Rara presentación de un linfoma espinal: informe de caso y diagnóstico mediante resonancia magnética / Rare presentation of a spinal lymphoma: case report and diagnosis by magnetic resonance imaging

El linfoma primario del sistema nervioso central es una forma de enfermedad extraganglionar originada en el cerebro, la leptomeninges, la médula espinal o los ojos. Los tumores espinales son neoplasias de baja prevalencia y pueden causar una morbimortalidad neurológica considerable. El linfoma aislado que surge dentro del conducto dural es la forma menos común de linfoma primario del sistema nervioso central: representa aproximadamente el 1% de los casos y se observa más a menudo en el contexto de diseminación secundaria que como el sitio primario de origen. Los síntomas son inespecíficos y dependen del nivel espinal involucrado. La presentación es insidiosa e incluye dorsalgia, debilidad y dificultad progresiva para la deambulación. La resonancia magnética es la modalidad de elección para búsqueda de lesiones dentro del conducto espinal/raquídeo, en pacientes que presentan síntomas neurológicos. El tratamiento quirúrgico no resulta útil, y el objetivo principal de la cirugía es conocer el diagnóstico histológico. (AU)

A primary central nervous system lymphoma is a form of extranodal disease originating in the brain, leptomeninges, spinal cord, or eyes. Spinal tumors are low-prevalence neoplasms and can cause considerable neurological morbidity and mortality. An isolated lymphoma emerging within the dural canal is the rarest form of primary central nervous system lymphoma: it accounts for approximately 1% of cases occurring more often in the context of secondary dissemination than as the primary site of origin. Symptoms are nonspecific and depend on the spinal level involved. The presentation is insidious and includes dorsalgia, weakness, and progressive difficulty in ambulatory function. MRI is the modality of choice to search for lesions within the spinal/rachial canal in patients presenting with neurological symptoms. Surgical treatment is not helpful, and the main objective of surgery is to know the histological diagnosis. (AU)

Primary central nervous system T-cell lymphoma in children and adolescents: a clinicopathological analysis of five cases / 中华病理学杂志

Objective: To study the clinicopathological characteristics, and further understand primary central nervous system T-cell lymphoma (PCNSTCL) in children and adolescents. Methods: Five cases of PCNSTCL in children and adolescents were collected from December 2016 to December 2021 at the First Affiliated Hospital of Zhengzhou University. The clinicopathological characteristics, immunophenotypic, and molecular pathologic features were analyzed, and relevant literatures reviewed. Results: There were two male and three female patients with a median age of 14 years (range 11 to 18 years). There were two peripheral T-cell lymphomas, not otherwise specified, two anaplastic large cell lymphoma, ALK-positive and one NK/T cell lymphoma. Pathologically, the tumor cells showed a variable histomorphologic spectrum, including small, medium and large cells with diffuse growth pattern and perivascular accentuation. Immunohistochemistry and in situ hybridization showed CD3 expression in four cases, and CD3 was lost in one case. CD5 expression was lost in four cases and retained in one case. ALK and CD30 were expressed in two cases. One tumor expressed CD56 and Epstein-Barr virus-encoded RNA. All cases showed a cytotoxic phenotype with expression of TIA1 and granzyme B. Three cases had a high Ki-67 index (>50%). T-cell receptor (TCR) gene rearrangement was clonal in two cases. Conclusions: PCNSTCL is rare, especially in children and adolescents. The morphology of PCNSTCL is diverse. Immunohistochemistry and TCR gene rearrangement play important roles in the diagnosis.

Melanocytic lesions of the central nervous system: a case series / Lesiones melanocíticas del sistema nervioso central: una serie de casos

ABSTRACT Background: Melanocytic lesions of the central nervous system (CNS) are an infrequent, broad and diverse group of entities, both benign and malignant, found in all age groups, with imaging findings ranging from well-circumscribed focal lesions to diffuse leptomeningeal involvement. On MRI, they are usually distinguished by a high signal on T1WI sequences, given the paramagnetic effect of melanin, thus making it difficult to differentiate among them. Objective: To describe the imaging and epidemiological characteristics of a retrospective series of CNS melanocytic lesions. Methods: MR images of 23 patients with CNS melanocytic lesions diagnosed between January 2012 and June 2018 were analyzed. Results: Most patients were female (14/23; 61%), with a median age of 47 years (range: 3 weeks to 72 years). The primary melanocytic lesions accounted for 8/19 cases (42.1%), which included neurocutaneous melanosis, meningeal melanocytomas and primary malignant melanomas. Secondary melanocytic lesions (metastatic) accounted for 10/19 cases (52.6%). There was one case of a tumor with secondary melanization, from a melanocytic neuroectodermal tumor of infancy. There were also four cases of primary ocular melanomas. The most frequent findings were the cerebral location, high T1WI signal and marked contrast-enhancement. Conclusions: The present review describes the wide variety of melanocytic lesions that could affect the CNS, emphasizing the MRI characteristics. Knowledge of the imaging, clinical and epidemiological characteristics of CNS melanocytic lesions is essential for their correct interpretation, given the significant overlap between lesion features and the variable prognosis.

RESUMEN Antecedentes: Las lesiones melanocíticas del sistema nervioso central (SNC) corresponden a un grupo infrecuente, amplio y diverso de entidades, tanto benignas como malignas, encontradas en todos los grupos etarios, con hallazgos imagenológicos que van desde lesiones focales bien circunscritas hasta un compromiso leptomeníngeo difuso. A la RM se distinguen por la alta señal en la secuencia T1WI, dado el efecto paramagnético de la melanina, haciendo difícil la diferenciación entre ellas. Objetivo: Describir las características epidemiológicas y de de una serie retrospectiva de lesiones melanocíticas del SNC. Métodos: Revisión de imágenes de RM de 23 pacientes con lesiones melanocíticas del SNC diagnosticadas entre enero de 2012 y junio de 2018. Resultados: La mayoría de los pacientes fueron mujeres (14/23; 61%), con edades comprendidas entre las 3 semanas de vida hasta los 72 años. Las lesiones melanocíticas primarias representaron 8/19 (42,1%), incluyendo: melanosis neurocutáneas, melanocitomas meníngeos y melanomas malignos primarios. Las lesiones melanocíticas secundarias (metastásicas) representaron 10/19 casos (52,6%). Hubo un caso de tumor con melanización secundaria (tumor neuroectodermico melanocítico de la infancia). Se incluyeron cuatro casos de melanomas oculares primarios. Los hallazgos más frecuentes fueron la localización cerebral, el aumento de señal T1 y el acentuado realce con el gadolinio. Conclusiones: Se describe la amplia variedad de lesiones melanocíticas encontradas en el SNC, enfatizando sus características a la RM. El conocimiento de sus características imagenológicas, clínicas y epidemiológicas es fundamental para su correcta interpretación, dado la notable superposición entre las presentaciones de las lesiones y lo variable de sus pronósticos.

Central nervous system high-grade neuroepithelial tumor with BCOR alteration: a short report

Central nervous system high-grade neuroepithelial tumors with BCOR alteration are rare. Currently, there are only 24 cases reported in the literature. These tumors are characterized by a change involving the BCOR gene and have a poor prognosis. Studies are needed to improve the current therapy and outcomes of these neoplasms. This case report describes the clinical history of a patient with this disease and aims to contribute to the current knowledge about this new entity.

Caracterização epidemiológica das neoplasias do sistema nervoso central em pacientes da região Norte do Brasil / Epidemiological characterization of central nervous system tumors in patients from Northern Brazil

Introdução: A incidência de neoplasias que acometem o Sistema Nervoso Central (SNC) tem aumentado gradativamente no mundo. No Brasil, as neoplasias encefálicas primárias são classificadas como a sétima causa de morte entre as neoplasias malignas. O objetivo do presente estudo foi caracterizar o perfil epidemiológico dos pacientes acometidos por neoplasias do SNC em um estado do Norte do Brasil.Métodos: Estudo retrospectivo, onde 196 prontuários, entre 2012 e 2016, de dois hospitais na região Norte do Brasil foram analisados.Resultados: O glioblastoma multiforme foi predominante (30,6%) entre as neoplasias primárias, seguido pelo astrocitoma (12,2%). As neoplasias secundárias, identificadas como metástases encefálicas, corresponderam a 29,9% da amostra, principalmente derivadas de neoplasias do pulmão e mama. A gravidade histológica das lesões neoplásicas foi mais frequente nos homens (p= 0,01). Foi observado que as neoplasias ocorreram com maior frequência a partir da 4º década de vida, exceto nos casos de astrocitoma e meduloblastoma, que foram detectadas principalmente em crianças e adultos jovens.Conclusão: A caracterização dos casos de neoplasias do SNC é de importante para a compreensão da situação atual deste problema de saúde pública na região norte do Brasil.

Introduction: The incidence of central nervous system (CNS) cancer has gradually increased worldwide. In Brazil, primary brain tumors are the seventh leading cause of death among malignant tumors. The objective of the present study was to characterize the epidemiological profile of patients with CNS cancer from a state in northern Brazil.Methods: This retrospective study analyzed 196 medical records between 2012 and 2016 from two hospitals in northern Brazil.Results: Glioblastoma multiforme was predominant (30.6%) among primary tumors, followed by astrocytoma (12.2%). Secondary cancer, defined as brain metastases, accounted for 29.9% of the sample and was mostly associated with lung and breast cancer. The histological severity of neoplastic lesions was more frequent in men (p = 0.01). Cancer occurred more frequently after the fourth decade of life, except in cases of astrocytoma and medulloblastoma, which mostly affected children and young adults.Conclusion: The characterization of CNS tumors is important for understanding the current situation of this public health problem in northern Brazil.

The value of CXorf67 and H3K27me3 for diagnosing germ cell tumors in central nervous system / 中华病理学杂志

Objective: To investigate immunohistochemical patterns of CXorf67 and H3K27me3 proteins in central nervous system germ cell tumors (GCTs) and to assess their values in both diagnosis and differential diagnosis. Methods: A total of 370 cases of central nervous system GCTs were collected from 2013 to 2020 at Huashan Hospital of Fudan University, Shanghai, China. The expression of CXorf67, H3K27me3 and commonly-used GCT markers including OCT4, PLAP, CD117, D2-40, and CD30 by immunohistochemistry (EnVision method) was examined in different subtypes of central nervous system GCTs. The sensitivity and specificity of each marker were compared by contingency table and area under receiver operating characteristic (ROC) curve. Results: Of the 370 cases there were 282 males and 88 females with a mean age of 19 years and a median age of 17 years (range, 2-57 years). Among the GCTs with germinoma, the proportions of male patients and the patients with GCT located in sellar region were both higher than those of GCTs without germinoma (P<0.05), respectively. CXorf67 was present in the nuclei of germinoma and normal germ cells, but not in other subtypes of GCT. H3K27me3 was negative in germinoma, but positive in the nuclei of surrounding normal cells and GCTs other than germinoma. In the 283 GCTs with germinoma components, the expression rate of CXorf67 was 90.5% (256/283), but no cases were positive for H3K27me3. There was also an inverse correlation between them (r2=-0.831, P<0.01). The expression rates of PLAP, OCT4, CD117 and D2-40 were 81.2% (231/283), 89.4% (253/283), 73.9% (209/283) and 88.3% (250/283), respectively. In 63 mixed GCTs with germinoma components, the expression rate of CXorf67 was 84.1% (53/63), while all cases were negative for H3K27me3. The expression rates of PLAP, OCT4, CD117 and D2-40 were 79.4% (50/63), 79.4% (50/63), 66.7% (42/63) and 87.3% (55/63), respectively. The 6 markers with largest area under ROC curve in ranking order were H3K27me3, CXorf67, D2-40, OCT4, PLAP and CD117 (P<0.05). Conclusions: CXorf67 and H3K27me3 have high sensitivity and high specificity in diagnosing germinoma. There is a significant inverse correlation between them. Therefore, they can both be used as new specific immunohistochemical markers for the diagnosis of GCTs.

Magnetic Resonance Imaging Manifestations of Rosai-Dorfman Disease in Central Nervous System / 中国医学科学院学报

Objective To investigate the clinical and magnetic resonance imaging(MRI) manifestations of Rosai-Dorfman disease(RDD) in central nervous system. Method The clinical and MRI data of 5 cases of RDD in central nervous system confirmed by pathology in the PLA General Hospital were analyzed retrospectively. Results The 5 cases included 4 males and 1 female,aged(39.8±21.7) years on average.Among them,4 cases were located in the intracranial area and 1 case in the thoracic spinal canal.The lesion showed isointense signal on T1 weighted image and iso,slight-hypo,and slight-hyperintense signals on T2 weighted image,and it presented intensively homogeneous enhancement in contrast-enhanced MRI.Two cases showed compressed brain area with edema around the left parietal and left frontotemporal dura,thickening and enhancement in the adjacent dura,and dural tail sign.Three cases presented bone destruction in adjacent diploe and thoracic vertebrae.One case showcased slight-hypo perfusion of the left parietal dura in arterial spin labeling. Conclusions RDD lesion usually appears as iso,slight hypo and slight hyper-intense signals on T2 weighted image and presents intensively homogeneous enhancement in contrast-enhanced MRI.The disease may involve the adjacent bone and the lesion shows slight hypo-perfusion on perfusion images.The MRI manifestations of RDD are characteristic,which are helpful for preoperative diagnosis and evaluation of RDD.

Isolated cerebral mucormycosis masquerading as a tumor in an immunocompetent patient

Mucormycosis is an opportunistic fungal disease that commonly presents as cutaneous or rhinocerebral infections associated with immunocompromised states. It may exceptionally present as isolated involvement of the brain with a varied clinical presentation, which may be difficult to diagnose early, leading to increased mortality. Herein, we report the case of a 42-year-old immunocompetent female with left-sided limb weakness and a history of recurrent vomiting and headache for the last two years. Clinically, glioma was suspected, but histopathological examination revealed a few broad aseptate fungal hyphae. As no other organ was involved, the diagnosis of isolated cerebral mucormycosis was rendered. Reporting this case, we show an unusual presentation of a central nervous system mucormycosis masquerading a tumor in an immunocompetent patient. The case also highlights the importance of a careful histopathological examination to avoid missing the presence of occasional fungal hyphae. Ideally, recognition of fungal hyphae in the brain, during intraoperative consultation, can prompt brain tissue culture for definitive diagnosis and early empirical antifungal therapy, which may prove life-saving.

Profile and management of central nervous system malformations in a tertiary hospital

Background: Central Nervous System (CNS) malformations rank among the commonest malformations. These may be identified in utero or noticed immediately after birth. Most studies showed Myelomeningocoele, a Neural Tube Defect (NTD) to be the commonest. Severe defects have a significant impact on long term disability. While some are preventable, most are surgically managed. Objective: To study the profile and management of CNS malformation in a tertiary hospital. Method: we undertook a 3-year retrospective study of the profile and surgical management, including the postoperative complications among Neonates admitted into the Special Care Baby Unit (SCBU). Data on place of delivery, Gender, Gestational age, Mode of delivery, Prenatal Diagnosis (Ultrasound Scan), types of malformations, types of surgeries, and postoperative complications were retrieved. Result: We found 71 patients with CNS anomalies (31.8% of all congenital anomalies), in delivery 10 (14.1%), out delivery 46(64.8%). Male=43, females=28. Term gestation 66 (93%), preterm 3(4.2%), post-term 2(2.8%). Delivered by Vagina (SVD) in 65(91.6%), Caesarean section 4(5.6%), and undocumented in 4(5.6%). Prenatal diagnosis in only 3(4.2%). Myelomeningocoele was 35(49.3%), meningocoele was 9(12.7%), Hydrocephalus was 18(25.3%), anencephaly was 1(1.4%), occipital Encephalocoele was 6(8.5%), Sincipital Encephalocoele was 2(2.8%). Operated on 53(75%), LAMA/lost to follow was 13(18%), preoperative death was 5(7%). Excision and closure of Neural Tube Defect were 34(64.2%), VP Shunt was 13(24.5%), Excision and closure of occipital Encephalocoele were 5(9.4%) craniofacial excision and repair of Sincipital Encephalocoele was 1(1.9%). CSF leak in 4(28.6%), wound infection in 3(21.4%), shunt infection and obstruction in 2(14.3%) each. meningitis, hypertrophied scar, and pseudo meningocoele in 1(7.1%) each. Conclusion: The commonest CNS anomaly is a Myelomeningocoele a preventable condition, Sincipital Encephalocoele is not common in our environment. Concomitant treatment of hydrocephalus averts post excision CSF Leak

Amiloidosis sistémica, a propósito de un caso de autopsia / SYstemic Amyloidosis. About an autopsy case

During history, amylidosis was observed associated to a great variety of inflammatory diseases, and due to this, appeared the term "secondary amyloidosis". The forms of sudden presentation without any apparent cause are classified as "primary amyloidosis", and also the localized amyloidosis was characterized, the same as the heredity variant. At present, three main grops are recognized as systemic amyloidosis: amyloidosis of light chains, the amyloidosis associated to the seric protein A, and the hereditary form. Systemic amyloidosis can involve practically any organ system, being the most commonly affected the heart and the kidney, which therefore determine the clinical evolution and the prognosis of the patient. The aim of this report, was to present a case of autopsy of systemic amyloidosis with involvement of the Central Nervous System, considering besides, the great extension of the disease in our patient

Tumores hipofisarios, casuística de Anatomía Patológica / Pituitary tumors: anatomopathological reports

This study examines a description of pituitary tumors considering an anatomopathological casuistic. The study of the tumors of the Central Nervous System (CNS) include the pituitary gland, located in the sella turcica. The pathology of the sellar region is represented by the adenomas, tumors of slow development with or without endocrine secretion, that usually involve the population of young adults. The aim of this report, was to describe the casuistic of the " J. Fernandez Hospital" between the years 2000 through 2017. A retrospective review was performed and 234 samples of the sellar region were processed. Mean age was 42 years with a range of 17 to 77 years. Sex distribution was 57% women and 43 % men. Of these, 77% of the cases resulted adenomas. The conclusions obtained in the study are detailed.

Effects of Brazilian scorpion venoms on the central nervous system

In Brazil, the scorpion species responsible for most severe incidents belong to the Tityus genus and, among this group, T. serrulatus, T. bahiensis, T. stigmurus and T. obscurus are the most dangerous ones. Other species such as T. metuendus, T. silvestres, T. brazilae, T. confluens, T. costatus, T. fasciolatus and T. neglectus are also found in the country, but the incidence and severity of accidents caused by them are lower. The main effects caused by scorpion venoms - such as myocardial damage, cardiac arrhythmias, pulmonary edema and shock - are mainly due to the release of mediators from the autonomic nervous system. On the other hand, some evidence show the participation of the central nervous system and inflammatory response in the process. The participation of the central nervous system in envenoming has always been questioned. Some authors claim that the central effects would be a consequence of peripheral stimulation and would be the result, not the cause, of the envenoming process. Because, they say, at least in adult individuals, the venom would be unable to cross the blood-brain barrier. In contrast, there is some evidence showing the direct participation of the central nervous system in the envenoming process. This review summarizes the major findings on the effects of Brazilian scorpion venoms on the central nervous system, both clinically and experimentally. Most of the studies have been performed with T. serrulatus and T. bahiensis. Little information is available regarding the other Brazilian Tityus species.(AU)

Ethanol intake and toxicity: in search of new treatments / Ingesta de etanol y toxicidad: en búsqueda de nuevos tratamientos

Prolonged alcohol consumption has consequences on the liver, producing necrotic precipitates and fibrosis, on the pancreas, causing the pancreatic acini to atrophy and destroying insulin-producing cells, and on the central nervous system (CNS), causing the gray and white matter in the frontal lobes of the brain and cerebellum to atrophy. Generally, alcohol is metabolized via oxidative pathways, where the enzymes alcohol dehydrogenase and aldehyde dehydrogenase participate during its metabolization in the liver and CNS, or via non-oxidative pathways during its metabolization in the pancreas. Ethanol metabolism can produce oxidative stress and tissue damage mediated by free radicals, causing morphological and functional alterations in the liver. In the pancreas, it can cause progressive and irreversible damage affecting the endocrine and exocrine functions, a result of the activation of the stellate cells, which are activated directly by alcohol, causing pancreatic fibrosis. In the CNS ethanol can bind directly to proteins, nucleic acids and phospholipids to develop its pathogenesis. The effects produced by alcohol can be counteracted by supplementation with antioxidants, which reduce the inflammation and areas of focal necrosis in the liver, inhibit the activation of pancreatic stellate cells, and reduce oxidative stress in the CNS. Additionally, in order to reduce the negative effects associated with alcohol consumption, recent studies have suggested the administration of antioxidants as a treatment strategy.

El consumo prolongado de alcohol tiene consecuencias en hígado, produciendo precipitados necróticos y fibrosis; en páncreas, provocando atrofia del acino pancreático y destrucción de las células productoras de insulina, y en Sistema Nervioso Central (SNC) generando atrofia de la sustancia gris y blanca en lóbulos frontales del cerebro y cerebelo. En general, el metabolismo del alcohol se consigue mediante las vías oxidativas, donde participan las enzimas alcohol-deshidrogenasa y aldehído deshidrogenasa durante su metabolización en hígado y SNC; o bien, mediante las vías no oxidativas durante su metabolización en páncreas. El metabolismo del etanol es capaz de producir estrés oxidativo y daño tisular mediado por radicales libres, causando alteraciones morfológicas y funcionales del hígado; en el páncreas, puede causar daño progresivo e irreversible afectando las funciones endocrinas y exocrinas de este órgano producto de la activación de las células estrelladas que son activadas directamente por el alcohol generando fibrosis pancreática; mientras que, en SNC se puede unir directamente a proteínas, ácidos nucleicos y fosfolípidos para desarrollar su patogenia. Los efectos producidos por el alcohol pueden contrarrestarse mediante la suplementación con antioxidantes, que reducen la inflamación y las zonas de necrosis focal en el hígado, inhiben la activación de células pancreáticas estrelladas, y reducen el estrés oxidativo en SNC. Asimismo, para reducir los efectos negativos asociados al consumo de alcohol, estudios recientes han propuesto la administración de antioxidantes como estrategia terapéutica.

Acidúria L2-hidroxiglutárica: circulação do gene em cães da raça Staffordshire Bull Terrier no Brasil em 2008-2015 / L2-hydroxyglutaric aciduria: circulation of the gene in Staffordshire Bull Terrier dogs in Brazil (2008-2015)

O objetivo do trabalho foi identificar a presença no Brasil do gene mutante L2HGDH em cães da raça Staffordshire Bull Terrier (SBT). Para tanto foi feito o teste genético em 76 cães provenientes de diferentes regiões do Brasil, no período de 2008 a 2015, sendo encontrados 55 animais (72,37%) livres do gene mutante L2-HGDH ou homozigotos dominantes, e 21(27,63%) portadores do gene mutante ou heterozigotos. Não foi encontrado nenhum animal homozigoto recessivo (afetado), porém pode-se observar que o gene circula no Brasil e que cães afetados podem aparecer.(AU)

The aim of this study was to identify the presence of a mutation in the L2-hydroxyglutarate dehydrogenase (L2-HGDH) gene in Staffordshire bull terriers in Brazil. Genetic testing was done in 76 dogs from different regions of the country, from 2008 to 2015. Fifty-five dogs (72.37%) were free of the mutant gene L2HGDH or homozygous-dominant, and 21 (27.63%) were carriers for the mutant gene or heterozygous. No homozygous recessive dogs (affected) were found, however, it is worth noting that the gene circulates in Brazil and that affected dogs can appear.(AU)

Aged Lewis rats exposed to low and moderate doses of rotenone are a good model for studying the process of protein aggregation and its effects upon central nervous system cell physiology / Ratos Lewis idosos expostos a baixa e moderada doses de rotenona são um bom modelo para estudar o processo de agregação proteica e seus efeitos sobre a fisiologia celular do sistema nervoso central

ABSTRACT Cell physiology is impaired before protein aggregation and this may be more relevant than inclusions themselves for neurodegeneration. The present study aimed to characterize an animal model to enable the analysis of the cell biology before and after protein aggregation. Ten-month-old Lewis rats were exposed either to 1 or 2 mg/kg/day of rotenone, delivered subcutaneously through mini-pumps, for one month. Hyperphosphorylated TAU, alpha-synuclein, amyloid-beta peptide and protein carbonylation (indicative of oxidative stress) were evaluated in the hippocampus, substantia nigra and locus coeruleus through immunohistochemistry or western blot. It was found that 2 mg/kg/day rotenone increased amyloid-beta peptide, hyperphosphorylation of TAU and alpha-synuclein. Rotenone at 1mg/kg/day did not alter protein levels. Protein carbonylation remained unchanged. This study demonstrated that aged Lewis rats exposed to a low dose of rotenone is a useful model to study cellular processes before protein aggregation, while the higher dose makes a good model to study the effects of protein inclusions.

RESUMO A fisiologia celular está prejudicada antes da agregação proteica podendo ser mais importante para a neurodegeneração do que as próprias inclusões. Assim, o objetivo deste estudo é caracterizar um modelo animal para analisar os mecanismos e efeitos da agregação proteica. Ratos Lewis com 10 meses de idade foram expostos a rotenona (1 ou 2 mg/kg/dia), administrada subcutaneamente, utilizando minibombas osmóticas. Os níveis de peptídeo beta-amiloide, TAU hiperfosforilada, alfa-sinucleína e proteínas carboniladas (indicativo de estresse oxidativo) foram avaliados por imunohistoquímica e western blot no hipocampo, substância negra e locus coeruleus. Foi demonstrado que 2 mg/kg/dia de rotenona promoveu aumento do peptídeo beta-amiloide, hiperfosforilação da TAU e alfa-sinucleína. Já 1 mg/kg/dia de rotenona não alterou os níveis dessas proteína nessas regiões. As proteínas carboniladas não se alteraram. Foi demonstrado que ratos Lewis idosos expostos a baixas doses de rotenona são modelo de estudo dos processos celulares antes da agregação proteica, enquanto 2 mg/kg/dia de rotenona permite estudos sobre os efeitos da agregação proteica.

Empleo de la radioterapia en el tratamiento a los tumores del sistema nervioso central en niños y adolescentes / Use of radiotherapy in treating central nervous system tumors of children and adolescents

INTRODUCCIÓN: los tumores del sistema nervioso central en los niños y adolescentes constituyen en Cuba la tercera localización en orden de frecuencia de todas las neoplasias; el tratamiento consiste en cirugía, seguida de la irradiación en la mayoría de los casos, y en algunos tipos histológicos, se añade la quimioterapia, o los anticuerpos monoclonales. OBJETIVO: valorar el empleo de la radioterapia en el tratamiento a los tumores del sistema nervioso central en niños y adolescentes. MÉTODOS: se trata de una serie de 98 niños y adolescentes entre 3 y 18 años de edad que fueron irradiados en el Instituto Nacional de Oncología y Radiobiología por padecer: 32 casos tumores del tallo cerebral (32,6 %), 19 meduloblastomas (19,4 %), 11 astrocitomas grado I-II (11,2 %), 9 astrocitomas III-IV y glioblastomas multiformes (gliomas de alto grado, 9,2 %), 6 craneofaringiomas (6,1 %), 6 tumores primitivos neuroectodérmicos (6,1 %), 5 oligodendrogliomas (5,1 %), 4 ependimomas (4,1 %), 3 germinomas (3,1 %), 2 pineoblastomas (2,1 %) y 1 caso de gliomatosis (1 %). Los pacientes fueron irradiados con aceleradores lineales, máscaras termoplásticas de inmovilización, planificación en 3D, conformada, o intensidad modulada, controles dosimétricos y de imágenes; en niños pequeños, se utilizó anestesia. RESULTADOS: 47 pacientes se encuentran vivos (47,9 %), con una tasa de supervivencia de 5 años de 50,2 %, y una mediana de 5,34 años. CONCLUSIONES: la radioterapia resulta ser un arma terapéutica eficaz para tumores del sistema nervioso central en niños y adolescentes.

INTRODUCTION: central nervous system tumors of children and adolescents are the most frequent location of neoplasias in Cuba, their treatment involves surgery followed by radiation in most cases, and chemotherapy or monoclonal antibodies in some histological types of tumors. OBJECTIVE: to evaluate the use of radiotherapy in treating central nervous system tumors of children and adolescents. METHODS: a series of 98 children and adolescents aged 3 to 18 years were treated with radiation at the National Institute of Oncology and Radiobiology including 32 brainstem tumors(32.6 %), 19 medulloblastomas (19.4 %), 11 grade I- II astrocytomas (11,2 %), 9 grade III-IV astrocytomas and multiform glioblastomas (high grade gliomas, 9.2 %), 6 craniopharyngiomas (6.1 %), 6 primitive neuroectodermal tumors (6.1 %), 5 oligodendrogliomas (5.1 %), 4 ependymonas (4.1 %), 3 germinomas (3.1 %), 2 pineoblastomas (2.1 %) and one case of gliomatosis (1 %). The patients were irradiated with linear accelerators, thermoplastic masks of immobilization, conformal 3D planning or modulated intensity, dosimetric and imaging controls whereas anesthesia was used in infants. RESULTS: forty seven patients survived (47.9 %) with a survival rate of 5 years (50.2 %) and a median of 5.34 years. CONCLUSIONs: radiation therapy proves to be an effective therapeutic tool for the treatment of central nervous tumors in children and adolescents.

Meningiomas em cães: aspectos clínicos, histopatológicos e imuno-histoquímicos / Meningiomas in dogs: clinical, histopathological and immunohistochemical aspects

As neoplasias no sistema nervoso central (SNC) de animais de companhia são frequentemente diagnosticadas, no entanto dados sobre prevalência são escassos. O objetivo deste estudo foi avaliar retrospectivamente a ocorrência de neoplasias primárias de SNC em cães atendidos em um Hospital-Escola Veterinário e descrever aspectos clínicos, histopatológicos e imuno-histoquímicos dos tumores mais frequentes. Quatorze casos (prevalência de 0,27%) de neoplasias primárias de SNC foram identificados no período de 1998 a 2013 e destes, 11 tiveram o diagnóstico de meningiomas. A idade média dos animais com meningioma foi 10 anos, sendo machos (7/11) e a raça Boxer (3/11) os mais afetados. Sete meningiomas eram espinhais e quatro intracranianos, sendo os principais sinais clínicos alteração na locomoção e convulsões, respectivamente. Metástase pulmonar ocorreu em dois casos. Em seis animais com meningioma espinhal foi realizada a mielografia, sendo que em um também foi realizada a tomografia. Em todos os casos os exames foram efetivos na visualização de desvio ou interrupção da coluna de contraste, com alterações sugestivas da presença de massa. Em cinco animais realizou-se cirurgia exploratória visando a confirmação da suspeita clínica ou retirada da massa, sendo que a sobrevida variou de 85 a 960 dias. Na avaliação histopatológica, os meningiomas foram classificados em transicional (4/11), meningotelial (2/11), papilar (2/11), angiomatoso (1/11), microcístico (1/11) e anaplásico (1/11). Destes, oito (8/11) apresentaram marcação positiva para tricrômio de Masson e um para vermelho congo nas técnicas histoquímicas. No painel imuno-histoquímico, todos os casos apresentaram imunomarcação positiva para vimentina, mas imunomarcação negativa para fator VIII e p53. A imunomarcação para S100 (6/11), GFAP (5/11) e pancitoqueratina (3/11) foi de intensidade variável. Na graduação histológica, dez meningiomas eram grau I e um grau III. O índice médio de proliferação...

Neoplasias of the central nervous system (CNS) of small animals are frequently diagnosed; however, data relative to prevalence are scarce. The aim of this study was to evaluate retrospectively the occurrence of primary CNS tumors in dogs in a Veterinary Teaching Hospital and describe clinical, histopathological and immunohistochemical aspects of the most common tumors. Fourteen cases of CNS primary neoplasia (prevalence of 0.27%) were identified from 1998 to 2013; from these 11 were diagnosed as meningiomas. The mean age of dogs with meningiomas was 10 years; male (7/11) and the Boxer breed (3/11) were most commonly represented. Seven meningiomas were located in the spinal cord and four intracranially; the main clinical signs were impaired locomotion and seizures, respectively. Pulmonary metastasis was found in two cases. Myelography was performed in six dogs with spinal meningiomas. In these cases it was effective in demonstrating the deviation or interruption of the contrast column. Exploratory surgery to confirm diagnosis or remove the tumor was performed in five animals and the survival rate varies 85 to 960 days. Meningiomas were histologically classified as transitional (4/11), meningothelial (2/11), papillary (2/11), angiomatous (1/11), microcystic (1/11) and anaplastic (1/11). Evaluation of histological grade was performed; 10 meningiomas were classified as grade I and one as grade III. The neoplastic cells of eight tumors (8/11) were positive for Masson's trichrome and one with the Congo red histochemical techniques. The immunohistochemical assays revealed in all tumors a positive immunoreactivity for vimentin but negative staining for factor VIII and p53. Immunolabelling for S100 (6/11), GFAP (5/11) and pancytokeratin (3/11) showed a variable staining intensity. The mean cell proliferation index was 3.2 mitotic figures and 3.4% for Ki-67 immunostaining. The results confirmed that meningiomas are the most frequent primary CNS neoplasia...

Headache and analgesic in postoperative craniotomy

Objective: To identify the most prevalent type of pain in postoperative craniotomy patients and evaluate the use of analgesic in this group of patients. Method: The case studies consisted of 55 patients undergoing craniotomy. Patients were evaluated from the first to the seventh postoperative day. Data were stored and analyzed using the Statistical Package for Social Sciences (SPSS) version 17.0. The variables analyzed were gender, demographic data, assessing the presence of pain, location of pain, severity of pain, use of analgesic, professionals who recorded the pain on the patients’ charts, and presence of insomnia. Results: The results show that headache, the main pain reported by the patients, is present from the first to the seventh postoperative day. It was observed that in some cases there was no prescription of analgesics and even in the group of patients who took analgesics, these drugs were not effective for the relief of headache. The final logistic regression model revealed that insomnia ( OR = 10.6, p = < 0.001 ), the pain recorded in the patients’ charts (OR = 2.38 , p = 0.016) and use of analgesic (OR = 2.03 , p = 0.014) adequately explained the outcome. Conclusion: Headache was the chief complaint of pain after postoperative craniotomy, and its intensity varied from mild to severe. The analgesic used was not effective for the control of postoperative headache. Proper handling of post-craniotomy headache by the multidisciplinary team can contribute to early neurological recovery of patients undergoing craniotomy, and should make the post-surgical care, with a view to early recovery and the humanization of care.