Schwannoma intraóseo en fémur, una presentación inusual. Reporte de caso / Intraosseous Schwannoma in femur, an unusual presentation. Case report

El Schwannoma o Neurilemoma es un tumor benigno de derivación neuroectodérmica que se origina en las células de Schwann, que constituyen la envoltura de los nervios. Es el tumor benigno más común de los nervios periféricos, tiene predisposición a originarse a partir de nervios periféricos sensoriales y puede presentarse como tumores en tejidos blandos. La presentación intraósea del Schwannoma es rara y corresponde al 0,2% de los tumores óseos primarios. Se presenta caso clínico de paciente masculino de 36 años de edad con tumor en cóndilo femoral medial de rodilla derecha de 4 años de evolución, cursando con dolor intermitente moderado a quien se le practicó el protocolo oncológico, imagenológico e histológico, de la Unidad de Oncología Ortopédica del estado Monagas. Se diagnosticó Schwannoma intraóseo, se procedió a realizar resección marginal y reconstrucción con alloinjerto y fijación con placa y tornillos con excelente evolución postoperatoria. El Schwannoma intraóseo es extraordinariamente raro y su ubicación en el fémur lo es aún más. El objetivo del presente trabajo es reportar un caso de Schwannoma Intraóseo, mostrar las estrategias para su diagnóstico y los tratamientos aplicados, así como la revisión de los datos al respecto existentes en la literatura(AU)

Schwannoma or Neurilemoma is a benign tumor of neuroectodermal derivation that originates in Schwann cells, which constitute the nerve sheath. It is the most common benign tumor of the peripheral nerves, has a predisposition to originate from peripheral sensory nerves, and can present as soft tissue tumors. The intraosseous presentation of Schwannoma is rare and corresponds to 0,2% of primary bone tumors. A clinical case of a 36-yearold male patient with a tumor in the medial femoral condyle of the right knee of 4 years of evolution is presented, presenting with moderate intermittent pain who underwent the oncological, imaging and histological protocol of the Oncology Unit. Monagas State Orthopedic. Intraosseous Schwannoma was diagnosed, marginal resection and allograft reconstruction and plate and screw fixation were performed with excellent postoperative evolution. Intraosseous Schwannoma is extraordinarily rare and its location in the femur is even more so. The objective of this paper is to report a case of Intraosseous Schwannoma, show the strategies for its diagnosis and the treatments applied, as well as the review of the existing data in the literature(AU)

Effect of Tacrolimus and Cyclosporine Immunosuppressants on Peripheral Nerve Regeneration: Systematic Review and Meta-analysis / Efeito dos imunossupressores tacrolimus e ciclosporina na regeneração de nervos periféricos: Revisão sistemática e metanálises

Abstract Peripheral nerve damage is an important cause of seeking medical attention. It occurs when the continuity of structures is interrupted and the propagation of nervous impulses is blocked, affecting the functional capacity of individuals. To assess the effects of the immunosuppressants tacrolimus and cyclosporine on the regeneration of peripheral nerves, a systematic review of the literature was carried out. The articles included were published until September 2018 and proposed to evaluate the effects of the immunosuppressants tacrolimus and cyclosporine on nerve regeneration and neuroprotection, available in the MEDLINE, EMBASE, Cochrane Library, Web of Science, Oxford Pain Relief Database, and LILACS databases. The research analysed a total of 56 articles, of which 22 were included in the meta-analysis. Statistical analysis suggests the protective effect of tacrolimus in the regeneration of the number of myelinated axons (95% confidence interval [CI]: 0.93-2.39; p< 0.01); however, such effect was not observed in relation to cyclosporine (95%CI: - 0.38-1.18; p» 0.08) It also suggests that there is a significant relationship between the use of tacrolimus and myelin thickness (95%CI» 2.00-5.71; p< 0. 01). The use of immunosuppressants in the regeneration of peripheral nerve damage promotes an increase in the number of myelinated axons in general, regardless of the administered dose. In addition, it ensures greater myelin thickness, muscle weight and recovery of the sciatic functional index. However, heterogeneity was high in most analyses performed.

Resumo As lesões nervosas periféricas são uma causa importante de busca por atendimento médico. Elas ocorrem quando há a interrupção da continuidade das estruturas e do bloqueio da propagação dos impulsos nervosos, afetando a capacidade funcional dos indivíduos. Para avaliar os efeitos dos imunossupressores tacrolimus e ciclosporina na regeneração de nervos periféricos, foi realizada uma revisão sistemática da literatura. Foram incluídos artigos publicados até setembro de 2018, que se propunham avaliar os efeitos dos imunossupressores tacrolimus e ciclosporina na regeneração nervosa e neuroproteção, disponíveis nas bases de dados MEDLINE, EMBASE, Cochrane Library, Web of Science, Oxford Pain Relief Database e LILACS. A pesquisa analisou um total de 56 artigos, dos quais 22 foram para metanálise. A análise estatística sugere o efeito protetor do tacrolimus na regeneração do número de axônios mielinizados (intervalo de confiança [IC] 95%: 0,93-2,39; p< 0,01); todavia tal efeito não foi observado em relação à ciclosporina (IC95%: - 0,38-1,18; p» 0,08). Ela também sugere haver uma relação significativa entre o uso do tacrolimus e a espessura da mielina (IC95%: 2,00-5,71; p< 0,01). O uso de imunossupressores na regeneração de lesão nervosa periférica promove um aumento no número de axônios mielinizados de forma geral, independentemente da dose administrada. Além disso, garante uma maior espessura da mielina, um maior peso muscular e restabelecimento do índice da função do nervo ciático. Todavia, a heterogeneidade foi alta na maioria das análises realizadas.

Avaliação para o manejo da úlcera neuropática associada à hanseníase: produção e validação de vídeo educativo / Evaluation for the management of leprosy-associated neuropathic ulcer: production and validation of educational video

A hanseníase é uma das maiores causas de complicações neuropáticas no mundo. Dentre estas, estão as úlceras neuropáticas, consideradas uma das causas mais comuns de amputações, morbidade e mortalidade, consequência de uma assistência à saúde embasada em procedimentos fragmentados com baixa resolutividade. Estudo de delineamento metodológico, com abordagem quali-quantitativa dos dados, que objetivou desenvolver um vídeo para intervenção educativa sobre avaliação para manejo da úlcera neuropática associada à hanseníase, a ser disponibilizado a enfermeiros. A pesquisa foi aprovada pelo Comitê de Ética em Pesquisa e foi realizada em 4 etapas: 1: Produção do roteiro e storyboard; 2:Validação do roteiro e storyboard; 3: Edição do vídeo e 4: Estudo piloto. A produção do conteúdo do roteiro e storyboard seguiu as recomendações do Ministério da Saúde e da Wound, Ostomy and Continence Nurses. A validação de conteúdo e aparência foi realizada por um comitê de especialistas. O instrumento apresentou os seguintes quesitos: objetivo; conteúdo; relevância; ambiente; funcionalidade, usabilidade e eficiência, os quais foram considerados validados a partir da concordância de 78% entre os avaliadores, calculada por meio do Índice de Validade de Conteúdo. Entre os especialistas na temática, houve maior prevalência do sexo feminino (88,9%), com idade entre 29 e 62 anos (média de 42,3 anos e desvio padrão de 11,0 anos), sendo a maior titulação acadêmica o doutorado (55,6%), e tempo de atuação profissional entre seis e 30 anos (média de 14,9 anos e desvio padrão de 8,2 anos). Dos especialistas em vídeo, a maioria era do sexo feminino (62,5%), com idade entre 30 e 46 anos (média de 37,5 anos e desvio padrão de 6,4 anos), com maior número de mestres (50%) e tempo de atuação profissional entre 2 e 24 anos (média de 10,8 anos e desvio padrão de 7,3 anos). Para todos os quesitos avaliados, obteve-se um Índice de Validade de Conteúdo superior ao mínimo preconizado. Após a adequação do roteiro e storyboard em face da validação, o vídeo foi gravado e, a partir de uma préedição, um estudo piloto (validação semântica) foi realizado por meio da técnica de grupo focal, com a participação de nove enfermeiros que atuavam na assistência à pessoa com hanseníase. Houve prevalência do sexo feminino (88,9%), com idade entre 29 e 50 anos e maior titulação acadêmica em nível de mestrado (55,6%), sendo que a maioria (55,6%) atuava na Estratégia Saúde da Família. A partir da análise de conteúdo do grupo focal, emergiram as categorias: dialogar sobre os objetivos propostos; identificar a aplicabilidade do vídeo na prática clínica do enfermeiro e valorizar a consulta de enfermagem na avaliação de pessoas com úlcera neuropática associada à hanseníase. Acredita-se que o vídeo educativo validado poderá auxiliar na realização de uma avaliação de enfermagem eficaz, segura e com qualidade à pessoa com úlcera neuropática decorrente da hanseníase

Leprosy is one of the major causes of neuropathic complications in the world. Among the complications most commonly found are neuropathic ulcers, considered one of the most common causes of amputations, morbidity and mortality in the world, as a consequence of health care based on fragmented procedures with low resolution. This is a methodological design study with qualitative and quantitative data, aiming to develop a video for educational intervention on evaluation for the management of neuropathic ulcer associated with leprosy, to be made available to nurses. The research was approved by the Research Ethics Committee and was carried out in 4 stages: 1: Production of the script and storyboard; 2: Script and storyboard validation; 3: Video editing and 4: Pilot study. The production of script content and storyboard followed the recommendations of the Ministry of Health and Wound, Ostomy and Continence Nurses. Validation of content and appearance was performed by a committee of experts. The instrument presented the following requirements: objective; content; relevance; environment; functionality, usability and efficiency, which were considered validated from the agreement of 78% between the evaluators, calculated through the Content Validity Index Among the specialists in the subject, there was a higher prevalence of female (88.9%), aged between 29 and 62 years (mean of 42.3 years and standard deviation of 11.0 years), the highest academic qualification being the doctoral degree (55.6%), and professional performance time between six and 30 years (mean of 14.9 years and standard deviation of 8.2 years). Of the video specialists, the majority were female (62.5%), aged between 30 and 46 years (mean of 37.5 years and standard deviation of 6.4 years), with a greater number of masters (50%) and time of professional activity between 2 and 24 years (mean of 10.8 years and standard deviation of 7.3 years). For all evaluated items, a Content Validity Index higher than the recommended minimum was obtained. After the adaptation of the script and storyboard in the face of validation, the video was recorded and, from a pre-edition, a pilot study (semantic validation) was performed through the focal group technique, with the participation of nine nurses who assisted the person with leprosy. There was a prevalence of females (88.9%), aged between 29 and 50 years, and a higher academic degree at the master's level (55.6%), with the majority (55.6%) working in the Family Health Strategy. From the content analysis of the focus group, the following categories emerged: dialogue about the proposed objectives; to identify the applicability of the video in the clinical practice of the nurse and to value the nursing consultation in the evaluation of people with neuropathic ulcer associated with leprosy. It is believed that the validated educational video can help in the performance of an effective, safe and quality nursing assessment to the person with neuropathic ulcer due to leprosy

Neurorrhaphy techniques: comparative stereological and experimental axonal study / Técnicas de neurorrafia: estudio experimental estereológico axonal comparativo

There is a great variety of injuries that affect peripheral nerves derived from acquired or congenital degenerative diseases affecting the central nervous system that cause loss of sensorimotor functions. The objective of this work was to perform an end-to-side or side-to-side experimental axonal stereological study in order to compare volume density of axons, endouneuro and myelin sheath (and muscle mass) in peroneal and tibial nerves, with anastomosis contact from 0.25 cm to 0.50 cm. After approval of the Ethics Committe, 20 male Wistar rats were divided into four groups of five rats each (G1= end-to-side neurorrhaphy; G2= side-to-side neurorrhaphy of 0.25 cm; G3= side-to-side neurorrhaphy of 0 cm and G4= Control of normality). After 180 days, fragments of peroneal and tibial nerves were collected for histological and stereological study. In comparative stereological experimental study between neurorraphies, the volume density of axons, myelin sheath of tibial and fibular nerves, as well as the post-surgical muscle mass, remains the same in end-to-side and side-to-side neurorraphies, regardless of contact area of anastomosis. It can be inferred, as surgical repair options, both end-to-side neurorrhaphy to recover and prevents atrophy of the endplate as side-to-side neurorraphy that is independent of the distance between the nerve stumps.

Gran variedad de lesiones atingen a los nervios periféricos, derivadas de enfermedades adquiridas o degenerativas congénitas que afectan la parte central del sistema nervioso y que ocasionan pérdida de funciones sensoriomotoras. El objetivo de ese trabajo fue realizar un estudio experimental estereológico axonal post neurorrafias termino-lateral o latero-lateral para comparar densidad de volumen de axones, endoneuro y vaina de mielina (así como masa muscular) en nervios fibular y tibial, con unión de contacto entre 0,25 cm y 0,50 cm. Tras la aprobación del comité de ética, fueran utilizados 20 ratones machos de la raza Wistar divididos en cuatro grupos de 5 ratones cada uno (G1= Neurorrafia término lateral; G2= Neurorrafia latero lateral de 0,25 cm; G3= Neurorrafia latero lateral de 0,50 cm y G4= Control). Posteriormente, fragmentos de los nervios tibiales y fibulares fueron procesados para estudios histológicos y estereológicos. En el estudio experimental estereológico comparativo entre neurorrafias termino-lateral y latero-lateral, la densidad de volumen de axones, endoneuro y vaina de mielina de nervios tibial y fibular y también la masa muscular post quirúrgica se mantuvo equitativa, independientemente del área de unión de contacto. Podemos inferir como opciones de reparación quirúrgica, que el tratamiento de la neurorrafia termino-lateral y latero-lateral previnen la atrofia de placa motora, independiente de la distancia entre los muñones nerviosos.

Neurofibroma Cantal Solitario: Reporte de Caso / Canthal Solitary Neurofibroma

Objetivos: presentar el caso de una paciente que desarrolló un neurofibroma solitario de localización cantal interna. Diseño de estudio: reporte de caso. Métodos: se reporta el caso de una paciente con un neurofibroma solitario localizado en el canto interno, a la vez que se presenta una breve revisión bibliográfica sobre esta patología infrecuente. Conclusiones: el neurofibroma solitario es una patología infrecuente de naturaleza benigna que puede comprometer el globo ocular, el párpado y la órbita, por lo que debe ser considerado dentro de los diagnósticos diferenciales de la patología tumoral de estas áreas anatómicas.

Objectives: to report the case of a patient with a solitary neurofibroma in the medial canthal area. Study design: case report. Methods: report the case of a patient with a solitary neurofibroma in the medial canthal area and review the pertinent literature. Conclusions: the solitary neurofibroma is a rare benign condition with the potential to compromise the eye, the eyelid and the orbit, so that should be considered in the differential diagnosis of tumoral pathology in these anatomic areas. Keywords: nerve sheath neoplasms, peripheral nervous system neoplasms, neurofibroma.

Clinical features and prognosis of prostate cancer with high-grade prostatic intraepithelial neoplasia

PURPOSE: To evaluate the clinical features and biochemical recurrence (BCR) in prostate cancer (PCa) with high-grade prostatic intraepithelial neoplasia (HGPIN). MATERIALS AND METHODS: We retrospectively analyzed the medical records of 893 patients who underwent a radical prostatectomy for PCa between 2011 and 2012 at Asan Medical Center; 752 of these patients who did not receive neoadjuvant or adjuvant therapy and were followed up for more than 1 year were included. The cohort was divided into two groups-patients with and without HGPIN-and their characteristics were compared. The Cox proportional hazards model was used to analyze factors affecting BCR. RESULTS: In total, 652 study patients (86.7%) had HGPIN. There were no significant differences in preoperative factors between the two groups, including age (p=0.369) and preoperative prostate-specific antigen concentration (p=0.234). Patients with HGPIN had a higher Gleason score (p=0.012), more frequent multiple tumor (p=0.013), and more perineural invasion (p=0.012), but no other postoperative pathologic characteristics were significantly different between the two groups. There were no significant differences in BCR (13.0% vs. 11.5%, p=0.665) and HGPIN was not associated with BCR (p=0.745). In multivariate analysis, only the T stage (p<0.001) was associated with BCR. CONCLUSIONS: PCa patients with HGPIN have a higher Gleason score, more frequent multiple tumors, and more perineural invasion than those without HGPIN. The presence of HGPIN is not an independent predictor of BCR.

Evaluación del sistema nervioso periférico por ultrasonido / Peripheral nervous system evaluation by ultrasound

Diseases of the peripheral nerves are common in the field of neurology, they are not infrequent to occur in association with orthopedic or rheumatic diseases, sometimes they can be confused or categorized as primary diseases of the musculoskeletal system. Ultrasound is a valuable and inexpensive tool to characterize the neural fibers and its components. New technologies help to localize, assess and perform dynamic maneuvers that enhance diagnostic accuracy in peripheral nerves. The aim of this article is to familiarize with the exploration of peripheral nerves. The typical test of nerves begins in the transverse plane, following the nerve from proximal to distal in the area of interest. Ultrasound is useful as a method when suspected pathology, as a monitoring tool and as part of precise interventional procedures...

Las enfermedades de los nervios periféricos son comunes en el campo de la Neurología, pueden presentarse en asociación con enfermedades ortopédicas o reumáticas, y son en ocasiones confundidas o categorizadas como enfermedades primarias del sistema musculoesquelético. El ultrasonido es una herramienta valiosa y de bajo costo que permite caracterizar la fibra neural. Con el avance tecnológico de los equipos actuales es mucho más fácil localizar, evaluar y realizar procedimientos invasivos en los nervios periféricos. El objetivo de este artículo es familiarizarse con la exploración de nervios periféricos. El examen típico comienza en el plano transverso, siguiendo al nervio desde proximal a distal en el área de interés. El ultrasonido tiene su utilidad como método diagnóstico de primera línea ante la sospecha de patología, como herramienta de seguimiento, así como parte de procedimientos intervencionistas precisos...

Manifestações músculoesqueléticas e auto-anticorpos em crianças e adolescentes com hanseníase / Musculoskeletal manifestations and autoantibodies in children and adolescents with leprosy

Introdução: A hanseníase é uma doença infecciosa crônica causada pelo Mycobacterium leprae. É considerada um dos maiores problemas de saúde pública nos países em desenvolvimento. Os principais sinais clínicos são manchas de pele com perda de sensibilidade e envolvimento de nervos periféricos. Manifestações musculoesqueléticas são descritas em adultos, mas este envolvimento é raramente descrito na população pediátrica. Objetivo: Avaliar envolvimento musculoesquelético e auto-anticorpos em pacientes pediátricos com hanseníase. Métodos: Foram avaliados 50 pacientes com hanseníase e 47 crianças e adolescentes saudáveis de acordo com manifestações musculoesqueléticas (artralgia, artrite e mialgia), síndromes dolorosas musculoesqueléticas (fibromialgia juvenil, síndrome de hipermobilidade articular benigna, síndrome miofascial e tendinite) e painel de auto-anticorpos e crioglobulinas. Escores de avaliação de saúde e tratamento foram realizados nos pacientes com hanseníase. Resultados: A frequência de manifestações musculoesqueléticas foi maior em pacientes com hanseníase comparada aos controles (14% vs. 0%, p=0,0012). Cinco pacientes com hanseníase tinham poliartrite assimétrica das pequenas articulações das mãos (10% vs. 0%, p=0,057). Comprometimentos da função do nervo, reação tipo I hansênica, e neuropatia silenciosa foram observados nos pacientes com hanseníase (p=0,0006; p=0,003; p=0,0059; respectivamente). Nenhum dos pacientes e controles apresentou síndromes de dor musculoesquelética e as frequências dos anticorpos e crioglobulinas foram semelhantes nos dois grupos (p > 0,05). Comprometimentos da função nervosa, reação hansênica tipo I e neuropatia silenciosa foram observados em pacientes com versus sem manifestações musculoesqueléticas (p=0,0036; p=0,0001; p=0,309; respectivamente), bem como subtipos de hanseníase multibacilar (86% vs. 42%, p=0,045). A escala visual analógica do médico (VAS), dos pacientes (VAS), de dor (VAS) e CHAQ foram maiores em...

Introduction: Leprosy is a chronic infectious disease caused by Mycobacterium leprae. It is considered one of major public health issue in developing countries. The important clinical signs of leprosy are hypopigmented or reddish localized skin lesions with loss of sensation and peripheral nerves involvement. Musculoskeletal manifestations were described in leprosy adult patients and these involvements were rarely described in pediatric leprosy population. Objective: To evaluate musculoskeletal involvement and autoantibodies in pediatric leprosy patients. Methods: 50 leprosy patients and 47 healthy children and adolescents were assessed according to musculoskeletal manifestations (arthralgia, arthritis and myalgia), musculoskeletal pain syndromes (juvenile fibromyalgia, benign joint hypermobility syndrome, myofascial syndrome and tendinitis) and a panel of autoantibodies and cryoglobulins. Health assessment scores and treatment were performed in leprosy patients. Results: The frequency of at least one musculoskeletal manifestation was significantly higher in leprosy patients compared to controls (14% vs. 0%, p=0.0012) and five leprosy patients had asymmetric polyarthritis of small hands joints (10% vs. 0%, p=0.057), Nerve function impairment, type I leprosy reaction and silent neuropathy were significantly observed in leprosy patients (p=0.0006; p=0.003; p=0.0059; respectively). None of the patients and controls presented musculoskeletal pain syndromes and the frequencies of all antibodies and cyoglobulins were similar in both groups (p>0.05). Further analysis of leprosy patients showed that the frequencies of nerve function impairment, type I leprosy reaction and silent neuropathy were significantly observed in patients with versus without musculoskeletal manifestations (p=0.0036; p=0.0001; p=0.309; respectively), as well as multibacillary subtypes in leprosy (86% vs. 42%, p=0.045). The median of physician visual analogue scale (VAS)...

Células cebadas en pulmón y nervio periférico en la intoxicación crónica con karwinskia humboldtiana en rata wistar: estudios histológico e histoquímico / Lung and peripheral nerve mast cells in chronic intoxication with karwinskia humboldtiana in wistar rat: histological and histochemical studies

Karwinskia humboldtiana (Kh) es un arbusto venenoso responsable de numerosos casos de intoxicación accidental en humanos. En estudios previos en nuestro laboratorio reportamos un incremento de células cebadas en nervio periférico (NP) durante la intoxicación con Kh, este hallazgo no ha sido reportado previamente en otros órganos durante esta intoxicación por lo que en el presente estudio buscamos la presencia de estas células en otros órganos, además de distinguir subpoblaciones de células cebadas mediante reacciones histoquímicas para la identificación de los gránulos de secreción. El objetivo de este estudio fue evaluar la presencia de células cebadas en órganos distintos al NP y diferenciar histoquímicamente la composición de sus gránulos. Se utilizaron 32 ratas Wistar, se dividieron en cuatro grupos (n= 8) en donde 5 ratas de cada grupo fueron intoxicadas y 3 fueron control no intoxicadas. A las ratas intoxicadas se les administraron por vía oral 3,5 g/kg del fruto seco y molido de Kh fraccionados en 5 dosis de 1,5; 0,5, 0,5; 0,5 y 0,5 g/kg los días 0, 3, 7, 10 y 14 respectivamente. Las ratas control solo recibieron agua. Cada grupo fue sacrificado a diferentes tiempos según la evolución de la parálisis. Se obtuvieron muestras de Hígado, Riñón, Pulmón y SNP, se procesaron hasta obtener bloques de parafina, se obtuvieron cortes y se tiñeron con azul de toluidina, PAS, Azul alciano/PAS y Azul alciano/Safranina. Se identificó la presencia de células cebadas en NP y pulmón con la tinción de azul de toluidina y se realizo un estudio morfométrico observando un incremento progresivo del número de células cebadas por grupo así como variaciones histoquímicas en sus gránulos en cada etapa y órgano analizado, lo que sugiere la participación de las células cebadas y sus secreciones en cada una de las etapas de la intoxicación crónica con el fruto maduro de Kh.

Karwinskia humboldtiana (Kh) is a poisonous shrub causing a number of accidental intoxications in humans. In previous studies in our laboratory, we reported an increased number of mast cells present in peripheral nerve of Kh intoxicated rats. This finding has not been reported in other organs of intoxicated animals. For this reason, in the present study we searched for mast cells in several organs, identifying mast cell subpopulations on the basis of different histochemical reactivity of their secretory granules. Thus the objective of the present study was to evaluate the presence of mast cells in organs other than peripheral nerve and, to distinguish mast cells by their granule content, applying histochemical reactions. 32 Wistar rats were divided into 4 groups (n=8). For each group, 5 rats were intoxicated with Kh and 3 received water only as a control.Intoxicated rats received 3.5 g/ Kg body weight of dry powder of Kh fruits, fractionated in 5 doses as follows 1.5, 0.5, 0.5, 0.5, 05 on days 0, 3,7,10,14 respectively. Control rats received water only. Each group was killed at different times during paralysis evolution. Samples of liver, kidney, lung and brain, were obtained and processed by routine technique until paraffin embedding. Sections were obtained and stained with toluidine blue, PAS, alcian blue/PAS and alcian blue/safranin. Mast cells infiltrates were observed in peripheral nerve and lung. Mast cells were counted. An increasing number of mast cells were recorded as well as variations in the histochemical pattern of their granules for each organ. These findings suggest a role for mast cells and their secretions in the intoxication with mature fruit of Kh.

Use of fine needle aspirate from peripheral nerves of pure-neural leprosy for cytology and PCR to confirm the diagnosis: A pilot study.

Background: The diagnosis of pure neural leprosy (PNL) remained subjective because of over-dependence of clinical expertise and a lack of simple yet reliable diagnostic tool. The criteria for diagnosis, proposed by Jardim et al., are not routinely done by clinicians in developing country as it involves invasive nerve biopsy and sophisticated anti-PGL-1 detection. We conducted a study using fi ne needle aspiration cytology (FNAC) coupled with Ziehl Neelsen staining (ZN staining) and Multiplex- Polymerase Chain Reaction (PCR) specifi c for M. leprae for an objective diagnosis of pure neural leprosy (PNL), which may be simpler and yet reliable. Aim: The aim of the study is to couple FNAC with ZN staining and multiplex PCR to diagnose pure neural leprosy patients rapidly, in simpler and yet reliable way. Methods: Thirteen patients of PNL as diagnosed by two independent consultants were included as case, and 5 patients other than PNL were taken as control in the study. Fine needle aspiration was done on the affected nerve, and aspirates were evaluated for cytology, ZN staining and multiplex- PCR. Results: Out of the 13 cases where fi ne needle aspiration was done, M. leprae could be elicited in the nerve tissue aspirates in 5 cases (38.4%) with the help of conventional acid-fast staining and 11 cases (84.6%) with the help of multiplex PCR. On cytological examination of the aspirates, only 3 (23%) cases showed specifi c epithelioid cells, whereas 8 (61.5%) cases showed non-specifi c infl ammation, and 2 (15.3%) cases had no infl ammatory cells. Conclusion: Our study demonstrates that in the fi eld of laboratory diagnosis of PNL cases, FNAC in combination with ZN staining for acid-fast bacilli (AFB) and Multiplex-PCR can provide a rapid and defi nitive diagnosis for the majority of PNL cases. FNAC is a less-invasive, outdoor-based and simpler technique than invasive nerve biopsy procedure. Thus, this study may enlighten the future path for easy and reliable diagnosis of PNL.

Thyrotoxic myo-neuropathy.

Myopathy is an important complication of thyrotoxicosis. Neuropathy is a less commonly reported complication, and is often subclinical. Here we report a patient of throtoxic myopathy with sub-clinical entrapment neuropathy. This case is reported to emphasise the importance of NCV to look for neuropathy.

Evaluación del efecto del tratamiento con ácido lipoico administrado a la rata Wistar intoxicada con el fruto de Karwinskia humboldtiana / Evaluation of the effect of treatment with lipoic acid administered to the Wistar rat intoxicated with the fruit of Karwinskia humboldtiana

La ingesta accidental de fruto de Karwinskia humboldtiana ocasiona una parálisis flácida, simétrica, progresiva y ascendente, similar al síndrome de Guillain-Barré. Evoluciona en el transcurso de 3 a 12 meses hasta su recuperación total, pero los casos graves terminan en la muerte por insuficiencia respiratoria. No existe un tratamiento específico. La lesión histopatológica descrita en nervio periférico de pacientes, y animales de experimentación corresponde a una desmielinización segmentaria acompañada de degeneración Walleriana. Una de las toxinas extraídas a partir de la semilla, la T-514, ocasiona un incremento de radicales libres in vitro. Los radicales libres se han relacionado con la desmielinización que se presenta en otros tipos de neuropatías como en la diabética. Ya que la lesión ultraestructural que se presenta en los modelos animales de diabetes es similar a la que se observa en la intoxicación experimental con fruto de K. humboldtiana, se decidió administrar un potente agente antioxidante, el ácido a-lipoico en un modelo de intoxicación crónica por fruto de K. humboldtiana. Sin embargo, no se observó mejoría sobre las manifestaciones clínicas evaluadas en los animales o sobre las lesiones histopatológicas presentes en el nervio periférico. Estos resultados sugieren que los radicales libres no son el mecanismo principal de lesión sobre el nervio periférico en la polineuropatía causada por K. humboldtiana.

The accidental ingestion of Karwinskia humboldtiana causes a flaccid, symmetrical, progressive and ascending paralysis, similar to Guillain-Barre syndrome. It evolves over the course of 3 to 12 months until full recovery, but severe cases end in death due to respiratory failure. There is no specific treatment. The histopathological lesions described in peripheral nerve of patients and in experimental animals, corresponds to segmental demyelination accompanied by Wallerian degeneration. One of the toxins extracted from the seed, T-514, causes an increase of free radicals in vitro. Free radicals have been associated to demyelination that occurs in other types of neuropathy such as diabetic neuropathy. Since the ultrastructural damage that occurs in animal models of diabetes is similar to that observed in experimental poisoning with the fruit of K. humboldtiana, we decided to administer a powerful antioxidant, a-lipoic acid, in a model of chronic poisoning due of K. humboldtiana. However, no improvement was observed on the clinical manifestations evaluated in animals or in the histopathological lesions in the peripheral nerve. These results suggest that free radicals are not the primary mechanism of injury on the peripheral nerve caused by K. humboldtiana.

Histopathological examination of nerve samples from pure neural leprosy patients: obtaining maximum information to improve diagnostic efficiency

Nerve biopsy examination is an important auxiliary procedure for diagnosing pure neural leprosy (PNL). When acid-fast bacilli (AFB) are not detected in the nerve sample, the value of other nonspecific histological alterations should be considered along with pertinent clinical, electroneuromyographical and laboratory data (the detection of Mycobacterium leprae DNA with polymerase chain reaction and the detection of serum anti-phenolic glycolipid 1 antibodies) to support a possible or probable PNL diagnosis. Three hundred forty nerve samples [144 from PNL patients and 196 from patients with non-leprosy peripheral neuropathies (NLN)] were examined. Both AFB-negative and AFB-positive PNL samples had more frequent histopathological alterations (epithelioid granulomas, mononuclear infiltrates, fibrosis, perineurial and subperineurial oedema and decreased numbers of myelinated fibres) than the NLN group. Multivariate analysis revealed that independently, mononuclear infiltrate and perineurial fibrosis were more common in the PNL group and were able to correctly classify AFB-negative PNL samples. These results indicate that even in the absence of AFB, these histopathological nerve alterations may justify a PNL diagnosis when observed in conjunction with pertinent clinical, epidemiological and laboratory data.

Eletroneuromiografia na avaliação das radiculopatias cervicais e lombossacrais / Electromyography in the evaluation of cervical and lumbosacral radiculopathy

A eletroneuromiografia (ENMG) é empregada como método de diagnóstico complementar no diagnóstico de radiculopatia desde 1950, contribuindo com importantes informações para o esclarecimento diagnóstico, planejamento do tratamento e acompanhamento evolutivo dos pacientes. A presente revisão baseada em evidências buscou referências com ênfase na indicação, sensibilidade, especificidade, reprodutibilidade e limitações do uso desse exame na avaliação das radiculopatias cervicais e lombossacrais. As referências apontam a ENMG como um exame bastante útil tanto na triagem quanto no diagnóstico diferencial na suspeita de radiculopatia cervical ou lombossacra, bem como na avaliação do grau e extensão da lesão, quando respeitadas as limitações da técnica.

Electromyography (EMG) has been employed as a complementary method in the diagnosis of radiculopathy since 1950, contributing important information for diagnostic clarification, treatment planning and patient follow-up. This evidence based review sought references with emphasis on the recommendation, sensibility, specificity, reproducibility and limitations of the use of this test in the evaluation of cervical and lumbosacral radiculopathy. The references point to EMG as being an extremely useful test both in the screening and in the differential diagnosis in the suspicion of cervical or lumbosacral radiculopathy, as well as in the evaluation of the degree and extent of the injury, when the limitations of the technique are considered.

Álcool e sistema nervoso central / Alcohol and central nervous system

Os efeitos patológicos do uso do álcool sobre o Sistema Nervoso Central (SNC) são bem conhecidos, embora nem todos tenham explicação satisfatória. Os sintomas podem advir de qualquer nível do neuroeixo incluindo o encéfalo, nervos periféricos e músculos. Traumatismos cranioencefálicos devido aos graves acidentes automobilísticos causados por motoristas embriagados também devem ser mencionados como consequência do álcool sobre o SNC. O fator genético é outro determinante importante do risco para a patogênese do grupo de sintomas que juntos são característicos ou indicativos da condição específica (alcoolismo), incluindo a predisposição individual para o alcoolismo.O objetivo deste texto é listar as complicações mais importantes do álcool sobre o SNC, importantes para o conhecimento do clínico. Enquanto a confirmação diagnóstica, o tratamento e o seguimento de várias das complicações mencionadas neste capítulo são normalmente de responsabilidade do neurologista, o clínico é frequentemente o primeiro a avaliar o paciente e deve estar suficientemente familiarizado com afecções do cérebro e da coluna espinhal para formular o diagnóstico e o tratamento inicial visando o controle ou a cura do problema; estas duas vertentes de abordagens têm provado alterar o provável curso de inúmeras afecções sobre o SNC, causados pelo abuso do álcool, especialmente a possibilidade de poder revertê-las.

The effects of pathologic patterns of alcohol use on the Central Nervous System (CNS) are a wellestablished fact, although not all have received a satisfactory explanation. The symptoms can be felt atany level of the neural axis, including the brain, peripheral nerves and muscles. Cranio-encephalic traumatism (CET) from severe car accidents caused by drunk drivers should also be mentioned as an unfortunate after-effect of alcohol abuse on the CNS. Genetic factors are another important determinant of the risk for the pathogenesis of the group of symptoms that together are characteristic or indicative of this specific condition (alcoholism), including aperson's inward disposition toward alcoholism. Our aim in this chapter is to list the most important complications of alcohol intake on the CNS, considered to be indispensable knowledge for all clinicians. Although diagnostic confirmation, treatment and follow-up of several of the complications mentioned in this chapter are usually within the province of the neurologist, the internist or general physician is oftenthe first to evaluate the patient and should be familiar enough with head and spine injury to formulate the initial diagnosis and treatment of the disorder by some remedial or curative process; this two-pronged approach has proved to involve a change in the forecast of the probable course of several affections of the CNS by alcohol abuse, especially of the possibility for recovery.

Assessment of perineural infiltration and spread of oral squamous cell carcinoma: A clinicohistopathologic study.

Background : As the tumor spreads through the pathway of least resistance, the present study was carried out to evaluate the presence of perineural infiltration and spread of oral squamous cell carcinoma (OSCC) along the perineural spaces in gingivobuccal sulcus tumors infiltrating into the mandible. AIMS and objectives : (1) To investigate the incidence of perineural invasion of OSCC along the inferior alveolar nerve and (2) to investigate the neurovascular bundle as a potential route of spread of OSCC. Materials and Methods : Twenty-six patients with histopathologically proven OSCC of the gingivobuccal sulcus with radiographic infiltration of the mandible were included. The surgical specimens were decalcified and serially sectioned. Each section was stained with hematoxylin and eosin and was screened for the presence of perineural invasion and spread. Results : Twenty-five specimens showed perineural infiltration but none of the cases showed perineural spread along the inferior alveolar canal. Also, not all cases showed any neurologic deficit. Follow-up of these cases showed early recurrence (6-8 months) in the study group. Conclusion : Perineural infiltration is present in OSCC but perineural spread along the inferior alveolar canal is absent. It is a bad prognostic indicator.

A Case of Lewis-Sumner Syndrome Showing Dramatic Improvement after Plasma Exchange

We report a patient with Lewis-Sumner syndrome (LSS) who showed an improvement only with plasma exchange (PE). The patient, 32-yr old man, had progressive multifocal motor-sensory deficits with persistent, multiple conduction blocks and marked slowing of NCVs. Nerve pathology supported a diagnosis of demyelinating neuropathy by revealing marked loss of myelinated fibers with inter- and intrafascicular variation. Although the patient was refractory to treatment with corticosteroid and intravenous immunoglobulin, PE produced a dramatic improvement. Our experience strongly proposes that PE should be tried for refractory LSS.

Focal and multifocal diabetic neuropathies

Diabetic neuropathy is the most common neuropathy in industrialized countries, with a remarkable range of clinical manifestations. The vast majority of the patients with clinical diabetic neuropathy have a distal symmetrical form that progress following a fiber-length dependent pattern, with predominant sensory and autonomic manifestations. This pattern of neuropathy is associated with a progressive distal axonopathy. Patients are exposed to trophic changes in the feet, pains and autonomic disturbances. Less often, diabetic patients may develop focal and multifocal neuropathy that includes cranial nerve involvement, limb and truncal neuropathies. This neuropathic pattern tends to occur after 50 years of age, mostly in patients with longstanding diabetes mellitus. The LDDP does not show any trend to improvement and either relentlessly progresses or remain relatively stable over years. Conversely the focal diabetic neuropathies, which are often associated with inflammatory vasculopathy on nerve biopsies, remain self limited, sometimes after a relapsing course.

A neuropatia diabética é a mais predominante das neuropatias nos países industrializados apresentando uma gama variável de manifestações clinicas. A maioria dos pacientes com neuropatia diabética apresenta uma forma simétrica distal que progride para um padrão fibra comprimento dependente com manifestações sensitivas e autonomicas. Este tipo de neuropatia é associado com uma axonopatia distal progressiva. Os pacientes apresentam modificações tróficas nos pés, dores e distúrbios autonômicos. Menos freqüentemente os pacientes diabéticos podem desenvolver neuropatia focal e multifocal que incluem envolvimento de nervos cranianos, tronco e membros inferiores. Este padrão de neuropatia é mais freqüente em pacientes com mais de 50 anos e com longa historia de diabetes. Este tipo de neuropatia fibra-comprimento dependente não apresenta melhora, progride lentamente ou permanece estável por vários anos. As neuropatias focais que são associadas freqüentemente com vasculopatias inflamatórias nas biópsias de nervo, permanecem auto limitadas por vezes com surtos de remissão.

Recent histopathological studies in leprosy, with particular reference to early diagnosis and leprous neuropathy.

In histopathological studies in leprosy, two important areas were identified in recently published work. They are early diagnosis and neuropathy. In histopathological examination, finding of M. leprae in tissues and/or granulomatous destruction of nerves are the two important findings to confirm the diagnosis. Immunopathological staining of M. leprae, PCR amplification of M. leprae antigen and S100 staining of Schwaann cells have considerably enhanced the sensitivity of histopathological diagnosis. If the two clinical findings such as hypopigmented patches with impaired sensation and thickened nerves accompanied by loss of sensation are the only ones that are taken into account for diagnosis, then a significant number of early patients will be missed. It is pointed out that biopsy examination of skin and nerves, when necessary, and skin-smear studies are indispensable diagnostic procedures. In the study of leprous neuropathy, there are several studies trying to decipher the entry of M. leprae into Schwann cells. The sharing of antigens between M. leprae and surface membrane of Schwann cells may be an important factor. However, there is much more to be learned in this area. In the control and prevention of neuritis, although corticosteroids administered along with multi-drug therapy was helpful, the benefit was not sustained.