Extending the Indications of 5-Aminolevulinic Acid for Fluorescence-Guided Surgery for Different Central Nervous System Tumors: A Series of 255 Cases in Latin America

Introduction Fluorescence guidance with 5-aminolevulinic acid (5-ALA) is a safe and reliable tool in total gross resection of intracranial tumors, especially malignant gliomas and cases of metastasis. In the present retrospective study, we have analyzed 5-ALA-induced fluorescence findings in different central nervous system (CNS) lesions to expand the indications of its use in differential diagnoses. Objectives To describe the indications and results of 5-ALA fluorescence in a series of 255 cases. Methods In 255 consecutive cases, we recorded age, gender, intraoperative 5-ALA fluorescence tumor response, and 5-ALA postresection status, as well the complications related to the method. Postresection was classified as '5-ALA free' or '5-ALA residual'. The diagnosis of histopathological tumor was established according to the current classification of the World Health Organization (WHO). Results There were 195 (76.4%) 5-ALA positive cases, 124 (63.5%) of whom underwent the '5-ALA free' resection. The findings in the positive cases were: 135 gliomas of all grades; 19 meningiomas; 4 hemangioblastomas; 1 solitary fibrous tumor; 27 metastases; 2 diffuse large B cell lymphomas; 2 cases of radionecrosis; 1 inflammatory disease; 2 cases of gliosis; 1 cysticercosis; and 1 immunoglobulin G4-related disease.

Neonatal acute liver failure with pulmonary yellow hyaline membrane and kernicterus

Background Neonatal acute liver failure (NALF) is a rare and life-threatening condition. It causes bilirubin to accumulate to a dangerous level in the body, causing permanent damage to vital organs such as the brain and lungs. In many cases, the etiology of NALF remains unknown. Case presentation We described a case of an 8-day-old baby girl who presented with poor oral intake, lethargy, and jaundice. Her clinical condition rapidly deteriorated with progression to multi-organ failure, and despite intensive resuscitation efforts, she expired. At autopsy, the most significant findings were liver necrosis, yellow hyaline membrane deposition in the lungs, and bilirubin deposition in the brain (kernicterus). Conclusions NALF is a rare and potentially fatal condition necessitating prompt recognition and disease-specific treatment approaches. Toxic accumulation of bilirubin in the lungs can lead to hypoxia and precipitate further ischemic injury to the liver.

Doença de Creutzfeldt-Jakob: relato de caso / Creutzfeldt-Jakob disease: case report

As doenças priônicas fazem parte do grupo das síndromes de demência rapidamente progressiva com neurodegeneração. Em humanos, a doença de Creutzfeldt-Jakob é a mais prevalente. Atualmente, seu diagnóstico pode ser baseado em uma combinação do quadro clínico, ressonância magnética e eletroencefalograma com alterações típicas, juntamente da detecção de proteína 14- 3-3 no líquido cefalorraquidiano. Este relato descreve o caso de uma paciente de 74 anos, natural de Ubá (MG), admitida em um hospital da mesma cidade com quadro de demência de rápida progressão, com declínio cognitivo, ataxia cerebelar e mioclonias. No contexto clínico, aventou-se a possibilidade de doença de Creutzfeldt-Jakob e, então, foi iniciada investigação para tal, com base nos critérios diagnósticos. Também foram realizados exames para descartar a possibilidade de doenças com sintomas semelhantes. O caso foi diagnosticado como forma esporádica de doença de Creutzfeldt-Jakob. (AU)

Prion diseases are part of the rapidly progressive dementia syndromes with neurodegeneration. In humans, Creutzfeldt-Jakob disease is the most prevalent. Currently, its diagnosis may be based on a combination of clinical picture, magnetic resonance imaging, and electroencephalogram with typical changes, along with the detection of 14-3-3 protein in cerebrospinal fluid. This report describes the case of a 74-year-old woman from the city of Ubá, in the state of Minas Gerais, who was admitted to a hospital in the same city with a rapidly progressive dementia, cognitive decline, cerebellar ataxia and myoclonus. In the clinical context, the possibility of Creutzfeldt-Jakob disease was raised, and then investigation was started for this disease, based on the its diagnostic criteria. Tests have also been conducted to rule out the possibility of diseases with similar symptoms. The case was diagnosed as a sporadic form of Creutzfeldt-Jakob disease. (AU)

Relationship between Electrical Conductivity and Related Biochemical Indicators of Rat Cerebrum Tissues and Postmortem Interval / 法医学杂志

Objective To explore the relationship between the electrical conductivity （EC） and biochemical indicators of rat cerebrum tissues and postmortem intervals （PMIs） and discuss the mechanism of applying EC to infer PMI. Methods Forty healthy Sprague-Dawley rats were sacrificed and stored in an environment of about 25 ℃. The whole cerebrum tissues of rats were removed respectively at different PMIs of 0, 1, 2, 3, 4, 5, 6, and 7 d, and then made into homogenized impregnation solution. The EC and related biochemical indicators （potassium, sodium, chloride, calcium, inorganic phosphorus, magnesium, uric acid, urea nitrogen and creatinine） in cerebrum tissue impregnation solution were determined, and the relationships among EC in impregnation solution, related biochemical indicators and PMI were analyzed. Results The EC in cerebrum tissues increased gradually with the extension of PMI, and the content of uric acid, urea nitrogen and inorganic phosphorus in its impregnation solution also increased gradually with the extension of PMI. The correlation of EC, uric acid, urea nitrogen, and inorganic phosphorus with PMI was relatively good （R2 was 0.95-0.99）, and there was a linear correlation between the content change of uric acid, urea nitrogen, inorganic phosphorus and EC （R2 was 0.97-0.99）. The changes of the other 6 kinds of biochemical indicators with the extension of PMI within 7 d after the rats' death were non-significant （P>0.05）. Conclusion The correlation between EC in cerebrum tissues, uric acid, urea nitrogen, inorganic phosphorus and PMI were relatively good, and combining various indicators can also improve the accuracy of PMI estimation.

Volumetría hipocampal: análisis comparativo de los métodos de evaluación en enfermedad de Alzheimer / Hippocampal Volumetry: Comparative Analysis of Evaluation Methods in Alzheimer's Disease

Objetivo La atrofia hipocampal es uno de los biomarcadores radiológicos más sensibles de la enfermedad de Alzheimer (EA) y existen diferentes métodos para evaluarla: análisis subjetivo visual (ASV), análisis objetivo manual (AOM) y análisis objetivo automático (AOA). Nos proponemos comparar esos métodos, y evaluar si el AOA presenta una confiabilidad cercana al AOM (método de referencia) y superior al ASV. Materiales y Métodos Se seleccionaron retrospectivamente imágenes de resonancia magnética (RM) fast spoiled gradient-echo (FSPGR) de 28 sujetos (14 con deterioro cognitivo leve, 7 con EAy 7 controles). El ASV fue realizado por 10 radiólogos, clasificando la atrofia hipocampal en: nula, leve, moderada o severa. El AOM se basó en la segmentación manual de los hipocampos por dos operadores. El AOA fue realizada por medio del software FreeSurfer 5.3. Se calcularon coeficientes de correlación rho de Spearman para las variables discretas y coeficientes de correlación intraclase para las variables continuas. Resultados Los coeficientes de correlación entre los dos operadores que realizaron el AOM fueron de 0,88 (p < 0,0001) para los hipocampos izquierdos y de 0,86 (p < 0,0001) para los hipocampos derechos. El coeficiente de correlación entre todos los ASV (promediados) y AOM fue de-0,81 (IC 95%-0,96- -0,66). Los coeficientes de correlación entre el AOA y el AOM fue de 0,54 (p < 0,0001) para los hipocampos izquierdos y de 0,61 (p < 0,0001) para los hipocampos derechos. Conclusión Si bien el AOA tiene moderada correlación con el método de referencia, no es superior al ASV promedio y se deberían tomar recaudos antes de ser implementado en la práctica asistencial.

Objective Hippocampal atrophy is one of the most sensible radiological biomarkers of Alzheimer's disease. There are different methods to evaluate atrophy: visual subjective analysis (VSA), manual objective analysis (MOA) and automatic objective analysis (AOA). We will compare these methods and evaluate if AOA has a confidence similar to MOA (gold standard), and better than VSA. Materials and Methods We retrospectively selected 3D FSPGR MRI from 28 subjects of whom 14 had mild cognitive impairment, 7 Alzheimer's disease and 7 controls. VSA was performed by 10 radiologists who classified hippocampal atrophy in none, mild, moderate and severe. ForMOA, two operatorsmanually segmented both hippocampus from all subjects. AOA was performed by FreeSurfer 5.3. Spearman's rho correlation coefficient was calculated for discrete variables and intraclass correlation coefficient was calculated for continuous variables. Results Correlation coefficients between the operators that performed MOA was 0.88 (p < 0.0001) for left hippocampi and 0.86 (p < 0,0001) for right hippocampi. Correlation coefficients between mean VSA and MOA was-0,81 (95% CI-0,96- -0,66). Correlation coefficients between AOA and AOM was 0.54 (p < 0.0001) for left hippocampi and 0.61 (p < 0.0001) for right hippocampi. Conclusion Even though AOA has moderate correlation with the gold standard it is not superior to average VSA and should be implemented with care in clinical practice.

Investigation of various events occurring in the brain tissue after calvarial defects in rats / Investigación de las alteraciones ocurridas en el tejido cerebral posterior a la creación de defectos en la calvaria de ratas

Bone damage and accidents, traumas can alter people's normal life, and damage the soft tissues. In this study, we aimed to investigate in calvarial defects in rats depending on the severity of cerebral contusion injury occurring in the temporal region. The rats were randomly divided into two groups: group 1 (control group), critical size cranial model with no treatment (n= 10); group 2 (14-day synthetic graft group given 7th day DEXA), critical size cranial model treated with Dexamethasone (0.05 mg/kg intramuscular injection) +Synthetic graft (n= 10) One calvarium defect of 7 mm was made in the parietal bone of each animal under general anesthesia. Calvarial defect results in dilatation of blood vessels, hemorrhage and deterioration was observed in glial fibrillary structures. Additionally, the increase in vascular endothelial growth factor expression showed a positive reaction with glial fibrillary acid protein astrocytes extensions. Apoptotic glial cells stained positive with Bcl-2. Calvarial defects caused by mild brain injury, to be induced by inflammatory cytokines, interrupting glial fibrillary degeneration by affecting the blood brain barrier is thought to promote apoptotic changes.

Daños óseos, accidentes y traumas pueden alterar la vida normal de las personas y dañar los tejidos blandos. Este estudio tuvo como objetivo investigar los defectos de calota en ratas en función de la gravedad de la lesión cerebral que ocurre en una contusión de la región temporal. Las ratas fueron divididas aleatoriamente en dos grupos: al grupo 1 (control), se le realizó un modelo de defecto craneal de tamaño crítico sin tratamiento (n= 10) y al grupo 2, se le realizó un modelo de defecto craneal de tamaño crítico que fue tratado con dexametasona (0,05 mg/kg vía i.m.) + injerto sintético (n= 10) (14 d con injerto sintético y el día 7 se le administró dexametasona). El modelo generó un defecto de 7 mm en el hueso parietal en cada animal, bajo anestesia general. Los defectos craneales produjeron dilatación de los vasos sanguíneos, hemorragias y deterioro en las estructuras gliales fibrilares. Además, el aumento de la expresión del factor de crecimiento vascular endotelial mostró una reacción con las positiva con la proteína ácida fibrilar de la glía el las extensiones de los astrocitos. Las células gliales apoptóticas se tiñeron positivas con Bcl-2. Los defectos de calota causan una lesión cerebral leve, inducidas por citoquinas inflamatorias, las que interrumpen la degeneración glial fibrilar al afectar la barrera hematoencefálica, induciendo cambios apoptóticos.

Síndrome de encefalopatía posterior reversible con afectación del tronco cerebral y médula espinal y retinopatía hipertensiva grado IV / Posterior reversible encephalopathy syndrome with brain stem and spinal cord involvement and grade iv hypertensive retinopathy

El síndrome de leucoencefalopatía posterior reversible se presenta con síntomas de edema cerebral e imágenes hiperintensas en las secuencias T2 y FLAIR en la resonancia magnética (RM) de cerebro. Las lesiones típicamente comprometen la región parietooccipital, aunque también pueden tener localizaciones atípicas. Presentamos el caso de una mujer de 33 años que consultó por trastornos visuales asociados con dolor de cabeza intenso e hipertensión grave (220/140 mmHg). El examen del fondo de ojo reveló retinopatía hipertensiva grado IV y la RM mostró lesiones hiperintensas en T2 y FLAIR a nivel centropontino, bulbo y médula espinal, las cuales regresaron tras el control sostenido de la presión arterial. (AU)

The posterior reversible leukoencephalopathy syndrome presents with symptoms of cerebral edema. On magnetic resonance imaging (MRI) of the brain, hyperintensities on T2 -weighted and FLAIR sequences typically affect the parietal-occipital region; however it may also have atypical locations. We report the case of a 33 year old patient with visual disturbances associated with intense headache and severe hypertension (220/140 mmHg). The Fundus examination revealed grade IV hypertensive retinopathy, and MRI showed hyperintense lesions in T2 and FLAIR affecting the pontine center, medulla and spinal cord, which regressed after sustain blood pressure control. (AU)

Right hand preference in implicit motor learning in children with high-functioning autism and Asperger syndrome

Cerebral hemispheres functioning have been found to be abnormal in children with ASD. The role of lateralization in implicit and explicit motor learning has received little attention in ASD researches. The main goal of this study is investigating the differences between two hands implicit and explicit motor learning in children with ASD and typical matched group. In the present random clinical trial study, 30 boys with ASD aged 7-11 were compared with 32 typical matched boys. Typical group and the ASDs, which were screened with ASSQ, were selected from elementary schools in Najafabad [Isfahan, Iran]. Participants performed a serial reaction time task [10 blocks] with each hand in implicit and explicit group with random allocation. Learning comparison between two groups showed significant difference which means explicit learning deficit in the ASDs with right [p=0.009] and left hand [p=0.004]. Results also indicated no significant difference in implicit learning between ASDs and typical matched group in right [p=0.385] and left hand [p=0.18]. Hands differences also showed speeded right hand in implicit learning in children with ASD [p=0.028] while no differences was seen in explicit learning and typical children. Explicit learning of right and left hand was impaired in children with ASD while implicit learning of both hands maintained intact and a right hand preference in implicit motor learning was observed in children with ASD due to left striatal system abnormality

Tos convulsa grave y su correlación anatomopatológica / Whooping cough and severe pathologic correlation

La tos convulsa es una infección bacteriana de las vías respiratorias, producida por Bordetella pertussis. En la era prevacunación fue una enfermedad endémica, con picos epidémicos cada 2 a 5 años. Luego de la vacunación generalizada, la tasa de infección disminuyó 150 veces. En Uruguay en el año 2011 hubo un aumento en los casos de tos convulsa, con mayor cantidad de ingresos a unidades de cuidados intensivos y de muerte por tos convulsa. Objetivo: describir los casos clínicos de 3 pacientes con tos convulsa grave que fallecieron en UCIN en el período de octubre-noviembre de 2011 y los hallazgos en la anatomía patológica. Se describen tres casos que se presentaron como tos coqueluchoide con fallo respiratorio que requirió asistencia ventilatoria mecánica. En los tres se confirmó el diagnóstico de infección por Bordetella pertussis. Los tres presentaron hiperleucositosis severa que requirió exanguinotransfusión y desarrollaron hipertensión pulmonar (HTP) severa con shock cardiogénico y fallo renal anúrico. En la anatomía patológica de los 3 pacientes se observó necrosis miocárdica extensa, necrosis tubular aguda y necrosis neuronal con edema cerebral. Conclusión: la tos convulsa ha resurgido en los últimos años, siendo de mayor gravedad en menores de 6 meses. Se destaca lo estereotipado de la presentación y evolución con hipoxemia refractaria e HTP que llevan al colapso cardiocirculatorio...

Expressão diferencial de marcadores relacionados ao ciclo celular e à morte celular entre doença de Alzheimer sintomática e assintomática em cérebros humanos post-mortem / Differential expression of cell cycle and cell death markers between symptomatic and asymptomatic Alzheimer´s disease in post-mortem human brains

A doença de Alzheimer (DA) é caracterizada por um declínio cognitivo progressivo associado ao acúmulo de peptídeo β-amilóide (placas neuríticas), proteína tau hiperfosforilada (emaranhados neurofibrilares), degeneração sináptica e morte neuronal no hipocampo e em outras regiões corticais. Vários estudos apontam uma reativação do ciclo celular em neurônios pós-mitóticos na DA, o que levaria à morte neuronal. Porém, ainda não existe um estudo que avalie marcadores do ciclo celular em indivíduos portadores da neuropatologia típica da DA, mas que não apresentem evidências de comprometimento cognitivo (DA assintomática). Diante disso, este trabalho pretende verificar se existe diferença entre indivíduos com DA sintomática, DA assintomática e indivíduos normais em relação a marcadores do ciclo celular e de morte celular. Nossos resultados mostram alterações significantes de marcadores do ciclo e morte celular nos indivíduos com DA sintomática comparados aos com DA assintomática e aos normais, enquanto que, entre os indivíduos com DA assintomática e sujeitos normais, não existem diferenças significativas. Este trabalho sugere associação entre o controle da maquinaria do ciclo celular nos neurônios pós-mitóticos, e a manutenção do status cognitivo normal...

Alzheimer's disease (AD) is characterized by progressive cognitive decline associated with accumulation of amyloid-β peptide (neuritic plaques), hyperphosphorylated tau protein (neurofibrillary tangles), synaptic degeneration and neuronal death in the hippocampus and in other cortical regions. Several studies indicate a reactivation of the cell cycle in AD post-mitotic neurons, leading to neuronal death. However, studies evaluating cell cycle markers in patients with AD neuropathology, but with no evidence of cognitive impairment (asymptomatic AD) are lacking. Therefore, this study intends to investigate whether there are differences among subjects with symptomatic AD, asymptomatic AD and normal individuals in relation to cell cycle and cell death markers. Our results show significant changes in both cell cycle and cell death markers in subjects with symptomatic AD compared to asymptomatic AD and normal individuals, while between asymptomatic AD individuals and normal subjects, there were no significant differences. This study suggests an association between the control of cell cycle machinery in post-mitotic neurons, and maintenance of normal cognitive status...

Leucoencefalopatía hipóxica tardía / Delayed Hypoxic Leukoencephalopathy

La Leucoencefalopatía Hipóxica Tardía (LHT) es una enfermedad desmielinizante de infrecuente presentación. Se produce como consecuencia de un fenómeno de hipoxia-anoxia y aparece días o semanas más tarde. Los síntomas y cambios imagenológicos remiten casi totalmente y tienen un buen pronóstico En este reporte se ilustra el caso de una joven que desarrolló LHT luego de sufrir un paro cardiorrespiratorio...

Estudos comportamentais e das alterações histopatológicas em camundongos pré-tratados com Bellis perennis no modelo de convulsão induzido por pilocarpina / Behavioral studies and histopathological changes in mice pretreated with Bellis perennis in pilocarpine-induced seizures

The aim of this study was to investigate the potential neuroprotective and anticonvulsant effects of ethanolic extract from flowers (EEF) of B. perrenis in adult Swiss mice (2 months old) after seizures induced by pilocarpine. The animals were divided into 8 groups. The first group was treated with vehicle (0.05 percent Tween 80 dissolved in 0.9 percent saline) and the second with pilocarpine (400 mg/kg, P400 group). The third, fourth and fifth group were pretreated with EEF (50, 100 or 150 mg/kg) and 30 min later received P400 (EEF 50, EEF 100 or EEF 150 plus P400 groups), respectively. In turn, the remaining groups were treated with EEF alone (50, 100 or 150 mg/kg EEF 50, EEF 100 or EEF 150 groups), respectively. After treatment, the groups were observed for 24 h and then euthanized and their brains removed for histopathological analysis. All P400 group animals showed seizures that progressed to status epilepticus. Pre-treatment with EEF produced a significant reduction in those indices. P400 and EEF 50 plus P400 groups showed 87.5 percent and 37.5 percent of animals with brain damage in the hippocampus, respectively. In P400 group, the damage rate in striatum was 75 percent. In turn, this region has seen a reduction of 46.99 percent neuronal damage of those of EEF 50 plus P400 group. According to our results we suggest that the EEF may modulate epileptogenesis and promote anticonvulsant and neuroprotective mechanisms in model of seizures induced by pilocarpine.

O objetivo desse estudo foi investigar o potencial efeito neuroprotetor e anticonvulsivante do extrato etanólico das flores de B. perrenis (EEF) em camundongos Swiss adultos (2 meses) após convulsão induzida por pilocarpina. Os animais foram divididos em 8 grupos. O primeiro grupo foi tratado com veículo (Tween 80 0,05 por cento dissolvido em salina 0,9 por cento) e o segundo com pilocarpina (400 mg/kg, grupo P400). Já o terceiro, quarto e quinto grupo foram tratados com EEF (50, 100 ou 150 mg/kg), e 30 min depois receberam P400 (grupos EEF 50, EEF 100 ou EEF 150 plus P400), respectivamente. Por sua vez, os demais grupos foram tratados somente com EEF (50, 100 ou 150 mg/kg; grupos EEF 50, EEF 100 ou EEF 150), respectivamente. Após os tratamentos, os grupos foram observados durante 24 h e em seguida eutanasiados e seus cérebros removidos para as análises histopatológicas. Todos os animais do grupo P400 apresentaram convulsões que progrediram para o estado de mal epiléptico. O pré-tratamento com EEF produziu uma redução significativa nesses índices. Os grupos P400 e EEF 50 plus P400 apresentaram 87,5 por cento e 37,5 por cento de animais com lesão cerebral no hipocampo, respectivamente. No corpo estriado dos animais do grupo P400 houve um comprometimento de 75 por cento. Por sua vez, nessa região foi vista uma redução de 46,99 por cento nesse comprometimento nos animais do grupo EEF 50 plus P400. De acordo com nossos resultados podemos sugerir que o EEF pode modular a epileptogênese e promover ação neuroprotetora e anticonvulsivante no modelo das convulsões induzidas por pilocarpina.

Detección de b-Amiloide, proteína TAU hiperfosforilada y ubiquitina por técnica de inmunohistoquímica en cerebros de caninos mayores de 10 años / Detection b-Amiloid, hyperphosphorylate TAU protein and ubiquitin for technique the immunohistochemistry in brains in dogs over 10 years

El interés en el estudio del proceso de envejecimiento cerebral y los cambios comportamentales relacionados con la edad en caninos y felinos geriátricos ha aumentado en la última década (Mentzel, 2005a; Ingram & Williams, 2010). Las alteraciones del comportamiento canino que responden a cambios fisiopatológicos relacionados con la edad y que involucran distintas esferas conductuales y cognitivas, se engloban bajo la denominación de Síndrome de Disfunción Cognoscitiva (SDC) (Mentzel, 2005b). El cual fue descrito en 1997 y se observó en perros de más de 9 años (Ingram & Williams). El Síndrome de Disfunción Cognoscitivo (SDC) es también llamado Alzheimer canino por las similitudes histopatológicas cerebrales y conductuales relacionadas. En el presente estudio, los caninos geriátricos presentaron los mismos depósitos proteicos anormales presentes en la enfermedad de Alzheimer, como las placas seniles, los cuerpos ubiquitinados y la angiopatía amiloide, pero no presentaron los ovillos neurofibrilares, los cuales son los responsables de la demencia que se padece en la enfermedad. Las únicas herramientas de evaluación prácticas en clínica para esta patología son las encuestas y formularios relacionados con aspectos comportamentales. Además existen exámenes diagnósticos postmortem que permiten identificar de una manera eficaz la presencia de elementos anormales involucrados en la neuropatología, uno de ellos es la inmunohistoquímica. En este estudio se utilizaron los anticuerpos monoclonales de uso humano anti-b-amiloide, anti-proteína tau y anti-ubiquitina en muestras de cerebros de perros mayores de 10 años. El modelo canino hoy en día está constituyendo un significado indispensable para el estudio de los procesos de neurodegeneración porque ha permitido un acercamiento a la teoría del problema desde nuevas perspectivas (Dimakopoulos & Mayer, 2002).

The interest in the study of the process of cerebral aging and the changes in the behaviour related to age in geriatric canine and feline has increased in the last decade (Mentzel, 2005a; Ingram & Williams, 2010). The alterations of the canine behaviour that respond to physiopathological changes related to the age and which involve different conducts and cognitive spheres are included under the denomination of Canine Cognitive Dysfunction Syndrome (CDS) called canine Alzheimer, considering the cerebral and histopathologic behavioural similarities. In the present study the geriatric patients displayed the same abnormal protein deposits present in Alzheimer disease, such as senile plaques ubiquitinate bodies and the amyloid angiopathy. However they did not display the balls of neurofibrillary tangles which are characteristic of the disease. The only practical tools of evaluation in clinic for this pathology are the surveys and forms related to behavioural aspects. In addition there are post mortem exams such as those in inmunohistochemistry, that allow an effective identification of abnormal elements present in the neuropathology. In this study the monoclonal antibodies were used anti-b-amyloid, anti-protein tau and anti-ubiquitin in samples of brains in dogs over 10 years of age. The canine model constitutes an indispensable meaning for the study of the neurodegeneration processes because it has allowed an approach to the theory of the problem from a new perspective (Dimakopoulos & Mayer, 2002).

Enfermedad de Addison en un niño con adrenoleucodistrofia ligada al cromosoma X / Primary adrenal failure associated to X-linked adrenoleukodystrophy: report of one case

Primary adrenal failure (PAF) can be congenital or acquired. X-linked adrenoleukodystrophy (ALD-X), produced by the mutation of the ABDC1 gene (Xq28), that leads to the plasma accumulation of very long chain fatty acids, is one of the congenital diseases associated to adrenal destruction. We report a 7 years old boy with fast progression of right strabismus and general symptoms as weariness, weakness and mucosal and skin pigmentation. A brain magnetic resonance image showed a leukoencephalopathy, characteristic of ALD-X. Low plasma cortisol, high ACTH levels and lack of response to ACTH test, confirmed the diagnosis of primary adrenal insufficiency. High plasma levels of C26:0 fatty acids, and C24/22, C26/22 ratios confirmed ALD-X.

Clima, cerebro y degeneración en Cabanis / Climate, brain and degeneration in Cabanis

Son analizados los argumentos defendidos por Cabanis en el Rapports du physique et du moral de l'homme, tomando como punto de partida las Memorias Octava y Novena. Estas Memorias, dedicadas a analizar las influencias del régimen y del clima, permiten leer de otro modo los argumentos utilizados por Cabanis para explicar la relación entre las características físicas y los hábitos morales de individuos y razas. Son analizadas las deudas con la historia natural, particularmente con la teoría de la degeneración de Buffon; con la tradición médica hipocrática, fundamentalmente con la teoría de los humores, y con los estudios de anatomía y patología cerebral. Finalmente, examinamos el papel que la medicina y la higiene, pensadas como ciencias humanas y morales, poseen en los proyetos de la regeneración de la especie humana.

Brain perfusion abnormalities associated to drug abuse in recent abstinent patients using SPECT 99m Tc-ethylen-cysteinate-dimer (ECD)

Several substances may produce brain perfusion abnormalities in drug-dependent patients. Their mechanism is unclear and several causes might be involved, especially vasospasm in cocaine consumption. Goal: To characterize residual brain perfusion abnormalities in substance-dependent population. We analyzed brain perfusion in 100 dependant patients (DSM-IV criteria) following a month of strict in-hospital abstinence (age:35 +/- 2y.o.; 86 percent men); 55 percent corresponded to poly-drug dependents, mainly to cocaine, alcohol and cannabis; 44 percent mono-drug users, mostly to alcohol. Results: Single Photon Emission Computed Tomography (SPECT) with 99mTc-ethylen-cysteinate-dimer (ECD) was abnormal in 54 percent of the cases, with bilateral cortical hypo-perfusion in 89 percent, focal in 54 percent and diffuse in 46 percent of them, with moderate or severe intensity in 61 percent. The abnormal perfusion group’s age was 38 +/- 12 versus 31 +/- 10 years in the normal SPECT group (P=0.005) with a consumption period of 16 +/- 11 versus 11 +/- 8 years, respectively (P=0.043). Only 29 percent of women had abnormal perfusion versus 58 percent of men (P=0.047). Abnormal brain perfusion in 64 percent of mono and 45 percent in poly-drug dependents (P=0.07). Psychometric tests performed in 25 patients demonstrated association between perfusion defects and cognitive abnormalities. Relative risk for abnormal psychometric test was 2.5 [95 percent;CI=1.1-5.6] for abnormal SPECT. Conclusion: Dependent population after a month of abstinence persists with cortical brain perfusion abnormalities, associated to age, sex and type of drug consumption.

Leucoencefalopatía hipóxico-isquémica retardada: caso clínico y revisión de la literatura / Hypoxic-ischemic delayed leukoencephalopathy: case report and review of the literature

Delayed Hypoxic-isquemic Leucoencephalopathy described in 1976 by Ginsberg is a brain white matter demyelinization phenomenon that occurred days or weeks after a hypoxic-isquemic injury followed by a complete recovery of the episode. The pathogenesis process remains unknown. We describe a 48 year old woman with cervico-uterine cancer in palliative treatment with opoids. She enters the emergency room with a respiratory depression, a prolonged hypotension and confusion, that it was recovered. At admission exhibits a recurrent pneumonia. Two weeks later, in conditions of discharge, initiates with agitation in context with rapidly progressive decline cognition, with concordant lesions of Leucoencephalopathy defined in the Magnetic Resonance (MR) study The metabolic profile, the cerebrospinal fluid and the electroencephalogram allowed dismissing other etiologic hypothesis. In front to the suspicious of Ginsberg syndrome, she had normal levels of Arylsulfatase. This acute post-hypoxic demyelinization process has been pathogenic interpreted as an arylsulfatase deficiency. Although numerous cases develop with normal arylsulfatase and the experimental studies of hypoxia, has support the hypothesis of a central hypoxic axonopathy due to failing in axonal transport as the base of the demyelinization phenomenal.

La leucoencefalopatía hipóxico-isquémica retardada (EHIR), descrita por Ginsberg en el año 1976, es un fenómeno desmielinizante de la sustancia blanca cerebral, que se origina días o semanas después de un daño hipóxico-isquémico que había sido seguido de una recuperación completa del episodio. La patogenia del proceso no está completamente establecida. Se presenta una mujer de 48 años portadora de cáncer cérvicouterino, en tratamiento paliativo con opiáceos. Ingresa al Servicio de Urgencia por una depresión respiratoria, hipotensión prolongada y compromiso de conciencia, donde es recuperada. Se hospitaliza por una neumopatía intercúrrente. Dos semanas más tarde, estando en condiciones de alta, se inicia agitación psicomotora en el contexto de un deterioro cognitivo rápidamente progresivo, que el estudio de Resonancia Magnética (RM) definió como lesiones concordantes con una leucoencefalopatía. El perfil metabólico, el líquido cefalorraquídeo y el electroencefalograma, permitieron descartar otras hipótesis etiológicas. Frente a la sospecha de un síndrome de Ginsberg, los niveles de arilsulfatasa fueron normales. Este proceso desmielinizante agudo post-hipóxico, ha sido interpretado patogénicamente como un déficit de arilsulfatasa. Sin embargo, la existencia de numerosos casos que cursan con arilsulfatasa normal y los estudios experimentales de hipoxia, ha avalado la hipótesis de una axonopatía central hipóxica, atribuible a fallas del transporte axoplásmico, como base para el fenómeno desmielinizante.

Foreign accent syndrome in Iran: case report

Foreign Accent Syndrome is a rare speech disorder caused by the damage sustained to left hemisphere of the brain, in which a combination of segmental and suprasegmental deviations in speech production creates foreign accent. Despite of segmental pronunciation problems, prosodic errors are mainly considered as the main characteristics of Foreign Accent Syndrome. The present paper offers a report on a 53-year Persian-speaking woman who is pronouncing the words and sentences with foreign accent after a stroke and central semiovale focal infarctions of left hemisphere. According to researchers' information and follow-up done, this patient is considered as the first case of FAS in Iran. Prosodic characteristics of the patient's speech have been compared with speech features of ordinary speakers of Persian language. Being in direction of previously reported research findings, the obtained results show that there are main deviations in prosodic characteristics of the patient's speech which are related to the speech rate, stress, and juncture