Encefalopatía traumática crónica, definición, diagnóstico y prevención: revisión de la literatura / Chronic traumatic encephalopathy, definition, diagnosis and prevention: review of literature

La encefalopatía traumática crónica (ETC) es una enfermedad neurodegenerativa que se produce como consecuencia traumatismos cerebrales repetitivos; concusiones, que son un síndrome clínico que se caracteriza por una alteración de la función cerebral. Una concusión, bajo su estricta definición, no debiese causar cambios estructurales en el cerebro por lo que no sería visible a través de imágenes, sí existen cambios a nivel microscópicos, bioquímicos y biomecánicos. La mayoría de los pacientes tienen completa resolución de sus síntomas dentro de 10 días (90 por ciento), pero existe un pequeño porcentaje que persiste con estos, pudiendo presentarse como un síndrome postconcusional, síndrome de segundo impacto o una encefalopatía traumática crónica. La ETC se caracteriza por la acumulación de prot-tau hiperfosforilada en neuronas y astrocitos. Estas se van a presentar en forma de ovillos o hilos neurofibrilares. En etapas iniciales las encontraremos de forma focalizada en la corteza frontal y en las formas más severas su distribución será más generalizada, distribuyéndose en la mayoría de las regiones del cerebro. Su diagnóstico se realiza a través de histopatología, por lo que hasta el momento sólo se ha logrado post-mortem. Se está trabajando en nuevas tecnologías asociadas a biomarcadores y PET para lograr una diagnostico premortem. El mayor énfasis en el manejo de esta taupatía es la prevención y adecuado manejo de las concusiones.

Chronic Traumatic Encephalopathy (CTE) is a neurodegenerative disease which is produced as a consequence of repeated brain trauma: concussions, which are a clinical syndrome characterized by an alteration in brain functions. A concussion, understrict definition, should not cause structural changes to the brain. Therefore, it would not be possible to see through images if there were changes at a microscopic, biochemical level. Most patients see their symptoms completely resolved within 10 days (90 percent), but there is a small percentage which persists, and these might cause a post-concussional syndrome, second impact syndrome of chronic traumatic encephalopathy. CTE is characterized by the accumulation of hyper-phosphorylated Tau protein in neurons and astrocytes. These appear in the form of neurofibrillary tangles. During the initial stages they are focalized in the frontal cortex and, in more severe cases, their distribution is more generalized, spreading through the majority of the regions in the brain. It is diagnosis is done through histopathology. Thus, it has only been possible to do post mortem. New technologies associated with bio-markers and PET are being worked on to achieve a pre-mortem diagnosis. The greatest emphasis in the handling of this tauopathy lies in the prevention and the adequate handling of concussions.

Impact of an educational program on the feeding of neurologically impaired children / Impacto de uma acao educativa na alimentacao de criancas neuropatas

PURPOSE: To verify the impact of an educative program focused on aspects related to feeding developed with a group of caregivers of children with chronic non-progressive encephalopathy. METHODS: Cross-sectional comparative study conducted with 30 children diagnosed with chronic non-progressive encephalopathy and their caregivers with the use of a questionnaire and video recordings of a meal conducted by the main caregiver. In order to verify the impact of an educational program in the knowledge and conduct of caregivers, patients were divided into two groups: study - consisting of caregivers submitted to a questionnaire and a video recording before and after the educational program; control - group in which caregivers underwent the procedures in two occasions, but without access to the educational program. RESULTS: Around 93.33% of caregivers were females, most had low educational level, and only 10% had a professional activity. Previous knowledge of caregivers concerning feeding was restricted, with 66% of caregivers not knowing what aspiration was, 60% being unfamiliar with the complications associated with such occurrence, and 86.66% stating that there is no relation between voice and swallowing. During feeding, only 26.66% of the caregivers used verbal commands related to feeding, and 50% did not realize the difficulties presented by their children. We observed a difference with regard to knowledge and conduct in the study group only. CONCLUSION: The educational program had a positive impact on the knowledge and conduct of caregivers concerning the feeding of their children with chronic non-progressive encephalopathy. .

OBJETIVO: Verificar o impacto de uma ação educativa voltada aos cuidados com a alimentação desenvolvida com um grupo de cuidadores de crianças com encefalopatia crônica não progressiva (ECNP). MÉTODOS: Estudo comparativo transversal realizado com 30 crianças com ECNP e seus cuidadores, tendo-se realizado aplicação de questionário e registro de uma refeição conduzida pelo cuidador principal. A fim de se verificar o impacto da ação educativa no conhecimento e conduta dos cuidadores, estes foram divididos em dois grupos: estudo, composto por cuidadores submetidos à aplicação de questionário e registro em vídeo antes e após realização da ação educativa; e controle, grupo submetido aos procedimentos em dois momentos, porém sem acesso à ação educativa. RESULTADOS: 93,33% dos cuidadores eram do sexo feminino, a maioria apresentou baixo nível de escolaridade e apenas 10% desenvolviam alguma atividade profissional. O conhecimento prévio dos cuidadores quanto aos aspectos relacionados à alimentação foi restrito, tendo 66% destes referido não saber o que era aspiração, 60% afirmado não saber as complicações associadas a esta e 86,66% terem negado existir relação entre voz e deglutição. Durante a alimentação, apenas 26,66% dos pais fizeram uso de comandos verbais relacionados à alimentação e 50% não perceberam as dificuldades apresentadas pelo seu filho. Observou-se diferença quanto ao conhecimento e conduta de cuidadores apenas no grupo estudo. CONCLUSÃO: A ação educativa realizada apresentou impacto no conhecimento e conduta dos cuidadores quanto à alimentação de seus filhos com ECNP. .

[Disorders of executive functions. Diagnosis and treatment]. / Trastornos de las funciones ejecutivas. Diagnóstico y tratamiento.

INTRODUCTION: Different contributions to the scientific literature made over the last few decades have confirmed the prefrontal cortex as the neurobiological basis of the executive functions. Today, both the evaluation protocols for carrying out diagnoses and the structuring of the treatment and neurocognitive stimulation plans must interpret each of the prefrontal syndromes (dorsolateral, medial or of the anterior cingulate, and orbitofrontal) involved in the aetiopathogenesis of the different neurodevelopmental and adult disorders in which the executive functions are affected. AIMS: To report on the latest advances in diagnosis and treatment of the executive functions and to stress the importance of identifying and understanding the three syndromes of prefrontal dysfunction in the phase of diagnosis and in neurocognitive rehabilitation. DEVELOPMENT: The authors carry out a review of the literature on the latest advances in neuroscience as regards the neurobiological and neuropsychological foundations of the executive functions and their diagnosis and treatment. CONCLUSIONS: Every day, the scientific community confirms the importance of identifying and understanding the brain circuits in the diagnostic stage, especially the prefrontal dysfunction syndromes involved in the neuropsychological deficits of the different neurodevelopmental and adult disorders, so as to be able to establish effective neurocognitive stimulation protocols.

TITLE: Trastornos de las funciones ejecutivas. Diagnostico y tratamiento.Introduccion. Los diferentes aportes de la literatura cientifica ratifican, desde hace un par de decadas, a la corteza prefrontal como base neurobiologica de las funciones ejecutivas. En la actualidad, tanto los protocolos de evaluacion para la realizacion del diagnostico como la estructuracion de los planes de tratamiento y estimulacion neurocognitiva deben hacer la lectura de cada uno de los sindromes prefrontales (dorsolateral, medial o del cingulo anterior, y orbitofrontal) implicados en la etiopatogenia de los diferentes trastornos del neurodesarrollo y del adulto en los que se ven afectadas las funciones ejecutivas. Objetivos. Exponer los ultimos avances sobre diagnostico y tratamiento de las funciones ejecutivas y resaltar la importancia de identificar y comprender en la fase diagnostica y en la rehabilitacion neurocognitiva los tres sindromes de disfuncion prefrontal. Desarrollo. Se realiza una revision bibliografica sobre los ultimos avances neurocientificos alrededor de las bases neurobiologicas, neuropsicologicas, diagnostico y tratamiento de las funciones ejecutivas. Conclusion. Cada dia la comunidad neurocientifica ratifica la importancia de identificar y comprender en la etapa diagnostica los circuitos cerebrales, especificamente los sindromes de disfuncion prefrontal implicados en los deficits neuropsicologicos de los diferentes trastornos del neurodesarrollo y del adulto para poder establecer protocolos eficaces de estimulacion neurocognitiva.

Impact of an educational program on the feeding of neurologically impaired children.

PURPOSE: To verify the impact of an educative program focused on aspects related to feeding developed with a group of caregivers of children with chronic non-progressive encephalopathy. METHODS: Cross-sectional comparative study conducted with 30 children diagnosed with chronic non-progressive encephalopathy and their caregivers with the use of a questionnaire and video recordings of a meal conducted by the main caregiver. In order to verify the impact of an educational program in the knowledge and conduct of caregivers, patients were divided into two groups: study--consisting of caregivers submitted to a questionnaire and a video recording before and after the educational program; control--group in which caregivers underwent the procedures in two occasions, but without access to the educational program. RESULTS: Around 93.33% of caregivers were females, most had low educational level, and only 10% had a professional activity. Previous knowledge of caregivers concerning feeding was restricted, with 66% of caregivers not knowing what aspiration was, 60% being unfamiliar with the complications associated with such occurrence, and 86.66% stating that there is no relation between voice and swallowing. During feeding, only 26.66% of the caregivers used verbal commands related to feeding, and 50% did not realize the difficulties presented by their children. We observed a difference with regard to knowledge and conduct in the study group only. CONCLUSION: The educational program had a positive impact on the knowledge and conduct of caregivers concerning the feeding of their children with chronic non-progressive encephalopathy.

[Visual neurorehabilitation of patients with cerebral damage using botulinum toxin]. / ción visual con toxina botulínica en pacientes con daño cerebral.

BACKGROUND: The neurorehabilitation of the patient with cerebral damage implies the reestablishment of the visual functions. Botulinum toxin can be considerate as a less invasive alternative for treatment. OBJECTIVE: to demonstrate the answer to the treatment using botulinum toxin of the visual motor alterations in patients with cerebral damage. METHODS: Descriptive study of patients with visual alterations associated to cerebral damage. The visual treatment included three areas: sensorial, refracting and motor under quimiodenervation with botulinum toxin, of May 2009 to May 2010. RESULTS: 48 patients were studied, age 22,4 years ± 23. The strabismus were: esotropia 52%, exotropia 39,5%, vertical 8%, nystagmus 4%. 50% of the patients had psychomotor delay. Some of the most important causes of cerebral damage were: Down syndrome, epilepsy, tumor, hydrocephalus, neuroinfection, infantile cerebral paralysis, multiple sclerosis, metabolic syndrome, cranial trauma, congenital cardiopathy, ventricular hemorrhage, cerebrovascular stroke. The dose of botulinum toxin was 8,1 UI ± 3. We registered good results in 56.5%, regular 23,9% and bad 19,5%. The global percentage of rehabilitation was 69% of correction with a r of Pearson of 0,5. DISCUSSION: Patients with cerebral damage have diverse types of visuomotor alterations, strabismus and nystagmus.Use of botulinum toxin as a paralytic muscle agent is a good alternative in these cases. CONCLUSION: The botulinum toxin is an effective option for the visual rehabilitation in patients with cerebral damage and prevents the progression of more cerebral changes secondary to strabismus.

Elevated concentrations of inflammation-related proteins in postnatal blood predict severe developmental delay at 2 years of age in extremely preterm infants.

OBJECTIVE: To evaluate the hypothesis that elevated levels of inflammation-related proteins in early postnatal blood predict impaired mental and motor development in extremely preterm infants. STUDY DESIGN: We measured concentrations of 25 inflammation-related proteins in blood collected on postnatal days 1, 7, and 14 from 939 infants born before 28 weeks gestation. An elevated level was defined as a concentration in the highest quartile for gestational age and day of blood collection. We identified impaired development at age 24 months using the Bayley Scales of Infant Development, Second Edition. The primary outcomes were scores on the Mental Scale or the Motor Scale of <55 (more than 3 SDs below the mean). RESULTS: For 17 of the 25 inflammation-related proteins, 1 or more statistically significant associations (P<.01) was found between an elevated blood level of the protein and a developmental impairment. Elevations on multiple days were more often associated with developmental impairment than were elevations present for only 1 day. The highest number of predictive elevations was found in day-14 blood. CONCLUSION: In extremely preterm infants, elevated levels of inflammation-related proteins in blood collected on postnatal days 7 and 14, especially when sustained, are associated with impaired mental and motor development at age 2 years.

[Permanent consecutive exotropia in children treated with botulinum toxin]. / Exotropía consecutiva permanente en niños tratados con toxina botulínica.

BACKGROUND: Congenital esotropia is the most frequent type of strabismus. Botulinum toxin is a treatment option with variable results. We undertook this study to determine the frequency and associated factors with consecutive permanent esotropia in patients diagnosed with congenital esotropia treated with botulinum toxin. METHODS: A retrospective review was achieved in patients with congenital esotropia treated with botulinum toxin and who remained in esotropia after a minimal follow-up of 6 months. Pre- and postnatal background, cycloplejia, magnitude of the pre-application deviation, injected dose and number of applications were analyzed. RESULTS: A total of 84 patients were included. Of all patients,12 (14.28%) remained in consecutive esotropia (six males and six females). Age range was from 5 months to 2 years (average: 10.75 months). Initial esotropia ranged from 20-50 prism diopters (PD) with an average of 37.9 ± 9.64 PD. One patient had variability in the magnitude of the deviation prior to treatment. During the maximum follow-up, the magnitude of the esotropia was from 10 to 40 (average, SD 18 ± 8.01 PD). In five subjects a variability was observed in the magnitude of the angle of deviation by a range of 10-40 PD, and in seven subjects the deviation was stable with an average of 20 PD. Eleven subjects had some degree of psychomotor delay (91.66%) and one subject had a non-significant history. CONCLUSIONS: In patients with consecutive permanent esotropia after application of botulinum toxin, the most prevalent characteristic is neurological.

[Clinical and endoscopic outcome after Nissen fundoplication for gastroesophageal reflux disease]. / Evolução clínica e endoscópica após fundoplicatura para tratamento da doença do refluxo gastroesofágico.

CONTEXT: The surgical treatment of gastroesophageal reflux disease is indicated in patients with a chronic condition when proton pump inhibitors therapy is ineffective or when symptoms are persistently recurrent. Fundoplication failure occurrence has been detected in some patients and highlights a needing of monitoring esophageal post-surgical condition since that clinical symptoms are not sensitive enough for indicating fundoplication failure. AIMS: To identify the frequency of a disrupted wrap in the postoperative period and to evaluate esophageal complications related with gastroesophageal reflux recurrence in children and adolescents. METHODS: The study was cross-sectional, prospective and descriptive, including 45 patients (16 months-16.9 years) who had undergone Nissen fundoplication in a school hospital. Twenty six patients (57.8%) were neurologically impaired. Upper gastrointestinal endoscopy was performed in order to determine fundoplication integrity, endoscopic and histopathological esophageal condition. Upper gastrointestinal endoscopies were performed from 12 up to 30 months after surgery. RESULTS: Patent wrap was identified in 41 patients (91.1%). Recurrent peptic esophagitis was found in 6 of 45 patients, 2 of which required a second fundoplication and other two had Barrett esophagus. Endoscopic peptic esophagitis was associated with a defective wrap (P = 0.005). Conclusions - Fundoplication was effective for treating esophagitis, even in patients with previous esophageal stenosis. Endoscopic follow up may detect surgery failure in children undergone anti-reflux surgery even in asymptomatic patients.

Evolução clínica e endoscópica após fundoplicatura para tratamento da doença do refluxo gastroesofágico / Clinical and endoscopic outcome after Nissen fundoplication for gastroesophageal reflux disease

CONTEXTO: O tratamento cirúrgico da doença do refluxo gastroesofágico está indicado para pacientes com doença crônica, associada ou não a complicações. A fundoplicatura é frequentemente indicada em substituição ao uso contínuo dos inibidores de bomba de prótons, para pacientes sem resposta, resposta parcial, ou dependentes de tratamento medicamentoso, ou ainda, quando houver recurrência dos sintomas com a descontinuação das medicações. No período pós-operatório, ocorrências de desmanche da válvula com recurrência da doença do refluxo indicam a necessidade de monitorização do procedimento cirúrgico. A avaliação do funcionamento da válvula, baseada na presença de sintomas, tem se mostrado instrumento ineficaz para essa monitorização. OBJETIVO: Identificar a frequência de anormalidades na válvula antirrefluxo e a frequência de complicações pépticas do esôfago no período de pós-operatório tardio da fundoplicatura em crianças e adolescentes. Métodos - Em estudo transversal, prospectivo e descritivo, em que foram avaliados 45 pacientes (idade = 16 meses a 16,9 anos) que tinham realizado fundoplicatura de Nissen num período de 12 a 30 meses prévios à avaliação. O aspecto da fundoplicatura e da mucosa esofágica foram avaliados por meio de endoscopia digestiva alta e estudo histológico. RESULTADOS: Doentes com encefalopatia crônica corresponderam a 26/45 (57,8 por cento) dos casos. Válvula antirrefluxo intacta foi identificada em 41/45 (91,1 por cento) dos pacientes. A fundoplicatura foi efetiva no tratamento do processo inflamatório esofágico, mesmo quando identificadas subestenose ou estenose de esôfago, no pré-operatório. As complicações identificadas foram esofagite péptica (6/45 pacientes) e necessidade de nova fundoplicatura (2/45 pacientes). A presença de anormalidades na válvula antirrefluxo associou-se ao achado de esofagite péptica (P = 0,005). Dois pacientes receberam o diagnóstico de esôfago de Barrett. CONCLUSÃO: A fundoplicatura...

CONTEXT: The surgical treatment of gastroesophageal reflux disease is indicated in patients with a chronic condition when proton pump inhibitors therapy is ineffective or when symptoms are persistently recurrent. Fundoplication failure occurrence has been detected in some patients and highlights a needing of monitoring esophageal post-surgical condition since that clinical symptoms are not sensitive enough for indicating fundoplication failure. AIMS: To identify the frequency of a disrupted wrap in the postoperative period and to evaluate esophageal complications related with gastroesophageal reflux recurrence in children and adolescents. METHODS: The study was cross-sectional, prospective and descriptive, including 45 patients (16 months-16.9 years) who had undergone Nissen fundoplication in a school hospital. Twenty six patients (57.8 percent) were neurologically impaired. Upper gastrointestinal endoscopy was performed in order to determine fundoplication integrity, endoscopic and histopathological esophageal condition. Upper gastrointestinal endoscopies were performed from 12 up to 30 months after surgery. RESULTS: Patent wrap was identified in 41 patients (91.1 percent). Recurrent peptic esophagitis was found in 6 of 45 patients, 2 of which required a second fundoplication and other two had Barrett esophagus. Endoscopic peptic esophagitis was associated with a defective wrap (P = 0.005). Conclusions - Fundoplication was effective for treating esophagitis, even in patients with previous esophageal stenosis. Endoscopic follow up may detect surgery failure in children undergone anti-reflux surgery even in asymptomatic patients.

Babies with brain damage who can not swallow: surgical management.

BACKGROUND: Neonates with severe neurological impairment are often unable to swallow, necessitating gastrostomy for feeding. Because of the risk of developing severe reflux, this procedure is often associated with fundoplication. OBJECTIVE: To assess the safety and efficacy of gastrostomy and Nissen fundoplication in 22 neonates with swallowing difficulties due to serious neurological impairment. METHOD: All children underwent an initial period of nasogastric feeding and after informed consent underwent gastrostomy and Nissen fundoplication. RESULTS: There were no significant intraoperative complications. There were two cases of postoperative periostomy leakage. Of the 22 neonates 16 were alive four months after surgery. Six neonates died of complications due to underlying disease. CONCLUSION: We concluded that gastrostomy and Nissen fundoplication are safe procedures and help parents give a better care to these children.

Babies with brain damage who can not swallow: surgical management / Recém-nascidos com lesão cerebral que não deglutem: manejo cirúrgico

BACKGROUND: Neonates with severe neurological impairment are often unable to swallow, necessitating gastrostomy for feeding. Because of the risk of developing severe reflux, this procedure is often associated with fundoplication. OBJECTIVE: To assess the safety and efficacy of gastrostomy and Nissen fundoplication in 22 neonates with swallowing difficulties due to serious neurological impairment. METHOD: All children underwent an initial period of nasogastric feeding and after informed consent underwent gastrostomy and Nissen fundoplication. RESULTS: There were no significant intraoperative complications. There were two cases of postoperative periostomy leakage. Of the 22 neonates 16 were alive four months after surgery. Six neonates died of complications due to underlying disease. CONCLUSION: We concluded that gastrostomy and Nissen fundoplication are safe procedures and help parents give a better care to these children.

INTRODUÇÃO: Neonatos com dano neurológico são freqüentemente incapazes de deglutir necessitando de gastrostomia para alimentação. Devido ao risco de refluxo grave, esse procedimento é associado à fundoplicatura. OBJETIVO: Avaliar a segurança e eficácia da gastrostomia com fundoplicatura Nissen em 22 neonatos com dificuldades de deglutição devido à lesão neurológica grave. MÉTODO: Todos os neonatos foram submetidos a um período inicial de alimentação por sonda nasogástrica e, após consentimento informado, foram submetidos a gastrostomia com fundoplicatura Nissen. RESULTADOS: Não houve complicações intra-operatórias significativas. Houve dois casos de vazamento pós-operatório da ostomia. Dos 22 neonatos, 16 estavam vivos 4 meses após a cirurgia. Seis neonatos morreram por complicações devido à doença de base. CONCLUSÃO: A gastrostomia com fundoplicatura Nissen é procedimento seguro e ajuda os pais a darem melhor cuidado a crianças com lesão neurológica.

Panencefalitis esclerosante subaguda: experiencia en la consulta de neuropediatría del Hospital J.M. de Los Ríos / Subacute sclerosing panencephalitis: experience in the ambulatory clinic of the Hospital J.M. de Los Rios

La panencefalitis esclerosante subaguda (PEES), o enfermedad de Van Boager, es una encefalopatía lentamente progresiva, originada por la infección persistente por una forma mutante del virus del sarampión, que ocasiona una desmielinización inflamatoria multifocal del sistema nervioso central. Es conocido que infecciones antes de los 2 años de edad aumentan el riesgo de padecer PEES, no demostrándose hasta la actualidad casos secundarios a vacunación. Presentar una revisión de cinco casos clínicos, que consultaron el Hospital de Niños "J.M de Los Ríos", en el periodo comprendido entre los años 1990-2005, con diagnóstico de PEES; en quienes se analiza la forma de presentación, manifestaciones clínicas, hallazgos en los estudios serológicos, de imágenes y evolución. Todos los pacientes tuvieron antecedentes de infección por sarampión cuatro de ellos antes de los dos años de edad. La edad promedio de presentación fue de 7 años 3 meses, con una media para el período de latencia de 5,2 años. En relación a las manifestaciones clínicas iniciales, en todos hubo crisis epilépticas mioclónicas, en dos de ellos trastornos conductuales y en otros dos somnolencia. Los estudios electroencefalográficos mostraron un patrón periódico en todos los casos. Los t¡tulos de anticuerpos antisarampión positivos en suero y líquido cefalorraquídeo confirmaron el diagnóstico en cuatro casos. La evolución fue fatal en un caso y desfavorable en los otros, con compromiso en áreas motoras, sensoriales y cognitivas. El tratamiento hasta los momentos sigue siendo preventivo, erradicando el sarampión y manteniendo un plan de vacunación.

Subacute sclerosing panencephalitis (SSPE), or disease of Boager Goes, is a slowly progressive encephalopathy originated by the persistent infection of a mutant form of the measles virus, which causes an inflammatory multifocal demielinization of the central nervous system. It is known that infections before 2 years of age increase the risk of SSPE. Cases following vaccination have not been demonstrated. To present a review of five children with SSPE attended at the Children's Hospital ¨J.M de Los Ríos" between 1990 and 2005. The initial presentation, clinical manifestations, serologic and imaging findings as well as the clinical outcome were analysed. All patients had a precedent measles infection, four of them before two years of age. The average age of presentation was 7 years 3 months, with an average for the period of latency of 5.2 years. In relation to the clinical initial manifestations, myoclonic events were common to all patients, two of them presented with cognitive deterioration and two with drowsiness. The EEG studies demonstrated a periodic boss in all cases. Anti measles antibodies titers in serum and spinal liquid confirmed the diagnosis in four cases. The outcome was fatal in one case and unfavourable in the others, whom presented motor, sensory and cognitive deterioration. Treatment at the present time continues to lie in preventive measures, supporting the plan of vaccination and aiming to the eradication of measles.

Heminegligência e reabilitação cognitiva: um relato de caso / Heminegligence and cognitive rehabilitation: a case report

Reabilitação cognitiva (RC) é um conjunto de técnicas estruturadas para o treinamento de pacientes com lesão cerebral que apresentam seqüelas de memória, linguagem, atenção, funções executivas. A heminegligência, fenômeno em que não se percebe a metade esquerda do corpo e do campo visual, é coomum em lesões de hemisfério direito e geralmente favorece mau prognóstico em pacientes hemiplégicos. O objetivo deste trabalho é apresentar relato de caso de um paciente heminegligente submetido a um programa de reabilitação cognitiva no Laboratório de Neuropsicologia, Cognição e Reabilitação Cognitiva da Faculdade de Medicina da Universidade Federal do Rio de Janeiro (FM/UFRJ). Realizou-se treinamento cognitivo, com tarefas de lápis e papel e scanning visuoespacial, para o treinamento da atenção. O programa de treinamento resultou em aumento da percepção do campo visual e do hemicorpo negligenciado, favorecendo a reconstrução da imagem corporal e a reabilitação física. Concluiu-se pela importância da realização de estudos integrando reabilitação física, neuropsicologia e neurologia para ampliar a idéia de reabilitação funcional.

Early development destructive brain lesions and their relationship to epilepsy and hippocampal damage.

Fifty-one consecutive adult patients with epilepsy and early development destructive brain lesions were divided into three main groups according to the topographic distribution of the lesion on magnetic resonance imaging: hemispheric (H) (n=9); main arterial territory (AT) (n=25) and arterial borderzone (Bdz) (n=17). Eight (89%) patients from group H presented status epilepticus in the first 5 years of life, five of them associated with fever. Seventeen of the 25 patients from group AT (76%) had an obvious hemiparesis observed early in life. In addition, major prenatal events were significantly more common in the group AT compared with the other two groups. Among patients from group Bdz, prenatal or postnatal events were not identified, except for one patient. Conversely, nine patients from group Bdz (60%) showed a history of perinatal complications. Hippocampal atrophy (HA) was determined by visual analysis in 74.5% of all patients and by volumetry in 92%. The frequency of HA was comparable among groups, but patients from group H presented the most severe atrophy and more frequent hyperintense T2 hippocampal signal. In conclusion, these three groups of patients with early destructive lesions and epilepsy (H, AT and Bdz), appear to have distinct pathogenic mechanisms. Our data show that there is a striking association of HA with different patterns of neocortical destructive lesions of early development. This association seems to be related to a common and synchronic pathogenic mechanism. The recognition of the pattern and degree of HA among these patients with intractable seizures may influence the surgical rationale.

Cerebral hemiatrophy: an associated finding in an epileptic child.

Cerebral hemiatrophy is a rarely occurring condition of different etiologies that can be regarded as the final stage of a number of different disease processes. It is characterized by a marked asymmetry of the cerebral hemispheres. A 12 year old girl with history of epilepsy since infancy and psychomotor delay presented in status epilepticus, developed marked cerebral edema, bilateral uncal herniation and bilateral infarcts of the posterior cerebral artery territories. Autopsy findings revealed left cerebral hemiatrophy as an incidental findings. The clinicopathologic features and classification of this entity are discussed.

Cerebral hemiatrophy: an associated finding in an epileptic child

Cerebral hemiatrophy is a rarely occurring condition of different etiologies that can be regarded as the final stage of a number of different disease processes. It is characterized by a marked asymmetry of the cerebral hemispheres. A 12 year old girl with history of epilepsy since infancy and psychomotor delay presented in status epilepticus, developed marked cerebral edema, bilateral uncal herniation and bilateral infarcts of the posterior cerebral artery territories. Autopsy findings revealed left cerebral hemiatrophy as an incidental findings. The clinicopathologic features and classification of this entity are discussed.

Effect of the dietary fiber glucomannan on chronic constipation in neurologically impaired children.

OBJECTIVE: Inadequate dietary fiber intake is a widely accepted explanation for chronic constipation in children with severe brain damage. The aim of our study was to evaluate the efficacy of glucomannan, a soluble fiber, as a treatment for chronic constipation in these children. STUDY DESIGN: Twenty children with severe brain damage and chronic constipation were randomly assigned to double-blind treatment with either glucomannan (n = 10) or placebo (n = 10) for 12 weeks. Stool habits, total and segmental gastrointestinal transit times, and anorectal motility were evaluated in all children before and after the treatment period. RESULTS: Glucomannan significantly increased (P <.01) stool frequency, whereas the effect of placebo was not significant. Laxative or suppository use was significantly reduced (P <.01) by glucomannan but was not affected by placebo. Clinical scores of stool consistency were significantly improved and episodes of painful defecation per week were significantly reduced by glucomannan (P <.01) but not by placebo. However, neither glucomannan nor placebo had a measurable effect on total and segmental transit times. CONCLUSIONS: In neurologically impaired children, glucomannan improves stool frequency but has no effect on colonic motility.

Hypothalamic obesity caused by cranial insult in children: altered glucose and insulin dynamics and reversal by a somatostatin agonist.

OBJECTIVE: Hypothalamic obesity is a rare sequela of cranial insult, for which pathogenesis and treatment remain obscure. In rodents ventromedial hypothalamic damage causes hyperphagia, obesity, hyperinsulinism, and insulin resistance. Reduction of insulin secretion in humans may attenuate weight gain. METHODS: Eight children with intractable obesity after therapy for leukemia or brain tumors underwent oral glucose tolerance testing (OGTT) with simultaneous insulin levels before and after treatment with octreotide for 6 months. RESULTS: In comparison with a 6-month pre-study observation period, patients exhibited weight loss (+6.0 +/- 0.7 kg vs -4.8 +/- 1.8 kg; P =.04) and decrease in body mass index (+2.1 +/- 0.3 kg/m(2) vs -2.0 +/- 0.7 kg/m(2); P =.0001). Recall calorie count decreased during the 6 months of treatment (P =. 015). OGTT demonstrated biochemical glucose intolerance in 5 of 8 patients initially and in 2 of 7 at study end, whereas insulin response was decreased (281 +/- 47 microU/mL vs 114 +/- 35 microU/mL; P =.04). Percent weight change correlated with changes in insulin response (r = 0.72, P =.012) and changes in plasma leptin r = 0.76, P =.0004). CONCLUSIONS: Patients with hypothalamic obesity demonstrate excessive insulin secretion. Octreotide administration promoted weight loss, which correlated with reduction in insulin secretion on OGTT and with reduction in leptin levels. Pre-study biochemical glucose tolerance improved in several patients while they were receiving octreotide. These results suggest that normalization of insulin secretion may be an effective therapeutic strategy in this syndrome.