Calcinosis cutis congénita tumoral asociada con encefalopatía epiléptica infantil temprana con variante patogénica en el gen FGF12 / Tumorous congenital calcinosis cutis associated with early childhood epileptic encephalopathy with a pathogenic FGF12 gene variant

Resumen Introducción: La calcinosis cutis es el depósito de sales insolubles de calcio en la piel y se clasifica, de acuerdo con su patogénesis, en distrófica, metastásica, idiopática, iatrogénica y calcifilaxis. La calcinosis idiopática se presenta en pacientes sanos y es asintomática; incluye la calcinosis escrotal, la calcinosis nodular de Winer o nódulos calcificados subepidérmicos y la calcinosis tumoral familiar. Esta última es una condición rara que se caracteriza por el depósito de calcio periarticular en pacientes normocalcémicos sin conexión al hueso. Caso clínico: Paciente de sexo masculino de 5 meses de edad, quien al séptimo día de vida fue hospitalizado por ictericia multifactorial, sepsis neonatal tardía y apnea con crisis epilépticas. La evolución fue tórpida, con ingresos hospitalarios por crisis epilépticas de difícil manejo, respuesta parcial a la difenilhidantoína y descontrol electrolítico. Mediante la secuenciación del exoma dirigido se detectó una variante patogénica de sentido equivocado en FGF12 que confirmó el diagnóstico de encefalopatía epiléptica temprana número 47. Además, el paciente presentó dermatosis congénita diseminada a las extremidades inferiores con afección en muslos, asintomática, bilateral y simétrica, constituida por hipopigmentación y fóveas duras a la palpación profunda. La biopsia mostró calcificación distrófica. Conclusiones: Se presenta el caso de un lactante con calcinosis cutis congénita profunda asociada con una variante patogénica en el gen FGF12 y con encefalopatía epiléptica, situación clínica que, a la fecha, no había sido reportada en la literatura.

Abstract Background: Calcinosis cutis is the deposit of insoluble calcium salts in the skin. It is classified according to its pathogenesis in dystrophic, metastatic, idiopathic, iatrogenic, and calciphylaxis. Idiopathic calcinosis is asymptomatic, occurs in healthy patients, and includes scrotal calcinosis, Winer's nodular calcinosis or subepidermal calcified nodules, and familial tumor calcinosis. The latter is a rare condition characterized by periarticular calcium deposition in normocalcemic patients with no bone connection. Case report: The case of a 5-month-old male patient, who on the seventh day of life was hospitalized for multifactorial jaundice, late neonatal sepsis, and apnea with epileptic seizures is described. His evolution was torpid, with hospital admissions due to epileptic seizures that were difficult to manage with partial response to the use of diphenylhydantoin and electrolyte alterations. By means of exome sequencing directed, a pathogenic variant of wrong direction in FGF12 was detected and the diagnosis of early epileptic encephalopathy number 47 was confirmed. Also, the patient showed disseminated congenital dermatosis to lower extremities affecting thighs, asymptomatic, bilateral and symmetrical, constituted by hypopigmentation and fovea hard to deep palpation. The biopsy showed dystrophic calcification Conclusions: The case of an infant with deep congenital cutis calcinosis associated with a pathogenic variant in the FGF12 gene with epileptic encephalopathy is described. To date, this clinical situation has not been previously reported in the literature.

Mucormicosis rino-órbito-cerebral crónica: una patología poco frecuente / Chronic rhino-orbital-cerebral mucormycosis: an uncommon pathology

Resumen La mucormicosis rino-órbito-cerebral (ROC) crónica es una patología poco frecuente, con un número reducido de casos publicados en la literatura, cuyas manifestaciones son muy diversas e inespecíficas. El tratamiento se basa en la experiencia de casos y series de casos. Las herramientas terapéuticas incluyen el uso de antifúngicos endovenosos y orales por tiempo prolongado, asociado o no a debridamiento quirúrgico amplio, pudiendo requerir incluso exenteración orbitaria. Presentamos a continuación un caso de mucormicosis ROC crónica, junto con las dificultades para su diagnóstico y manejo, en el que destaca el enfrentamiento multidisciplinario. Dada la poca frecuencia de esta enfermedad, nos parece relevante difundirlo.

Abstract Chronic rhino-orbital-cerebral mucormycosis is a rare condition with a small number of cases that have been published, whose manifestations are very diverse and nonspecific. The treatment is based on case series experiences. Therapeutic options include the use of long-term intravenous and oral antifungals, associated or not with extensive surgical debridement, and may even require orbital exenteration. We present below a case of chronic rhino-orbital-cerebral mucormycosis with the challenge of diagnosis and management in which multidisciplinary work is fundamental. Since it is an uncommon pathology, it seems relevant to share the information.

Encefalopatías epilépticas determinadas genéticamente / Genetically determined epileptic encephalopathies

Las encefalopatías epilépticas es un grupo de síndromes epilépticos caracterizados por el deterioro cognitivo más allá de lo esperado debido a la actividad epiléptica. Se caracterizan por presentar resistencia farmacológica grave, electroencefalogramas profundamente anormales, inicio en la niñez temprana, deterioro neurocognitivo, fenotipo variable y resonancia magnética de cerebro usualmente normal. Frecuentemente estos síndromes están genéticamente determinados. Su diagnóstico correcto y oportuno puede contribuir y guiar el consejo médico y terapia adecuada, influyendo así en el pronóstico a corto, mediano y largo plazo. En este artículo se revisan los hallazgos electroencefalográficos, genéticos y opciones terapéuticas más recomendadas, facilitando así la conducta clínica. Las encefalopatías epilépticas incluidas en este artículos abarcan los síndromes de Ohtahara, encefalopatia mioclónica temprana, epilepsia focal migratoria de la infancia, West, Dravet, estado mioclónico en encefalopatías no progresivas, Doose, Lennox-Gastaut, Landau-Kleffner y epilepsia con espiga-onda continuas durante el sueño de onda lenta.

Epileptic encephalopathies is a group of epileptic syndromes characterized by progressive cognitive impairment beyond the expected for the epilepsy activity. They are characterized by severe pharmaco-resistant epilepsy, severely abnormal electroencephalograms, early-age onset, neurocognitve impairment, variable phenotype and usually normal brain MRI. These syndromes are usually genetically determined. A correct and timely diagnosis could help and guide the medical counselling and the correct therapeutic approach improving the short, medium and long term outcomes. In this article we review the electroencephalographic and genetic findings along with the most recommended therapeutic options facilitating the clinical management. We include the following epileptic encephalopathy syndromes: Ohtahara, early myoclonic encephalopathy, epilepsy of infancy with migrating focal seizures, West, Dravet, non-progressive myoclonic status, Doose, Lennox-Gastaut, Landau-Kleffner and continuous spike-wave during sleep epilepsy.

Dyke-Davidoff-Masson syndrome and chiari type ii malformation / Síndrome de Dyke-Davidoff-Masson e malformação de chiari tipo II

Dyke-Davidoff-Masson Syndrome is a syndrome associated with refractory epilepsy. The Chiari II malformation is a complex congenital malformation of the brain. The authors report a case of a 15 years-old adolescent presenting Dyke-Davidoff-Masson syndrome and Chiari type II malformation association. This case demonstrates an unusual association in neuroimaging tests that indicates the need to evaluate associated diseases, such as myelomeningocele, corpus callosum dysgenesis and syringohydromyelia.

A Síndrome de Dyke-Davidoff-Masson é uma síndrome associada à epilepsia refratária. A malformação de Chiari II é uma malformação congênita complexa do cérebro. Os autores relatam um caso de uma adolescente de 15 anos apresentando a síndrome de Dyke-Davidoff-Masson associada à malformação de Chiari tipo II. Este caso demonstra uma associação incomum nos exames de neuroimagem que indica a necessidade de avaliar doenças associadas, como mielomeningocele, disgenesia do corpo caloso e a siringohidromielia.

Aspergilosis cerebral como causa de lesión cerebral focal asociada SIDA: a propósito de un caso y revisión de la literatura / Focal brain lesion due to cerebral aspergillosis in a patient with AIDS: case report and literature review

Resumen La aspergilosis cerebral es una patología infrecuente, pero de elevada mortalidad en pacientes con SIDA. Es importante considerarla entre los diagnósticos diferenciales ante una lesión expansiva cerebral. Se requiere un alto grado de sospecha para poder realizar un diagnóstico precoz. Se presenta el caso de un paciente con infección por VIH con un cuadro neurológico rápidamente progresivo por Aspergillus sección flavi. Se realiza una revisión de 40 casos publicados de aspergilosis cerebral en pacientes con SIDA.

Cerebral aspergillosis is a rare disease with high mortality rates in AIDS patients. It is important to take this into account in the differential diagnosis of a brain expansive lesion. A high level of suspicion is required to make an early diagnosis. We present a case of an HIV-infected patient with progresive neurological disease caused by Aspergillus flavi. We review 40 previously published cases of central nervous system aspergillosis in patients with AIDS.

Hidatidosis cerebral: comunicación de seis casos pediátricos / Cerebral hydatid disease: report of six pediatric cases

Hydatid disease is a parasitic infection whose etiologic agent is Echinococcus granulosus. Human is an accidental intermediate host and the most common site is the liver. The brain involvement is unusual and up to 75% of cases are described in the pediatric population. We present six children with cerebral hydatid disease admitted to the Pediatric Hospital J.P. Garrahan. All had neurological involvement on admission. The images showed single cystic lesion in the brain. They did not present involvement in other organs. Serology was negative in all cases. Medical and surgical treatment in all cases. The clinical outcome was favorable without sequelae in five of them and one had a residual paresis right faciobrachiocrural. This infection should be considered in the differential diagnosis of cystic tumor lesions of the central nervous system.

La hidatidosis es una infección parasitaria causada por Echinococcus granulosus. El ser humano es un hospedero intermediario accidental. La localización más frecuente es la hepática. El compromiso cerebral es inusual, se describe que hasta 75% ocurre en población pediátrica. Se presenta una serie de seis niños con hidatidosis cerebral internados en el Hospital de Pediatría J. P. Garrahan. Todos presentaron compromiso neurológico al ingreso. Las imágenes mostraron lesiones quísticas únicas en el cerebro. No tuvieron compromiso de otros órganos. La serología fue negativa en todos los casos. El tratamiento fue médico-quirúrgico. La evolución clínica fue favorable sin secuelas en cinco de ellos y uno presentó una hemiparesia faciobraquiocrural derecha como secuela. Esta infección debe considerarse entre los diagnósticos diferenciales de lesiones tumorales quísticas del sistema nervioso central.

Clinical electroencephalogram (EEG) evaluation is improved by the amplitude asymmetry index / Avaliação da assimetria de amplitudes amplia o uso clínico do eletroencefalograma (EEG)

ABSTRACT Cerebral hemispheres, although similar, are neither completely symmetrical in structure nor equivalent in function. EEG asymmetry studies have been directed more to frequency than to amplitude analysis. Objective Better definition of normal amplitude asymmetry values on the classical EEG frequency bands. Results EEG amplitude asymmetry index (AAI) is physiologically low in normal adults, differences usually lesser than 7%. Conclusion Persistent or intermittent amplitude asymmetry regional differences higher than 7% may be suggestive of pathology after adequate correlation with clinical data and EEG classical visual analysis.

RESUMO Os hemisférios cerebrais não são simétricos em morfologia ou funções. Tradicionalmente, os estudos de assimetria do EEG focaram mais na análise de frequências do que na de amplitudes. Objetivo Contribuir para melhor definição dos valores normais de assimetria de amplitudes nas faixas de frequência clássicas do EEG. Resultados O índice de assimetria de amplitudes é fisiologicamente baixo em adultos, usualmente menor do que 7%. Conclusão Se adequadamente correlacionadas aos dados clínicos e à análise visual do EEG, assimetrias persistentes ou intermitentes de amplitude acima de 7% podem ser sugestivas de patologia.

Aspiración estereotáctica de una larva de Spirometra spp. / Stereotactic aspiration of Spirometra mansonides larvae

Brain sparganosis is a non-common parasite infection by Diphyllobothrium or Spirometra mansonoides larvae. This last one is responsible for most of the infestations in humans. We report a 19 years male patient bearer of a brain sparganosis. The patient presented with headache and left hemiparesis. CT diagnosis of right thalamic lesions was made and aspiration biopsy was performed using stereotactic system, obtaining a whole and death larvae. Histopathology confirms a CNS parasitism and it was treated initially with albendazol. ELISA test confirmed Spirometra spp. infestation. The patient developed asymptomatic with total remission of the lesions. It constitutes the second report in Cuba of brain sparganosis.

Se presenta el caso clínico de un varón con 19 años de edad y el diagnóstico de una esparganosis cerebral. Consultó por cefalea y una hemiparesia izquierda. En una tomografía computarizada cerebral con contraste se observaron lesiones talámicas derechas. Se realizó una biopsia cerebral guiada por estereotaxia con aspiración completa de un verme. En el estudio histopatológico se planteó un probable parasitismo de SNC y fue tratado inicialmente con albendazol. Se confirmó la infección por Spirometra spp. por test de ELISA. Evolucionó con regresión de síntomas y remisión imagenológica de las lesiones. Este caso constituye el segundo reporte en Cuba de una infestación cerebral por este parásito y aspiración estereotáctica de la larva de Spirometra spp.

Encefalopatía respondedora a corticoides asociada a tiroiditis autoinmune (SREAT). Una causa no habitual de compromiso de conciencia en pediatría, reporte de 2 casos / Steroid responsive encephalopathy associated with autoimmune thyroiditis (SREAT): an unusual cause of encephalopathy in children, report of two cases

Introduction: steroid responsive encephalopathy associated with autoimmune thyroiditis (SREAT) is a rare condition in children. The pathogenesis and etiology of SREAT has not yet been clearly identified. Clinical features include acute or subacute encephalopathy with neuropsychiatric symptoms, associated with abnormally elevated thyroid antibodies (TA) and symptoms improvement with corticosteroid treatment. Methods and Patients: we present 2 clinical cases; the first a 6 years 8 months male with cephalic myoclonic seizures and behavioral changes, the second a 12 years 10 months female with 4 hospitalizations for encephatlopathy, dystonia and psychomotor agitation. In both patients thyroid function tests and TA were compatible with Hashimoto’s thyroiditis, this associated with neuropsychiatric symptoms did raise the diagnosis of SREAT. Glucocorticoid therapy was started, the first case showed remission of seizures and behavioral improvement, however the second patient had insufficient response, so second line therapy with intravenous immunoglobulin was introduced with good response. This therapy was supplemented by additional long-term glucocorticoids use but when suspended both patients presented relapsing symptoms. Conclusions: Although SREAT is rarely suspected at presentation, it is necessary to consider this diagnosis in patients with encephalopathy, neuropsychiatric symptoms and elevated TA. Further studies are required to elucidate the pathophysiology of this disease and follow-up work to assess comorbidities and long-term complications in pediatric patients.

La encefalopatía de la prematuridad, una entidad nosológica en expansión / Encephalopathy of prematurity, a developing nosological entity

Con frecuencia se emplean los términos leucomalacia periventricular, daño a la sustancia blanca y encefalopatía de la prematuridad como sinónimos; sin embargo, no son exactamente lo mismo. Se realiza esta revisión con el objetivo de fundamentar la consistencia del término encefalopatía de la prematuridad, a partir de una actualización sobre su fisiopatología, diagnóstico y pronóstico. La leucomalacia periventricular tiene 2 componentes principales, uno macroscópico quístico y otro microscópico, que puede ser focal o difuso. El daño a la sustancia blanca es el hallazgo neuropatológico más común de parálisis cerebral en los recién nacidos pretérminos, que identifica cualquier anormalidad en la sustancia blanca. La encefalopatía de la prematuridad, en cambio, es un término mucho más abarcador, porque en su origen parecen tener importancia los trastornos destructivos primarios y los relacionados con el desarrollo cerebral. Su fisiopatología se ha tratado de explicar mediante 2 modelos teóricos: el infeccioso-inflamatorio y el hemodinámico. Para su diagnóstico resultan necesarias la evaluación neuroimaginológica y la del neurodesarrollo, en la que a menudo se diagnostica la presencia de parálisis cerebral o trastornos del desarrollo intelectual, aisladamente o en asociación. Es muy probable que la encefalopatía de la prematuridad tienda a afianzarse cada día más como una entidad nosológica independiente, dadas sus implicaciones médicas y sociales.

Some terms such as periventricular leukomalacia (PVL), white matter damage (WMD) and encephalopathy of prematurity are used as synonyms; however, they are not exactly the same. This review was aimed at substantiating the consistency of the term encephalopathy of prematurity, on the basis of its physiopathology, diagnosis and prognosis. Periventricular leukomalacia has two main components, one macroscopic cystic and the other microscopic one which can be focal or diffuse. The white matter damage is the most common neuropathological feature of cerebral palsy in preterm infants, which identifies any anomalies in the white matter. In turn, encephalopathy of prematurity is a much more comprehensive term because it seems that both primary destructive disorders and those related to brain development are influential in its origin. Its physiopathology has been explained by two theoretical models: the infective-inflammatory one and the hemodynamic one. Neuroimaginological and neurodevelopmental evaluations are necessary for the diagnosis, where the presence of cerebral palsy and of intellectual development disorders, either isolated or combined, is detected. It is much likely that encephalopathy of prematurity tends to increasingly consolidate as an independent nosological entity, taking into account its medical and social implications.

[Value of magnetic resonance spectroscopy in diagnosis of patients with focal brain lesion]

Objective: To describe the spectrum of Magnetic Resonance spectroscopy in focal brain lesions and determine its diagnostic accuracy in comparison to conventional MRI using histopathology as gold standard in differentiating various types of focal brain space occupying lesions. New line.

Patients and methods: 52 patients [21 males and 31 females] with single focal brain space occupying lesion diagnosed by post contrast CT or MRI are included in this study. Complete clinical evaluation, MRI, and MRS examination were performed for all these patients before surgical biopsy and/or resection. Correlation of all these findings was done with histopathology obtained in all these patients

Results: The most important markers in differentiating and grading various types of single focal brain space occupying lesion were choline level and choline/N-acetyl aspartate ratio. There is statistically significant difference between mean of Cho/NAA in high grade glioma as it was 23.13 and low grade glioma where mean of choline/NAA was 6.21 [P value = 0.02]. Lipid and lactate peaks were more frequent in high grade tumors and lipid peak together with Choline/N-acetyl aspartate ratio could differentiate between metastasis and metastasis with delayed radiation necrosis. Alanine peak appear to be pathognomonic for meningioma. Perilesional MRS metabolites could detect actual histopathological [subclinical] extension and so differentiate between high grade and low grade tumors and between high grade tumors and metastasis

Conclusion: MRI+MRS is more sensitive than MRI alone in diagnosis, differential diagnosis, and grading of focal brain space occupying lesion. MR spectroscopy could differentiate various types of focal brain space occupying lesion with sensitivity of 92.3% while sensitivity of MRI alone was 76.9%

RM de encéfalo sin anestesia en pacientes recién nacidos: experiencia inicial en Clínica Alemana de Santiago / Cerebral mri performed without anesthesia in newborn (neonatal) patients. initial experience in Clinica Alemana of Santiago

Given that hypothermia cases have occurred in newborns after MRI with anesthesia and reproducing what was observed in centers visited overseas, we implemented a supervised protocol to evaluate the results of cerebral MRI performed without anesthesia or sedation in patients hospitalized in our institution. Objective: To display results of the protocol for performing cerebral MRI without anesthesia in our newborn patients. Materials and methods: A retrospective study, we evaluated cerebral MRI performed on newborns from september 2012 - july 2013. Protocol for performing MRI without anesthesia was applied in institutionalized patients. Variables are compared with MRI performed using anesthesia, during this period. Results: 22 cerebral MRI were performed, 14 without anesthesia. The duration of the study without anesthesia was 48 minutes on average, obtaining adequate images in 13 studies, reprogramming the examination with anesthesia in one case. Conclusions: The protocol implemented allows the acquisition of diagnostic-quality cerebral MRI, obviating the anesthesia.

Dado casos de hipotermia en recién nacidos (RN) posterior a resonancia magnética (RM) con anestesia y reproduciendo lo observado en centros extranjeros visitados, implementamos protocolo supervisado para evaluar los resultados de RM encefálicas realizadas sin anestesia o sedación en pacientes hospitalizados en nuestra institución. Objetivo: Mostrar resultados del protocolo para realización de RM encefálica sin anestesia en nuestros pacientes RN. Materiales y métodos: Estudio retrospectivo, evaluamos RM encefálicas realizadas en RN entre septiembre 2012 - julio 2013. Se aplicó protocolo para realización de RM sin anestesia en pacientes institucionalizados. Se comparan variables con RM realizadas con anestesia en ese periodo. Resultados: Se realizaron 22 RM encefálicas, 14 sin anestesia. La duración del estudio sin anestesia fue de 48 minutos promedio, obteniendo imágenes adecuadas en 13 estudios, reprogramando el examen con anestesia en un caso. Conclusiones: el protocolo implementado permite obtener RM encefálicas de calidad diagnóstica, obviando la anestesia.

Cephaloceles a Dakar : etude de 30 cas au CHUN Fann

Les cephaloceles se definissent comme des hernies du contenu de la boite cranienne a travers un defect du crane. Le but de l'etude est de rapporter notre experience sur la prise en charge des cephaloceles et d'evaluer l'evolution du traitement chirurgical. Methode : trente enfants ont ete admis au service de neurochirurgie de fann sur une periode de 5 ans et demi pour prise en charge d'une cephalocele. Il s'agit d'une etude retrospective. Resultats : L'age moyen des patients a l'admission etait de 4 mois avec un sex-ratio de 0;9. Plus de 57 de meres avaient un age superieur a 30 ans. Le mariage consanguin a ete retrouve dans une proportion de 36;7. La supplementation durant la grossesse en fer acide folique a ete effective chez 81;48 des femmes. La localisation du defect osseux au niveau occipital etait retrouvee dans 70 contre 16;7 en fronto-ethmoidale; 10 en sincipitale et 3;3 en frontale unilaterale. Tous les patients ont beneficie d'une tomodensitometrie cerebrale et ont ete operes. Le recours a la derivation ventriculo-peritoneale a ete necessaire dans six cas (20) pour une hydrocephalie. L'evolution a ete favorable chez 29 patients (96;7). Un deces a ete constate apres la cure de la cephalocele. Le suivi post-operatoire a varie de 3 mois a 2 ans. Conclusion : Ce travail souligne la survenue de cephaloceles chez le 2eme enfant de la fratrie; une predominance de la localisation occipitale; ainsi que la rarete du diagnostic echographique antenatal. La prevention semble etre le meilleur traitement

Monitoring of parasitic cysts in the brains of a flock of sheep in Egypt / Monitoramento de cistos parasitários no cérebro de um rebanho de ovinos no Egito

Cerebral parasitic cysts constitute a major problem for livestock. Among these, coenurosis and toxoplasmosis are predominant. Here, a total number of 60 sheep obtained from a private farm in Suez province, Egypt, were examined postmortem to detect visible parasitic cysts, and microscopically to detect small-sized entities. Necropsy revealed bladder-like cysts measuring 0.5-6.5 cm in diameter that were filled with a translucent fluid containing a large number of protoscolices. Accordingly, the cysts were identified as the metacestode Coenurus cerebralis. Among the sheep examined, 11 animals (7 males and 4 females) (18.3%) were infected. Most of the cysts were located in the cerebral hemispheres, with numbers ranging from one to three per infected animal. The effect of the presence of cysts in the brain tissue was evaluated. Histopathologically, pseudocysts of the apicomplexan Toxoplasma gondii were found in two animals with no detectable inflammatory cell reactions. In conclusion, coenurosis and toxoplasmosis are serious parasitic problems that play a significant role in sheep management in Egypt, as a result of close contact between livestock and dogs and cats, which play a critical role in the life cycle of these parasites.

Cistos cerebrais parasitários constituem um grande problema para o gado. Entre estes, coenurosis e toxoplasmose são predominantes. Aqui, um número total de 60 ovelhas obtidas em uma fazenda particular na província Suez, Egito, foram examinadas post-mortem para a detecção de cistos parasitários visíveis e microscopicamente para detectar cistos de pequenas dimensões. A necropsia revelou cistos medindo entre 0,5-6,5 cm de diâmetro, preenchidos com um fluido transparente, contendo um grande número de protoscolices. Por conseguinte, os cistos foram identificados como o metacestóide Coenurus cerebralis. Entre as ovelhas examinadas, 11 animais (7 machos e 4 fêmeas) (18,3%) estavam infectados. A maior parte dos cistos estavam localizados nos hemisférios cerebrais, com números variando de um a três em ovinos infectados. O efeito da presença de cistos no tecido do cérebro foi avaliado. Histopatologicamente, pseudocistos de Toxoplasma gondii foram encontrados em dois animais sem reações inflamatórias detectáveis. Em conclusão, coenurosis e toxoplasmose são graves problemas parasitários que desempenham um papel significativo no manejo de ovelhas no Egito, como resultado do contacto íntimo dos animais com os cães e gatos, que desempenham um papel crítico no ciclo de vida desses parasitas.

Mansonic neuroschistosomiasis / Neuroesquistossome mansonica

Mansonic neuroschistosomiasis (MN) is not only the most common but also the most serious ectopic presentation of the infection by Schistosoma mansoni. Both, brain and spinal cord can be independently affected by the infection, but the later is more frequently affected. Brain MN by itself is due to the presence of eggs and/or adult worms in situ and can be symptomatic or asymptomatic. Unlike the brain MN, spinal cord mansonic neuroschistosomiasis is more frequently symptomatic. In both forms the intensity, the seriousness and also the clinical characteristics of signs and symptoms depend on the amount of eggs in the compromised region and on the intensity of the inflammatory reaction surrounding the eggs. Cerebrospinal fluid examination and magnetic resonance imaging are important diagnostic tools. Both corticosteroids and drugs against S. mansoni are used in the treatment. The outcome may largely depend upon the prompt use of these drugs.

A neuroesquistossome mansônica (NM) é não apenas a mais comum, mas também a mais grave apresentação da infecção pelo Schistosoma mansoni. Tanto o encéfalo quanto a medula podem ser independentemente afetadas pela doença, embora a última o seja de forma mais frequente. A NM encefálica é secundária à presença dos ovos e/ou da forma adulta do verme in situ, e pode ser sintomática ou não. Ao contrário da forma encefálica, a NM medular é mais frequentemente sintomática. Em ambas as formas a gravidade dos sintomas dependerá na quantidade de ovos na região comprometida e na intensidade da reação inflamatória ao seu redor. Os exames do líquido cefalorraquiano e de imagem por ressonância magnética são importantes ferramentas diagnósticas. Corticosteróides e drogas parasiticidas são usadas no tratamento desta doença, e seu prognóstico dependerá diretamente do rápido uso destas drogas.

Enfermedad de Rosai-Dorfman extranodal: presentación de un caso con afectación intracraneal aislada / Rosai-Dorfman Disease extranodal: report of a case with isolated intracranial involvement

La enfermedad de Rosai-Dorfman (ERD) es una enfermedad idiopática benigna caracterizada por una proliferación anormal de histiocitos. Desde que fue descripta por Rosai y Dorfman en 1969 son pocos los casos publicados en la literatura, aproximadamente 750 en el mundo. 1- 4La presentación clínica más frecuente son linfadenopatías cervicales bilaterales indoloras. La afectación extranodal ocurre en el 43% de los casos y el compromiso del sistema nervioso central en un 4%.1,3-8 Amos y cols. publican la existencia de 111 casos hasta el año 2008 con afectación del sistema nervioso central (SNC).9 Hasta el año 2011 se han publicado un total de 136 casos.En este artículo presentamos el caso de una paciente con ERD intracraneal sin afectación ganglionar, afectación excepcionalmente rara (únicamente se han publicado 24 casos).