Abstract Hereditary transthyretin amyloidosis with peripheral neuropathy (ATTRv-PN) is an autosomal dominant inherited sensorimotor and autonomic polyneuropathy with over 130 pathogenic variants identified in the TTR gene. Hereditary transthyretin amyloidosis with peripheral neuropathy is a disabling, progressive and life-threatening genetic condition that leads to death in ~ 10 years if untreated. The prospects for ATTRv-PN have changed in the last decades, as it has become a treatable neuropathy. In addition to liver transplantation, initiated in 1990, there are now at least 3 drugs approved in many countries, including Brazil, and many more are being developed. The first Brazilian consensus on ATTRv-PN was held in the city of Fortaleza, Brazil, in June 2017. Given the new advances in the area over the last 5 years, the Peripheral Neuropathy Scientific Department of the Brazilian Academy of Neurology organized a second edition of the consensus. Each panelist was responsible for reviewing the literature and updating a section of the previous paper. Thereafter, the 18 panelists got together virtually after careful review of the draft, discussed each section of the text, and reached a consensus for the final version of the manuscript. ATTRvPN ATTRv PN (ATTRv-PN 13 gene disabling lifethreatening life threatening 1 untreated decades transplantation 1990 countries Brazil developed Fortaleza 2017 paper Thereafter draft text manuscript 199 201 19 20 2

Resumo Polineuropatia amiloidótica familiar associada a transtirretina (ATTRv-PN) é uma polineuropatia sensitivo-motora e autonômica hereditária autossômica dominante com mais de 130 variantes patogênicas já identificadas no gene TTR. A ATTRv-PN é uma condição genética debilitante, progressiva e que ameaça a vida, levando à morte em ~ 10 anos se não for tratada. Nas últimas décadas, a ATTRv-PN se tornou uma neuropatia tratável. Além do transplante de fígado, iniciado em 1990, temos agora 3 medicamentos modificadores de doença aprovados em muitos países, incluindo o Brasil, e muitas outras medicações estão em desenvolvimento. O primeiro consenso brasileiro em ATTRv-PN foi realizado em Fortaleza em junho de 2017. Devido aos novos avanços nesta área nos últimos 5 anos, o Departamento Científico de Neuropatias Periféricas da Academia Brasileira de Neurologia organizou uma segunda edição do consenso. Cada panelista ficou responsável por rever a literatura e atualizar uma parte do manuscrito. Finalmente, os 18 panelistas se reuniram virtualmente após revisão da primeira versão, discutiram cada parte do artigo e chegaram a um consenso sobre a versão final do manuscrito. ATTRvPN ATTRv PN (ATTRv-PN sensitivomotora sensitivo motora 13 TTR debilitante vida 1 tratada décadas tratável fígado 1990 países Brasil desenvolvimento 2017 manuscrito Finalmente 199 201 19 20 2

BACKGROUND The lepromatous pole is a stigmatising prototype for patients with leprosy. Generally, these patients have little or no symptoms of peripheral nerve involvement at the time of their diagnosis. However, signs of advanced peripheral neuropathy would be visible during the initial neurological evaluation and could worsen during and after multidrug therapy (MDT). Disabilities caused by peripheral nerve injuries greatly affect these patients’ lives, and the pathophysiological mechanisms underlying nerve damage remain unclear. OBJECTIVES To evaluate the outcome of peripheral neuropathy in patients with lepromatous leprosy (LL) and persistent neuropathic symptoms years after completing MDT. METHODS We evaluated the medical records of 14 patients with LL who underwent nerve biopsies due to worsening neuropathy at least four years after MDT. FINDINGS Neuropathic pain developed in 64.3% of the patients, and a neurological examination showed that most patients had alterations in the medium- and large-caliber fibers at the beginning of treatment. Neurological symptoms and signs deteriorated despite complete MDT and prednisone or thalidomide use for years. Nerve conduction studies showed that sensory nerves were the most affected. MAIN CONCLUSIONS Patients with LL can develop progressive peripheral neuropathy, which continues to develop even when they are on long-term anti-inflammatory and immunosuppressive therapy. Generally diagnosis However . (MDT) lives unclear (LL 1 643 64 3 64.3 medium largecaliber large caliber treatment affected longterm long term antiinflammatory anti inflammatory (MDT 6 64.

ABSTRACT Castor bean is a very important crop, however, in Brazil it still has relatively low productivity, 0.47 Mg ha-1 as a mean. Besides that the Country has significant castor oil demand and depends on international commerce to complement its production. The solution of these problems goes through the development of genotypes highly productive and adapted to the producing regions. So, the objective of this research was to evaluate the productive potential and selection of castor progenies of the cultivar FCA-PB, resulting from three types of pollination, in two crops and in two localities of the State of São Paulo. Grain productivity was evaluated, and some genetic parameters were estimated. In relation to the 2005/2006 crop, the progeny 49 cultivated in the locality of São Manuel produced 4170.66 kg ha-1 of grains. The highest heritability coefficients were observed in the 2005/2006 crop in the locality of Araçatuba. Progenies 1, 5, 6, 8, 15, 18, 19, 21, 27, 31, 35, 36, 38, 45, 49, 55, 56 and 58 were selected. Genotype interaction with the location indicated productive materials and adapted to the state of São Paulo.

RESUMO Objetivo O objetivo do presente estudo foi obter dados da neurocondução do frênico e exame com agulha do diafragma em pacientes com asma de difícil controlee comparar com um grupo normal. Métodos O estudo consiste em realizar radiografia de tórax, espirometria, pressão máxima inspiratória e expiratória, neurocondução do nervo frênico e eletromiografia do músculo diafragma em 20 pacientes asmáticos de difícil controle e comparar com 27 controles. Resultados Encontramos redução da área do potencial de ação muscular composto do nervo frênico e a eletromiografia do musculo diafragma estava normal em todos os pacientes. Conclusão É possível que a redução da área do potencial de ação muscular composto do nervo frênico nos pacientes com asma de difícil controle associado a eletromiografia normal do músculo diafragma esteja relacionada as alterações da fibra muscular do mesmo devido à sobrecarga de atividade.

ABSTRACT Objective The aim of this study was to obtain data on phrenic neuroconduction and electromyography of the diaphragm muscle in difficult-to-treat asthmatic patients and compare the results to those obtained in controls. Methods The study consisted of 20 difficult-to-treat asthmatic patients compared with 27 controls. Spirometry, maximal inspiratory and expiratory pressure, chest X-ray, phrenic neuroconduction and diaphragm electromyography data were obtained. Results The phrenic compound motor action potential area was reduced, compared with controls, and all the patients had normal diaphragm electromyography. Conclusion It is possible that a reduced phrenic compound motor action potential area, without electromyography abnormalities, could be related to diaphragm muscle fiber abnormalities due to overload activity.

Os autores propuseram-se a realizar uma revisão sistemática em conceitos atuais sobre a hanseníase neural primária, consultando as seguintes bases bibliográficas on-line: MEDLINE, Lilacs/SciELO e Embase. Os estudos selecionados foram classificados conforme o grau de recomendação e o nível de evidência científica de acordo com o “Oxford Centre for Evidence-based Medicine”. Os seguintes temas foram revisados: investigações clínica e laboratorial cutâneas, ou seja, exame, esfregaço e biópsia de pele e reação de Mitsuda; investigação neurológica (anamnese, eletroneuromiografia e biópsia de nervo); investigação sorológica e testes moleculares, ou seja, testes sorológicos para detecção de um glicolipídio fenólico e reação de cadeia de polimerase (PCR) e tratamento (critérios de classificação para definição de tratamento específico, tratamento com esteroides e critérios de cura).

The authors proposed a systematic review on the current concepts of primary neural leprosy by consulting the following online databases: MEDLINE, Lilacs/SciELO, and Embase. Selected studies were classified based on the degree of recommendation and levels of scientific evidence according to the “Oxford Centre for Evidence-based Medicine”. The following aspects were reviewed: cutaneous clinical and laboratorial investigations, i.e. skin clinical exam, smears, and biopsy, and Mitsuda's reaction; neurological investigation (anamnesis, electromyography and nerve biopsy); serological investigation and molecular testing, i.e. serological testing for the detection of the phenolic glycolipid 1 (PGL-I) and the polymerase chain reaction (PCR); and treatment (classification criteria for the definition of specific treatment, steroid treatment, and cure criteria).

Quarenta e quatro pacientes com sinais de neuropatia periférica foram acompanhados no Ambulatório de Hanseníase da Fundação Oswaldo Cruz por período que variou de 4 meses até 4 anos, com o intuito de confirmar ou afastar o diagnóstico de neuropatia hanseniana. Os sintomas neurológicos apresentados foram hipoestesia (41), parestesia (28), espessamento neural (22), dor nos nervos (20), paresia (20), amiotrofia (8). Dez pacientes dos 44 tiveram o diagnóstico de hanseníase confirmado. A confirmação diagnóstica se deu através da biópsia de áreas hipoestésicas sem lesão dermatológica (3 pacientes), pelo aparecimento de estados reacionais (duas reações reversas e 4 neurites reacionais) e pelo aparecimento de lesão cutânea não reacional característica da forma "borderline" lepromatosa. O acompanhamento clínico regular dos pacientes sem diagnóstico no primeiro exame mostrou ser um método que permitiu o diagnóstico da forma neurítica pura da hanseníase, quando os métodos objetivos de diagnóstico não confirmarem de imediato a doença.

Forty-four patients with neuritic leprosy were individually followed for periods ranging from 4 months to almost 4 years for the purpose of ascertaining the presence and/ or absence of leprosy. The neural symptoms presented were sensory impairment (41), parasthesia (28), nerve enlargement (22), nerve tenderness (20), paresia (20), amyotrophy (8). Leprosy was diagnosed in ten out of the total number of patients studied. Leprosy was confirmed by the appearance of reactional neuritis (4), reversal reaction (2), biopsy of the hypoesthesic area (3) and the appearance of non-reactional cutaneous lesion. We reported an experience in the diagnosis of neuritic leprosy and its most frequent clinical presentation with which clinicians have to be acquainted. We can also state that the clinical follow-up was an effective strategy for the diagnosis of the disease when diagnostic facilities are not available or have not confirmed the diagnosis.