pesados, embora não se observem diferenças significativas entre os tratamentos. Algumas plantas submetidas ao anelamento apresentaram problemas de cicatrização, o que provocou a morte das mesmas, recomendando-se evitar a realização desta prática nas condições em que se realizou este trabalho.